Congenital S

CHAPTER
61
Adult Congenital Heart Disease

Hillel Laks Daniel Marelli Mark Plunkett Jeff Myers
The steady rise in individuals who have survived congenital heart disease (with or without treatment) into adulthood is expected to create specialized requirements that mandate strategic collaborative care protocols for this swelling subpopulation. If one excludes all patients born before 1990 and those not diagnosed in the rst year, assuming stable mortality in early adulthood, nearly 760,000 adults will have congenital heart disease by 2020.16 Adults with congenital heart disease who are referred for surgery fall into three general categories: those without previous surgery, those with previous palliation, and those with complete physiologic or anatomic repair returning for revision of their repair because of residual defects or sequelae from their repairs.1,2,46 In the current era, there is a trend toward surgical correction of congenital heart defects in the neonatal period or during infancy. This approach aims at minimizing the long-term consequences of congenital heart defects, such as myocardial dysfunction, endocarditis, and the hematologic and cerebral complications of cyanosis.720 There are, however, some patients who present as adults (particularly from underdeveloped countries) without previous surgery. More common lesions in this category include aortic valve disease, coarctation, pulmonary stenosis, atrial septal defect, and patent ductus arteriosus. Less commonly seen are tetralogy of Fallot, ventricular septal defect, Ebstein anomaly, and coronary arteriovenous stulas. Palliated adults are also unusual and include patients with systemic-to-pulmonary artery shunts, Glenn cavopulmonary shunts, and pulmonary artery bands. The largest group includes adults who present with residual lesions or sequelae from previous surgeries. These conditions may include patch leaks, recurrent valvular or outow tract stenoses, recurrent coarctation, pulmonary valve regurgitation, valve stenosis after tissue valve replacement or homograft insertion, or aneurysm formation in the pulmonary artery or aorta. Both primary repair and redo procedures are frequently complex and at increased risk from long-standing abnormal physiology and hemodynamics. Surgical care of the adult with congenital heart disease requires a multidisciplinary team experienced in pediatric and adult cardiology and cardiac surgery.
GENERAL MANAGEMENT Preoperative Evaluation

The natural history of the congenital defect, the sequelae of previous surgical interventions, and the development of newly acquired cardiovascular disease mandate thorough evaluation during preoperative surgical planning. Longstanding cyanosis and pressure or volume overload may all result in right or left ventricular dysfunction and secondary valvular regurgitation that may require repair. Additionally, cyanosis and underperfusion of the lungs may cause the development of aortopulmonary collaterals that may require coil embolization prior to reoperation. Pulmonary vascular resistance is usually affected by long-standing excessive ow or by severe underperfusion and, in older patients, by ongoing pulmonary thromboembolism. Older patients may also develop coronary artery disease that requires concomitant revascularization. Transthoracic or transesophageal echocardiography provides excellent information regarding the segmental and morphologic cardiac anatomy. Additionally, hemodynamic data can assist in evaluating valvular function, stenosis, and direction of shunting. In most complex cases, cardiac catheterization is required and pulmonary vascular resistance is calculated directly. Quantitative perfusion lung scans are useful to assess right- and left-sided blood ow. Magnetic resonance angiograms with three-dimensional reconstruction can provide excellent views of anatomy and are particularly
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Part VII Other Cardiac Operations
helpful in preparation for redo coarctation procedures to delineate the aortic arch and descending aorta.
Procedures Requiring Reoperation

Redo median sternotomy can be hazardous and result in massive hemorrhage if thin-walled vascular structures are adherent to the posterior sternum. We obtain computed tomography scans in select patients to view the retrosternal structures. In some patients, the femoral artery and vein are exposed prior to sternotomy. If the aorta, right atrium, or a conduit is adherent to the back of the sternum, cardiopulmonary bypass may be initiated with femoral artery and vein cannulation using thin-walled cannulas. If the right atrium or right ventricle is entered inadvertently, an intracardiac defect such as an atrial or ventricular septal defect could result in catastrophic systemic air embolism. This complication can be avoided by instituting deep hypothermia with cardiopulmonary bypass, keeping the heart full, and discontinuing the cardiac dissection until the heart brillates. In the presence of aortic regurgitation, decompression of the left ventricle is accomplished by cannulating the left ventricular apex via a small submammary incision. Bleeding may be a problem during chest opening due to the presence of numerous large thin-walled vessels throughout the mediastinum that are commonly found in chronically cyanotic patients. Chronic cyanosis and polycythemia are associated with a coagulopathy due to platelet dysfunction. Chronic hepatic congestion affects the coagulation factors and exacerbates coagulopathy. The use of antibrinolytic agents such as aprotinin, aminocaproic acid, or tranexamic acid is considered in high-risk patients except when circulatory arrest is used. Topical hemostatic agents have proven very useful for oozing surfaces. Autologous blood donation (when not contraindicated) and the use of cell-saving devices are routine. A polytetrauoroethylene (GORE-TEX or PTFE) pericardial substitute is used at closure for patients requiring later surgery, such as palliative procedures or after use of homografts or tissue valves. This membrane facilitates the dissection of reentry and the risk of injuring a retrosternal structure is markedly reduced.
with acquired heart disease. Both right and left ventricular function may be compromised and require support. Both pulmonary and systemic vascular resistance may require manipulation. For this reason, right atrial (RA), pulmonary artery (PA), and left atrial (LA) pressuremonitoring lines are used when indicated. LA lines are used rather than attempting to obtain PA wedge pressures because of the danger of PA rupture and possible discrepancy between PA diastolic pressures and LA pressure. Transesophageal echocardiography is required postoperatively to assess left and right ventricular function, to evaluate left- and right-sided valve function, and to look for shunts. Ventilator control to reduce partial carbon dioxide pressure, as well as inhaled nitric oxide and milrinone, all are useful ways to reduce pulmonary vascular resistance.
Associated Procedures
Many adults with congenital heart disease require corrective surgery after associated procedures. In particular, all patients above the age of 40 years require preoperative coronary angiography. Due to a history of shunts or increased chest wall collaterals from cyanosis, many patients have aortic insufciency secondary to increased venous return to the systemic ventricle. Such patients should be considered for aortic valve repair.21 Other associated procedures often performed in adults with congenital heart disease include bicuspid aortic valve repair or replacement, mitral or tricuspid valve repair or replacement, and implantation of epicardial pacemaker leads and generator.2233 Our preferred approach for epicardial pacemaker implantation is a subxiphoid approach. Atrial tissue is easily identified near the inferior vena cava in most patients. A ventricular site is usually identified on the diaphragmatic surface of the heart. We prefer nonpenetrating steroid-eluding leads that are sutured onto the epicardium. The pacemaker battery is placed in the preperitoneal position beneath the fascia or in the subcutaneous tissues of the left upper quadrant. Transvenous leads are generally contraindicated in the systemic circulation because of the risk of thromboembolism. This is of particular importance in patients with single-ventricle physiology.
Myocardial Protection
Right and left ventricular hypertrophy, dilatation, or dysfunction may be present. Noncoronary collateral ow is usually increased and bronchial ow can be torrential. Myocardial protection is therefore critical to the outcome of complex procedures. A left ventricular vent and deeper hypothermia to 24C are required, and both antegrade and/or retrograde cardioplegia are given every 10 minutes. A purse-string suture is placed around the coronary sinus to improve retrograde distribution.
SPECIFIC CONGENITAL MALFORMATIONS Atrial Septal Defects

Anatomy Atrial septal defects (ASDs) of the secundum type are the most common lesions, but sinus venosus defects of both the superior and inferior vena caval types, as well as ostium primum ASDs, are seen in adults (Fig. 61-1).
Postoperative Care
The adult with congenital heart disease may present a far more complex postoperative course than the usual adult
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Chapter 61 Adult Congenital Heart Disease
Figure 61-1. Diagram showing the locations of the most common types of atrial septal defects. Sinus venosus defects are close to the superior vena cava-to-right atrial junction and are frequently associated with partial anomalous pulmonary venous drainage (not shown). The right superior pulmonary vein may drain directly into the superior vena cava or at the junction of the superior vena cava and right atrium.
Physiology and indications for surgery The amount of left-to-right atrial shunting is variable. In older patients, as right ventricular dysfunction and tricuspid regurgitation develop, the degree of left-to-right shunting may decrease. The left-to-right shunt may increase in other patients due to hypertension and reduced left ventricular compliance. Although most patients with an ASD are asymptomatic through the second decade, by the third or fourth decade adults commonly develop atrial fibrillation or a reduction in exercise tolerance, and eventually heart failure.3440 It is preferable to close the defect when the diagnosis is made, before the development of these sequelae. As long as the Qp:Qs (pulmonary flow:systemic flow ratio) is greater than 1.5:1 and the calculated pulmonary vascular resistance is less than 6 to 8 U/m2 (or Wood units/m2), depending on systemic vascular resistance at the time of measurement, closure is usually indicated. There is often associated tricuspid valve regurgitation, which is the result of annular dilatation due to volume overload of the right ventricle. Indeed, such regurgitation may account for shortness of breath on exertion. This can be particularly true for patients who have patch leaks after repair and who are referred for reoperation to address symptoms out of proportion to shunt size. Patients with a patent foramen ovale and systemic embolization are also candidates for closure of the defect. About 15 to 20% of children with an ASD eventually develop pulmonary vascular disease.4143 If it does not occur by the end of the second decade, it is very unlikely to occur. Pulmonary vascular disease eventually causes reversal of the shunt and development of hypoxia that may be intermittent
and severe, depending on right ventricular function and tricuspid regurgitation. Patients with a patent foramen ovale and systemic embolization are candidates for closure of the defect. In the past, patients with pulmonary vascular disease were considered inoperable and were candidates for eventual lung transplants and ASD closure. The use of long-term prostacyclin has allowed some patients to lower their pulmonary vascular resistance and develop a left-to-right shunt. We have successfully proceeded to ASD closure and continued prostacyclin therapy in some of these patients. Another option is an adjustable (see below) or apped patch closure, to allow a small amount of residual right-to-left shunting postoperatively.44 Operative procedure The operation is performed using cardiopulmonary bypass and moderate hypothermia. For cosmetic purposes in young women, a submammary skin incision with median sternotomy is used. For the last 10 years we have used a small right anterolateral thoracotomy (Fig. 61-2). Another option is a midline incision in the lower half of the sternum. Superior and inferior vena cava cannulation is achieved directly through the thoracotomy incision using rightangled metal-tipped cannulas. If aortic cannulation is difcult, femoral artery cannulation is used. The aorta is clamped and cold blood cardioplegia alternating with cold blood is run continuously to keep the left atrium and ventricle full to prevent air entry. The chest cavity is lled with carbon dioxide throughout the procedure. The ASD is closed either primarily or with a pericardial patch. The right-sided pulmonary veins must all be identied, and if they are draining anomalously, they are bafed to the left
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Figure 61-2. Patient position for minimally invasive right thoracotomy approach for repair of an atrial septal defect. A 5- to 7-cm incision is made in the submammary crease. An additional incision may be made in the groin crease for femoral artery cannulation.
side with a pericardial patch. It is also important to identify the inferior vena caval orice so that it is not inadvertently bafed into the left atrium by sewing the patch to a welldeveloped eustachian valve. Redundant right atrial wall may be excised and a regurgitant tricuspid valve (if present) is repaired with an annuloplasty, particularly if the tricuspid annulus is dilated to 34 to 38 mm. In patients with chronic atrial brillation, a Maze procedure is performed.45,46 After closure of the defect and right atrium, extensive de-airing is performed prior to releasing the cross-clamp. This includes syringe and large-bore needles, de-airing of the left atrium, and venting of the aorta. Rarely, if air has entered the left ventricle during the procedure, a small submammary left-sided thoracotomy is made, and a small left ventricular apical vent is also inserted to assist in deairing. The left ventricle is inspected with transesophageal echocardiography for residual air prior to release of the cross-clamp. Outcomes Long-term follow-up after repair of isolated ASDs is well documented.4751 When patients are operated on at or before 25 years of age, normal life expectancy is anticipated, but this may not be the outcome when patients are corrected after 25 years, when both right and left ventricular reserve are diminished following a long-standing ASD. However, patients with ASD closure after 40 years of age may still realize improvement of symptoms. At the University of Alabama, it was found that age and New
York Heart Association (NYHA) class were fairly well correlated, thus suggesting that ASD closure is indicated when the defect is hemodynamically significant (Qp:Qs 1.5:1). Older age per se is not a risk factor for operative mortality. Recently, keen interest in the use of intravascular devices to close ASDs has developed.5256 The Amplatzer device was recently approved by the Food and Drug Administration for clinical use. Follow-up of 5 to 7 years is now available showing good long-term results. This trend will grow, as the Amplatzer and CardioSEAL devices are currently approved by the Food and Drug Administration for atrial septal defect closure. Long-term follow-up is pending. There is growing interest in the use of endoscopically assisted placement of these devices, with or without robotic aid, to close these defects.57
Ostium Primum Defects and Late Reoperations After Repair of an Atrioventricular Canal
Ostium primum (partial canal) defects Ostium primum defects are unusual in adults; however, survival similar to that for a large ASD is expected for partial atrioventricular canal defects with minimal valvular incompetence. The suture line is placed on the tricuspid aspect of the defect and outside the conduction tissue, which is left beneath the patch. Atrioventricular valve (right or left) regurgitation is often present and should be
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A1 and P1. In this way, a low-prole mechanical valve can be positioned away from the left ventricle outow, with a projection into the left atrium.71 Leaet remnants are used to anchor the valve along the other segments of the annulus. Left ventricular outow tract obstruction after atrioventricular canal repair While left ventricular outow tract obstruction (LVOTO) due to tunnel-like narrowing is unusual in the setting of either partial or complete canal, there have been reports of other types of obstruction occurring late after repair. del Nido and associates have summarized this possibility well. They observed that it is more common in partial canal, and that it may be acquired rather than congenital. This underlines the need for lifelong surveillance.72,73 Repairs must be individualized and can be complex. Later reoperations are not uncommon. The anatomy that results in left ventricular outow tract obstruction may be related to left superior leaet chordae that are attached to the outlet septum, or to outgrowths of accessory valve tissue in the left ventricular outow tract. If they are primary chordae, the former requires division and re-attachment, repair, or valve replacement, while the latter may simply be excised. Other causes of left ventricular outow tract obstruction include a subaortic membrane or abnormal papillary muscle position.72 Many of these can be approached through the aortic valve.73 The group from Toronto has suggested that the outow tract can be effectively shortened and widened, by augmenting or lifting the left side of the superior bridging leaet. This is likened to conversion of a Rastelli A anatomy to a Rastelli C. This restores (increases) the angle between the plane of the outlet septum and that of the septal crest toward normal.74 A patch of glutaraldehyde-xed pericardium may then be used to close the resulting defect and lifts the leaet away from the left ventricle outow. A modied or full Konno procedure may be needed for cases in which there is tunnel-like narrowing with ventricular hypertrophy.
Figure 61-3. Diagram showing closure of ostium primum atrial septal defect combined with mitral valve repair and tricuspid annuloplasty. The cleft in the anterior leaet is closed with interrupted sutures. A mitral annuloplasty is used when indicated or the commissures are plicated. Autologous pericardium is used to close the atrial septal defect. The suture line is placed on the tricuspid valve side, and then continued inferiorly on the atrial wall, outside the conduction tissue (shown by the dotted line in the diagram), then under the lip of the coronary sinus and back to the edge of the ASD. The conduction tissue is thus left under the patch, and the coronary sinus drains into the right atrium. When indicated a tricuspid annuloplasty is performed using glutaraldehyde-xed autologous pericardium and 2-0 polytetrauoroethylene sutures for the annuloplasty.
repaired at surgery5862 (Fig. 61-3). Long-term results are excellent. Late left atrioventricular valve regurgitation after repair of atrioventricular canal About 10 to 30% of operated atrioventricular canal patients may present because of left atrioventricular valve malfunction.63,64 They are often successfully treated with a re-repair of the mitral valve; the most common cause is a residual or recurrent cleft in the anterior leaet. Annuloplasty or commissuroplasty may also be needed.6567 This can be in the form of several isolated stitches along the annulus or a band along the posterior aspect of the annulus. There is limited experience with creating a double-orice valve.68,69 A unique approach has been reported by the group from Toronto. The dysplastic superior and inferior bridging leaets are detached radially along the anterior annulus. They are then augmented using glutaraldehyde-xed autologous pericardium, allowing for better leaet coaptation. An annuloplasty is also performed.70 Some will require valve replacement which can be difcult due to the annulus projecting towards the left ventricle outow tract. This is because the inlet septum is shortened and the outlet septum is lengthened. A crescent-shaped patch may be used to lengthen the mitral-to-aortic continuity along the circumference of the annulus in the section of
Ventricular Septal Defects

Unrestrictive ventricular septal defects (VSDs) are mostly associated with congestive heart failure in infancy and are usually repaired in early childhood. Adult survival with an unrestrictive VSD can occur if there is concurrent pulmonary outow obstruction that restricts pulmonary blood ow, or if the development of severe pulmonary vascular disease reduces or reverses the left-to-right shunt (Eisenmenger syndrome). Restrictive VSDs are more commonly found in the adult and may be the result of a persistent small defect, partial spontaneous closure of a larger defect, or a residual patch leak following surgical repair. Controversy continues as to whether restrictive VSDs should be closed. A large study by Kidd and associates showed a 25-year survival of 87%, but noted a signicant increase in the risk of serious arrhythmias and sudden death.75 The evolving concept of monitoring unoperated
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Figure 61-4. Technique of aortic valve repair using pericardial pledgeted sutures to resuspend redundant leaets. Additional apposition can be achieved by adding sutures below the commissures or at the level of the annulus.
patients in adults with congenital heart disease was reinforced by Glen and coworkers in a study of 1448 patients with VSDs.76 He noted that 22% of these patients had associated defects. Most were detected initially but aortic insufciency and infundibular stenosis often developed subsequently. If follow-up of these patients was continued until age 30, 89% of patients with secondary aortic regurgitation would be detected. Nonoperative intervention is most appropriate in patients with small defects, a Qp:Qs ratio of 1.5:1.0, no left ventricular overload, and normal pulmonary artery pressures. Anatomy Most VSDs are categorized into four anatomic types. The perimembranous type is the most common. It is located under the septal leaet of the tricuspid valve. The subarterial VSD is located in the supracristal area and may lie directly beneath the annulus of the pulmonary valve. Because of the proximity of the aortic annulus to such defects, the right cusp of the aortic valve may prolapse into the defect, reducing the effective orice and limiting the shunt. Aortic regurgitation is frequently associated with this defect.77 With surgical closure of a subarterial VSD, the aortic valve may require repair with suspension of the prolapsing valve leaet (Fig. 61-4). The endocardial cushion type of VSD is located in the inlet of the right ventricle and beneath the septal leaet and posterior leaet of the tricuspid valve. This VSD may have associated mitral valve defects such as cleft anterior leaet and therefore require mitral valve repair at the time of surgery. The repair usually involves suture closure of the anterior leaet cleft and may require reduction of the dilated annulus with annuloplasty. The muscular type of VSD may occur anywhere within the muscular ventricular septum. This type is unusual in adults, as it has a tendency to close spontaneously in the rst years of life.78 Physiology Use of the term restrictive implies a pressure gradient between the ventricles and a restriction to ow across the
defect. The amount of left-to-right shunting across the defect can be quantied by calculation of the Qp:Qs ratio. VSDs with left-to-right shunting that results in a Qp:Qs ratio of 1.5:1 are usually not considered for repair, if there is no other associated pathology (e.g., aortic leaet prolapse and insufciency). Double-chambered right ventricle is an obstruction in the mid-portion of the right ventricle that divides the chamber into two segments: a high-pressure lower chamber and a low-pressure upper chamber. The development of obstructive tissue in the right ventricle is a direct result of a restrictive VSD producing a jet effect that strikes adjacent myocardium and produces an area of bromuscular proliferation. Right ventricular hypertrophy develops secondary to the obstruction and contributes to the progression of right ventricular hypertension. In many patients the VSD will eventually close, as the bromuscular rim of tissue proliferates around and over the defect. In these patients, the VSD is often identied at the time of surgery with resection of the obstructing tissue in the right ventricle. In patients with large unrestrictive VSDs, the probability of developing severe pulmonary vascular disease is about 50% by the third decade of life.79,80 Consequently, patients eventually die of complications of Eisenmenger syndrome if their VSDs remain unrepaired. These patients ultimately will become cyanotic as a result of right-to-left shunting, and the only surgical options available at that point are either heartlung transplantation or lung transplantation with repair of the VSD. A pulmonary vascular resistance greater than 6 U/m2 is considered a high risk for isolated VSD closure. A number of promising new medical therapies are being applied to patients with pulmonary hypertension. Use of nitric oxide donors such as sildenal, endothelin antagonists, and prostaglandin analogs may alter the outcome in these patients and potentially reverse the pulmonary hypertension to levels that will allow for surgical correction. Preoperative evaluation An adult patient with a VSD should be evaluated by chest radiograph and echocardiography. A chest radiograph may
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show an enlarged right heart shadow and right ventricular hypertrophy. It may also reveal chronic changes in the pulmonary vasculature, prompting further evaluation by catheterization. Transthoracic and transesophageal echocardiography are both useful in dening VSDs anatomically and identifying associated lesions.81 An estimate of the pulmonary artery pressures may also be obtained. Indications for surgery are helpful in determining the need for catheterization. Patients with embolic complications and very small defects with high gradients and no evidence of pulmonary hypertension can usually be closed without catheterization. However, patients with signicant symptoms attributed to their VSD should undergo catheterization to rule out pulmonary hypertension. Right and left heart catheterization is useful to conrm the anatomic ndings and to accurately measure the pulmonary vascular resistance and estimate the Qp:Qs ratio. In patients more than 40 years old or in those with increased risk factors, coronary artery disease must also be excluded by angiography. Indications for surgery Adults with small restrictive VSDs who are asymptomatic often require no surgical intervention and should receive endocarditis prophylaxis as necessary. In general, if the Qp:Qs ratio is 1.5:1 and the calculated pulmonary vascular resistance is under 6 U/m2, surgical closure of a VSD can be performed safely and is recommended. The development of a double-chambered right ventricle with outow obstruction is also an indication for operative intervention. The occurrence of infective endocarditis in an adult with a restrictive VSD is a rare but compelling indication for repair of the defect. In adults with VSD and Eisenmenger syndrome, heart-lung transplantation or lung transplantation with closure of the defect may be considered. Operative procedure Operative repair of VSDs is performed on cardiopulmonary bypass with moderate systemic hypothermia and blood cardioplegia for myocardial protection. Generally, greater operative exposure is required than for the repair of ASDs. However, many of the cosmetic modifications discussed in the section on atrial septal defects are appropriate here. Bicaval cannulation with caval snares helps to produce a bloodless eld. A left-sided vent is also helpful in evacuating blood from the left heart. This can either be placed through the left superior pulmonary vein or through an incision in the atrial septum. An appropriately sized patch is then created and sewn in place using either interrupted, pledgeted horizontal mattress sutures or a continuous running suture. The patch is cut slightly larger than the defect so that the sutures can be placed away from the edge and the conduction system. Most adults with a perimembranous, inlet, and/or muscular VSD can have the defect repaired through the tricuspid valve annulus. If the edges of the defect are difcult to visualize due to multiple attachments of the septal leaet to the edges of the defect, the septal leaet may be incised at its base
adjacent to the annulus or radially through the septal leaflet, exposing the VSD under the leaflet. Subarterial VSDs may be approached through the pulmonary valve or the right ventricular outow tract. Exposure through the pulmonary valve is particularly helpful in placing sutures through the pulmonary valve annulus without damaging the valve. The patch material for closure of VSDs may be synthetic (e.g., PTFE or Dacron), or glutaraldehyde-xed autologous pericardium, which we have preferred for over 15 years. PTFE may produce less hemolysis than pericardial patches. However, it may also be less well-incorporated and therefore small residual VSDs may not close as readily. Postoperatively, elevated pulmonary artery pressures may be treated with inhaled nitric oxide. A fenestrated VSD patch can be created to transiently allow right-to-left shunting and maintain cardiac output in the immediate postoperative period. It can subsequently be closed in the cardiac catheterization lab. Currently, transcatheter closure of VSDs remains experimental.82,83 Outcomes Long-term follow-up is recommended to monitor pulmonary artery pressures in those adult patients with large VSDs that are repaired late in life.84 This is achieved echocardiographically if there is a mild tricuspid regurgitation from which to calculate the right ventricular pressure, or by measuring the pulmonary valve opening time. The pulmonary vascular resistance may continue to rise after VSD closure, resulting in systemic or suprasystemic right ventricular pressures. This may eventually cause the onset of angina, right heart failure, or even sudden death. In an analysis of 52 patients between the ages of 16 and 67 years repaired at the University of California, Los Angeles, there was no mortality. In most patients, the VSD repair was combined with additional procedures such as aortic valve repair, pulmonary valve replacement, and tricuspid valve repair. Except in patients with congenitally corrected transposition, the incidence of complete heart block following VSD closure is approximately 1%. Overall, the long-term outcome for these patients has been excellent.
Patent Ductus Arteriosus

Natural history and indications for surgery Isolated patent ductus arteriosus (PDA) may present with congestive heart failure by the third or fourth decade of life.85 Occasionally, the ductus may become aneurysmal secondary to ow characteristics. The aortic end of the PDA is usually calcied in the older adult patient. A longstanding left-to-right shunt may lead to pulmonary vascular disease. The cumulative death rate in childhood is about 0.5% per year. This doubles to 1% by adulthood and increases to 2 to 4% by midlife. There is always a risk of endocarditis (regardless of the PDA size) that is dependent on the presence of abnormal ow. Adults with
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large PDAs may develop Eisenmenger syndrome and the classic nding of differential cyanosis in the lower body. It is imperative to determine pulmonary vascular resistance and reactivity preoperatively. If the resistance is 6 to 8 U/m2, the ductus should not be closed, and the patient may be considered for lung or heart-lung transplantation. Operative procedure Surgical closure of a PDA is usually carried out via a small posterior thoracotomy. Thoracoscopic procedures are probably not appropriate for the adult because of the frequency of calcification and the greater risk of rupture while ligating the ductus.86,87 In patients over 40 years of age, or in the presence of severe ductal calcification or aneurysm, consideration should be given to performing PDA ligation via a median sternotomy using cardiopulmonary bypass.88,89 During cooling, the branch pulmonary arteries are snared. This prevents steal from the descending aorta. The ductus is exposed via an incision in the PA. Using low ow, the ductus is closed on the PA side with horizontal pledgeted mattress sutures, or the PA defect is patched with glutaraldehyde-xed pericardium or a PTFE patch. Patients with Eisenmenger syndrome may be candidates for either single- or double-lung transplantation combined with PDA closure, or heart-lung transplantation if there is signicant ventricular dysfunction and tricuspid valve regurgitation. Catheter closure by interventional cardiology specialists using coils or other devices may be possible depending on the size and length of the ductus.9093 In the last 5 years, catheter-based closure of this lesion in the adult has been reported as the most common after ASD, VSD, and coarctation.94 Similarly, surgeons have also reported a catheter-based approach in which the patent ductus is excluded with a stent graft placed in the proximal descending aorta.9597
anomalies occur in 59 to 92% of patients and include a bicuspid aortic valve in 40 to 80% and aortic arch hypoplasia in 33%.98 Natural history and indications for surgery Aortic coarctation in adults usually presents with upperbody hypertension, typically in the second or third decade of life.99 Lower extremity pulses are usually weak and delayed. Although these patients comprise a selected group who have survived free of complications beyond childhood, long-term complications include aneurysm formation of the aorta and aneurysmal dilatation of intercostal arteries, which may eventually rupture. This latter anomaly is important because the initial portion of these arteries should be occluded at the time of surgery if they appear disproportionately enlarged. Other complications include premature coronary artery disease, left ventricular hypertrophy, aortic dissection and rupture, endocarditis, and intracranial hemorrhage. In adults with coarctation, congestive heart failure may develop from long-standing hypertension. Patients with an associated bicuspid aortic valve may develop stenosis and/or incompetence. 23 If aortic coarctation is left untreated, 90% of patients eventually die by the age of 50 due to cardiac causes or stoke. The oldest patient in our series was 81 years old. Repair also may be required for patients who have had previous coarctation repairs with recurrence or for patients who have developed recurrence after previous balloon aortoplasty. As many as 70% of infants who have unrepaired arch hypoplasia at their initial operation may require reoperation as adults. Residual coarctation following repair in childhood is also due to failure of growth of the anastomosis or technical factors such as a short subclavian ap aortoplasty. In patients who were initially treated with a patch, aneurysm formation may occur and require reoperation. Surgical repair is indicated when the gradient across the coarctation is 30 mm Hg at rest.100 If the gradient is less and the anatomic obstruction severe, an exercise test will reveal a more severe gradient, and repair is indicated. Preoperative evaluation The anatomy of the entire left ventricular outow tract and thoracic aorta must be dened prior to surgical intervention. Previously repaired patients may have additional levels of obstruction from a hypoplastic aortic arch that has failed to grow or a stenotic/regurgitant aortic valve. Surgical intervention should be tailored to address all levels of obstruction. Echocardiography is performed to evaluate the aortic valve, ventricular function and hypertrophy, and the aorta. In adults, magnetic resonance angiography with three-dimensional computerized reconstruction to assess the transverse arch, isthmus, and descending aorta is often useful (Fig. 61-5). Operative procedure The preferred method is resection with extended end-to-end anastomosis (Fig. 61-6), although patch repair is used for
Coarctation of the Aorta

Anatomy Coarctation in adults may occur in previously unrepaired patients or patients who have had prior surgical correction. In unrepaired patients an abnormality of the media creates a posterior shelf, opposite the ligamentum arteriosum. This shelf may extend circumferentially. The abnormality, similar to cystic medial necrosis histologically, predisposes patients to late complications such as dissection and aneurysm formation. There is often extensive collateral circulation in adults with coarctation. The source is mainly from branches of the subclavian artery and internal mammary arteries, and intercostal chest wall circulation. Collateral ow into the descending aorta is dependent on enlarged intercostals at the level of the third and fourth ribs beyond the coarctation. Additional cardiovascular
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Figure 61-5. Angiogram of a 27-year-old patient who presented with residual coarctation after initial repair in which a 16-mm interposition graft was inserted. She presented with stenosis at the distal transverse arch and at the interposition graft with proximal hypertension. Repair using a left atrial-to-aortic bypass circuit involved repairing the distal arch and replacing the graft with a 20-mm PTFE conduit and extended anastomosis.
reoperations or where the collaterals are particularly enlarged and difcult to mobilize.99,101103 Tube-graft interposition is used when indicated to relieve long segments of obstruction. Special precautions are taken to reduce the major risk of spinal cord ischemia. Arterial lines are placed in the upper and lower extremities for monitoring blood pressure during aortic clamping. The distal pressure should be maintained above 50 mm Hg throughout the procedure. Somatosensory evoked potentials are monitored intraoperatively to aid in the decision to use extracorporeal circulation to help prevent spinal cord ischemia during aortic crossclamping. In extensive reoperations or operations for aneurysms, the cerebrospinal uid pressure is monitored by catheter, and the uid is allowed to drain if pressure exceeds 10 cm H2O (essentially central venous pressure). The cerebrospinal uid pressure is monitored for 24 hours postoperatively. The goal is to optimize perioperative perfusion of the spinal cord by increasing the pressure gradient during and after aortic clamping. The patient is placed on a temperature-regulated blanket and cooled to 33 to 34C. Cold saline is used to bathe the left chest cavity to aid in cooling, and the room is cooled. Positioning for the left thoracotomy is important in that one must prep and drape the groins to have access to the femoral arteries if left atrial-to-femoral artery (or descending aorta) bypass becomes necessary during the operation. One must carefully identify chest wall collaterals, which can bleed massively and which must be ligated individually during the thoracotomy.
The aorta is mobilized extensively, and the ligamentum arteriosum is divided and oversewn. Large intercostal branches are identied and encircled in preparation for snaring. On induction of anesthesia, the patient is given 30 mg/kg of methylprednisolone sodium succinate and lidocaine 2 mg/1 kg intravenously, as well as 8 g of mannitol. The patient is anticoagulated with 1 mg/kg of heparin. The aorta is clamped when the rectal temperature is 34C or below. Once the aorta is clamped proximally and distally, if the distal pressure is below 50 mm Hg, distal aortic bypass is instituted. The distal pressure is maintained above 60 mm Hg. This consists of left atrial-to-descending aortic bypass using a centrifugal pump. In more complex redo or aneurysm procedures, pulmonary artery-to-descending aortic bypass can be used with an oxygenator. Upper extremity pressure is maintained at about 120 mm Hg systolic. Once the aortic coarctation is resected, reconstruction with a tube graft or end-to-end anastomosis is carried out with 4-0 polypropylene suture mounted on a small needle. For patients undergoing reoperation for recurrent coarctation, mobilization of the aorta for end-to-end anastomosis may be difcult and cause excessive blood loss. In these patients, the aorta is clamped proximal to the left subclavian artery, and a glutaraldehyde-xed autologous pericardial patch may be used to enlarge the aorta from the base of the subclavian artery to the distal aorta. An excessively large patch should be avoided to prevent late aneurysm development.
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Figure 61-6. Resection of coarctation of the aorta. (A, B) With extended end-to-end anastomosis. (C, D) After excision of the coarctation site and reconstruction of the posterior wall by end-to-end anastomosis, a glutaraldehyde-xed autologous pericardial patch is used to enlarge the isthmus and the site of coarctation repair. The patch is measured to avoid excessive dilatation, which can result in late aneurysm formation.
Some institutions prefer construction of an ascendingto-descending aortic bypass graft in patients with recurrent coarctation. The operation has been performed through either a right thoracotomy or via a posterior pericardial approach through a median sternotomy.104,105 Both have reported a low mortality and morbidity with excellent relief of hypertension and resolution of the blood pressure gradient between the upper and lower extremities. A 16- to 20mm graft is used and the repair through the right chest can be performed off bypass. Both approaches avoid the complications associated with a redo left thoracotomy, including injury to the recurrent laryngeal nerve, intercostal arteries, and lung. Aortic bypass graft also addresses signicant arch hypoplasia and avoids the need for clamping of the hostile aorta that may be thin or calcied. There should be no gradient between the upper and lower extremities upon release of the clamps. During closure, special care is taken to control intrathoracic bleeding and to check chest tube and pericostal suture sites. Hypertension is controlled and is treated aggressively in the intensive care unit. Abdominal pain and distension
may be present in 5% of patients postoperatively. Management is usually conservative. The patient is given nothing by mouth for at least 24 hours postoperatively until bowel sounds return. In older patients who have a coarctation and also require coronary revascularization or aortic valve repair or replacement, we prefer a median sternotomy approach with cannulation of both the ascending aorta and the femoral artery. After completing the coronary revascularization, an adequately sized Dacron graft can be placed between the ascending aorta and the proximal abdominal aorta through the diaphragm. Another option to consider is a clamshelltype incision to access the descending thoracic aorta and the mediastinum simultaneously. Outcomes Outcome following repair is generally good, and follow-up may be assisted with transesophageal echocardiogram, computed tomographic scan, or magnetic resonance imaging. The latter is useful to detect aneurysm formation or recoarctation.106,107 Recoarctation is dened as a gradient 20 to 30
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mm Hg at rest. The possibility of coronary disease and systemic hypertension requires lifelong monitoring. Catheter-based techniques are being used for both primary coarctations and recurrences.108110 Residual mild gradients are common after angioplasty and/or stenting of primary coarctations and we therefore prefer surgical therapy.99 Since the presence of a residual gradient of 10 mm Hg is associated with an increased risk of adverse cardiovascular events, intervention must result in long-term, near-total resolution of the gradient.111 Remembering that the primary goal is resolution of hypertension, surgery has consistently resulted in long-term relief in over 60% of patients. Recent analyses have shown that angioplasty alone results in a high rate of reintervention and is markedly worse at curing hypertension than surgery.112 Angioplasty was found to be comparable to surgery with regard to morbidity, but appears to be much less effective in the cure of hypertension. For selected recurrent coarctations, stenting may be the preferred method, provided that an excellent anatomic relief of the obstruction can be achieved.113 However, in younger patients in whom a long-term result is required, surgery may still be preferable, even in complex lesions.
branch pulmonary arteries, either primarily or secondary to shunts. Aortopulmonary collaterals arising from the aorta may coexist, as is commonly found in patients having pulmonary atresia with ventricular septal defect. Occasionally, cyanotic adults will present with unrepaired TOF and severe pulmonary obstruction, but adequate collateral pulmonary blood ow to allow survival beyond childhood. Physiology The pathophysiology of TOF results in restriction of pulmonary blood ow secondary to obstruction of right ventricular outow and right-to-left shunting across the ventricular septal defect. The age at presentation and the degree of cyanosis vary directly with the degree of obstruction to pulmonary blood ow. Patients undergoing palliative shunt procedures in childhood to increase pulmonary blood ow may do quite well if the resulting oxygenation remains adequate with subsequent growth. These systemic-to-pulmonary shunts are often outgrown at an early age, requiring reintervention for additional palliation or more denitive repair. While currently the modied Blalock-Taussig shunt or central shunt is used for palliation in most patients, the Potts (ascending aorta-to-right pulmonary artery) and Waterston (descending aorta-to-left pulmonary artery) shunts were used in the past and may be found in some adult patients. Indications for surgery Residual VSDs, residual or recurrent obstruction to pulmonary blood ow, and severe pulmonary insufciency with progressive right ventricular dilatation and dysfunction are all indications to consider reoperation in adults who have undergone previous complete repair of TOF. Patients who have previously undergone right ventricle-to-pulmonary artery conduit placement often present later in life with conduit stenosis requiring replacement. Patients who have had TOF repair in late childhood may have other sequelae that may have an impact on late reoperative surgery. The longterm volume load from a large shunt may produce permanent left ventricular dysfunction. The pulmonary vascular resistance may be elevated. Mild or even moderate aortic valve regurgitation is not uncommon due to dilatation of the aorta and may require aortic valve repair or replacement. Pulmonary valve regurgitation is very common after repair of TOF, since approximately 70 to 80% are repaired with a transannular patch. Even though exercise capacity may be decreased, the vast majority of patients tolerate this well unless they have an additional residual VSD or pulmonary artery stenosis. In addition, some patients, without these associated residual defects, will slowly develop right ventricular dilatation and severe tricuspid regurgitation. This may progress for over 20 years, and patients are currently presenting late as they become symptomatic from combined pulmonary and tricuspid valve regurgitation. In view of the risks of sudden death and the progressive nature of right ventricular dysfunction, surgical intervention is recommended.
Tetralogy of Fallot
Tetralogy of Fallot (TOF) is the most common cyanotic heart defect in children, constituting approximately 10% of all congenital heart disease. It is therefore one of the most common cyanotic congenital heart defects found in adults. Successful repair of TOF in childhood has spanned almost four decades, with many of those patients now returning as adults for reoperation.114123 These patients constitute the majority of adults presenting for surgical intervention for TOF. There are also adults with TOF who underwent palliative procedures in childhood but never underwent complete repair of the defect.124 Occasionally, a patient with a wellbalanced TOF defect and adequate pulmonary stenosis to protect their pulmonary vasculature will reach adulthood without any operative intervention. Anatomy TOF is classically dened by Fallots four original pathologic ndings: obstruction of right ventricular outow, ventricular septal defect, overriding aorta, and right ventricular hypertrophy. The defect is the result of an anterior displacement of the infundibular septum during development, resulting in obstruction to right ventricular outow and a malalignment ventricular septal defect. The aorta is displaced toward the ventricular septum, resulting in an overriding position, and hypertrophy of the right ventricle is a direct consequence of the outow obstruction. The obstruction to pulmonary blood ow is often at multiple levels, which may include subvalvular, valvular, and supravalvular stenosis. The pulmonary valve is frequently malformed or bicuspid and the annulus is often small. Hypoplasia of the pulmonary arteries may be present if the obstruction to pulmonary blood ow is severe, and there is often stenosis of the
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Figure 61-7. A three-dimensional magnetic resonance image documenting aneurysmal dilatation of the right ventricular outow tract in a patient with previous repair of tetralogy of Fallot.
Aneurysms of the right ventricular outow tract may occur following the use of an excessively large transannular pericardial patch with the initial repair (Fig. 61-7). Such aneurysmal dilatation may progress, especially if there is associated right ventricular outow tract obstruction. Sudden death after TOF repair accounts for a signicant number of late deaths. It usually occurs in patients who have had a right ventricular incision and have right ventricular dilatation combined with an elevated right ventricular pressure above 60 mm Hg.125 All else being equal, a QRS duration of greater than 180 milliseconds is associated with an increased risk of sudden death, and a progressive increase in QRS duration is considered a factor in deciding on reoperation.126 Any ventricular arrhythmias should be evaluated by electrophysiologic studies and focal pathways should be treated with catheter ablation. Pacemakers are required in fewer than 4% of patients late after TOF repair.127 They may be indicated for sick sinus syndrome, the combination of right bundle-branch block with left anterior hemiblock, or the late onset of complete heart block. Preoperative evaluation The preoperative evaluation in adults with TOF must take into consideration the patients previous operative interventions. In addition to chest radiography and electrocardiography, transthoracic or transesophageal echocardiography has
become the fundamental diagnostic tool in most of these patients. Angiography is indicated to dene specic hemodynamics such as the pulmonary vascular resistance and to dene the pulmonary artery anatomy. Aortic and selective injections are performed to look for aorta-pulmonary collaterals. The coronary anatomy must be known, as 4 to 5% of TOF patients have an left anterior descending artery arising from the right coronary artery, which can be injured by a transannular incision. Adults over age 40 and those with risk factors for early coronary artery disease should undergo coronary angiography to evaluate the need for concomitant coronary bypass. Magnetic resonance imaging with angiography or computed tomography scans with three-dimensional reconstruction have proven valuable for dening the anatomy. In all patients with TOF, there should be an evaluation of the size and patency of the pulmonary trunk and its branches, the size of the pulmonary annulus, the stenosis and/or competency of the pulmonary valve, the proximal coronary artery location, and the presence of systemic-topulmonary artery collaterals. If there is a right ventricle-topulmonary artery conduit or an aneurysmal transannular patch present, magnetic resonance angiography or computed tomographic imaging studies should be used to evaluate the proximity of these structures to the sternum and to the midline site of the redo sternotomy. Preoperative electrophysiologic studies may be indicated if signicant arrhythmias are identied.
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Operative procedure In most adults with TOF, bicaval cannulation is used, and myocardial protection involves both antegrade and retrograde cold blood cardioplegia followed by warm blood cardioplegia and warm blood reperfusion. Ventricular distension is avoided with venting of the left ventricle. Atrial septal defects should be sutured primarily or closed with a patch of native pericardium. Ventricular septal defects may be approached through the right atrium or through the right ventricular scar or outow tract patch. A PTFE or glutaraldehyde-xed pericardial patch may be used. The right ventricle is remodeled by resection of scar from the previous ventriculotomy and any aneurysmal tissue in the right ventricular outow tract. The pulmonary valve is replaced, usually with an oversized porcine bioprosthetic valve seated below the native annulus in the right ventricular outow tract (Fig. 61-8). A 27- or 29-mm porcine valve can usually be accommodated in all adults. A transannular hood of pericardium or PTFE is used to cover the porcine valve and establish continuity to the pulmonary artery. Homografts are used to replace previously inserted conduits (Fig. 61-9). The pulmonary homograft lasts longer than the aortic homograft but develops regurgitation earlier. The tricuspid valve can usually be repaired with an annuloplasty and rarely requires replacement. In some cases adherence of the septal leaet to the VSD patch can be repaired by suturing it to the adjacent anterior and posterior leaets. Denitive procedures in adults may require takedown of previously placed shunts. Systemic-to-pulmonary artery type shunts should be controlled as soon as cardiopulmonary bypass is instituted. Takedown of a Waterston shunt is done from within the pericardium. The right pulmonary artery is
mobilized. The aortic cannulation site for cardiopulmonary bypass is placed distally. When cardiopulmonary bypass is initiated, shunt ow is controlled with a clamp ush with the aorta, and the patient is cooled to 20C. Cardioplegia is administered after aortic clamping. With the heart arrested and at low ow, the shunt clamp is released, and the anastomosis is excised from the aorta. This mobilizes the right pulmonary artery. The aorta is closed primarily. The incision in the right pulmonary artery is extended proximally and distally to relieve any stenoses. The right pulmonary artery is reconstructed with a pericardial or PTFE patch. Takedown of a Potts anastomosis usually requires a period of low ow or circulatory arrest.128 The shunt is occluded by pressure on the left pulmonary artery while blood is cooled to below 20C. With the head down, the left pulmonary artery is incised and opened under low ow so that the opening to the aorta can be occluded with a Hegar dilator. Under low ow or circulatory arrest, the aortic side is closed primarily, and the pulmonary artery is repaired with a pericardial patch (Fig. 61-10). Takedown of a Blalock-Taussig shunt is usually achieved by dissection, ligation, and division of the shunt at the initiation of cardiopulmonary bypass. Outcomes Long-term results are well documented in patients who had complete repair of TOF in the late 1950s and early 1960s.129134 Actuarial survival ranges from 77 to 90% at between 20 and 30 years of follow-up. Late mortality from cardiac causes accounts for about two-thirds of all late deaths. Between 40 and 60% of these are sudden and presumed to be due to arrhythmias or to heart block. Other causes include right ventricular outow abnormalities
Figure 61-8. Pulmonary valve replacement with patch enlargement of the right ventricular outow tract. The incision extends across the annulus and beyond the bifurcation to the left pulmonary artery. The outsized porcine valve (27 or 29 mm) is placed within the right ventricular outow tract to accommodate the larger size valve.
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Figure 61-9. Homograft replacement of the right ventricular outow tract with hood augmentation of the proximal anastomosis and patch enlargement of the branch pulmonary arteries. A reinforced PTFE conduit may be placed behind the aorta to re-establish continuity between the right and left pulmonary arteries.
(obstruction, pulmonary incompetence, and aneurysm) and congestive heart failure partly related to residual VSDs, which were reported in 1 to 8% of all operated patients in these early series. Currently, residual VSDs are expected in less than 5% of repairs for TOF.
The results of primary repair of TOF in adults are very good. Presbitero and colleagues reported an operative mortality of 2.8% in a series of 40 adults with TOF repairs.129 There were two residual VSDs and two patients with residual right ventricular outow tract obstruction. The results of
Figure 61-10. Patch repair of a Potts shunt anastomosis. The main and left pulmonary arteries are incised (A) to expose the opening (B) in the posterior proximal left pulmonary artery. The defect is closed with a pericardial or prosthetic patch (C).
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reoperative surgery in adults with TOF are also generally good. Mortality ranges from 7 to 20%. Pome and associates reported an actuarial survival of 87% for 22 patients at 20year follow-up for this particular cohort.122 Eighty-nine percent of patients were NYHA class I, and only 1 patient (5.5%) was in class III. Two women in this series experienced uncomplicated pregnancy. In the series from the Mayo Clinic reported by Uretzky and coworkers, 5 patients (12%) had a second reoperation.133 At the University of California, Los Angeles, we have operated on adults with TOF ranging in age from 16 to 65 years. Most had patch repairs of the ventricular septum, insertion of a right ventricular outow patch, and pulmonary valve replacement. The oldest TOF patient underwent primary repair and coronary artery bypass grafting. There was no mortality or signicant morbidity in this series.
Such elevated gradients may preclude complete repair. Magnetic resonance angiograms with three-dimensional reconstruction give detailed pictures of the anatomy. Indications for surgery Patients with inadequate pulmonary blood ow are limited due to cyanosis and may require unifocalization if they have an adequate bed for future repair. If they do not, they may be candidates for a palliative shunt. Patients with excessive pulmonary blood ow and failure can also be treated by unifocalization with reduction of the total ow. The size of the shunt to the unifocalization is crucial to adjust ow to the pulmonary vasculature. After unifocalization on one side (Fig. 61-11), the opposite side is unifocalized 6 months to 1 year later (Fig. 61-12), followed 6 months to 1 year later by a complete repair (Fig. 61-13). Shunted patients should be repaired if they have an adequate size pulmonary bed, unless they are inoperable because of high pulmonary vascular resistance or poor ventricular function. A residual VSD should be closed in previously repaired patients if the left-to-right shunt is 1.5:1 or above. Conduit or valve obstruction is reoperated when there are symptoms, or if the right ventricular pressure at rest is two-thirds to three-fourths of systemic pressure. If there is severe pulmonary valve regurgitation with right ventricular dilatation or tricuspid regurgitation, reoperation should be undertaken. Staged surgical repair The goal of surgical management of pulmonary atresia, ventricular septal defect, and multiple aorta-to-pulmonary collateral arteries is closing the ventricular septal defect and establishing continuity between the right ventricle and pulmonary artery. Ultimately, successful denitive repair requires an adequate pulmonary vascular bed, without which VSD closure and right ventricular-to-PA continuity will lead to right ventricular failure due to a prohibitively high pulmonary vascular resistance. Thus, all efforts during the operative staging are designed to maximize the size, distribution, and normal ow of the pulmonary arteries while preserving myocardial function. Early palliative procedures in patients with excessive or inadequate pulmonary blood ow were designed to create a balanced pulmonary blood ow and encourage growth of the true pulmonary arteries. Unifocalization procedures join the multifocal sources of pulmonary ow (true pulmonary arteries and aorta-topulmonary artery collaterals) into a single source that can ultimately be accessed in the anterior mediastinum via median sternotomy. The unifocalization procedure is performed through a posterolateral thoracotomy incision. A double-lumen endotracheal tube is employed, when possible, for large children and adults. Single-lung ventilation of the contralateral lung, when tolerated, greatly facilitates exposure. We prefer autologous pericardial tube unifocalization of aortopulmonary collaterals and true pulmonary arteries.
Pulmonary Atresia with Ventricular Septal Defect and Major Aorta-to-Pulmonary Artery Collaterals
Patients with this complex lesion sometimes survive to adulthood without surgery because of adequate pulmonary blood ow from collaterals. Others have shunts or unifocalization procedures or complete repairs.135140 Anatomy The true pulmonary arteries may be absent, hypoplastic, and continuous or discontinuous. The collaterals may be the dominant or only blood supply to the lung or supply only lesser areas of the lung. The VSD is subaortic and usually single. Depending on previous shunts, there may be stenoses in the proximal or distal pulmonary arteries. If repaired there may be a residual VSD, obstruction between the right ventricle and PA, and tricuspid valve regurgitation. Physiology Patients who have not had repair and who are considered well-balanced have a saturation of 80 to 84%. These patients with complete mixing have a left-to-right shunt of between 1.5:1 and 2:1. Therefore, they all have a volumeoverloaded heart. The volume overload results in reduced exercise tolerance. Because of high ow and elevated pressure, they may have developed increased pulmonary vascular resistance in the area of some of the collaterals. The aorta and aortic valve tend to dilate, and 50% develop aortic valve regurgitation. Ventricular dilatation and dysfunction can also occur. Preoperative evaluation Echocardiography is used to exclude additional VSDs and to evaluate the aortic valve. Angiography is performed to delineate the collaterals and true pulmonary arteries, as well as to evaluate transpulmonary gradient, which may be high if there was uncontrolled large collateral ow hypertension.
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Figure 61-11. Unifocalization of the pulmonary artery blood supply to the right lung using a pericardial tube. Through a lateral thoracotomy a side-to-side anastomosis is created to each major collateral and an adjacent incision made in the autologous pericardium placed behind the lung. The pericardium is turned into a tube by suturing the edges and the collaterals are ligated proximal to the tube.The posteriorly lying tube is extended by a 16-mm PTFE graft to the anterior mediastinum. A 6mm PTFE shunt is made between the subclavian artery and the 16-mm PTFE extension.
Figure 61-12. A 19-year-old patient previously underwent left pericardial tube unifocalization to three collaterals at age 18 years. On the right side there was a single collateral to a large pulmonary artery supplying the entire right lung. Right-sided unifocalization was achieved by ligating the collateral and placing a 20mm PTFE graft from the right pulmonary artery to the ascending aorta, creating a central restrictive anastomosis.
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Figure 61-13. The complete repair performed 6 months after the right unifocalization shown in Fig. 6112. The ventricular septal defect was closed. An aortic homograft conduit was placed between the right ventricle and the left unifocalization. The right unifocalization was then connected to the homograft by a 16-mm reinforced PTFE tube placed behind the aorta.
Finally, denitive repair in this disorder entails patch closure of the anterior malaligned ventricular septal defect and establishment of continuity between the right ventricle and the pulmonary arteries. All systemic-to-pulmonary artery shunts, including redundant collaterals and surgically created shunts, have been previously occluded or are readily accessible from the anterior mediastinum for occlusion at the time of denitive biventricular repair. Measurement of the ratio of right ventricle-to-left ventricle systolic pressure allows intraoperative assessment of the repair. A ratio of 0.75 or less immediately after termination of cardiopulmonary bypass is acceptable, and the ratio can be expected to decrease in the rst few days after operation. Higher ratios suggest inadequate pulmonary runoff and will likely result in right ventricular failure. If the pressure on the right side is near systemic or suprasystemic, perforation of the ventricular septal defect patch may provide survival and reasonable palliation. Outcomes From 1983 through 2000, 105 children and adults have presented to our institution with pulmonary atresia, ventricular septal defect, and multiple aorta-to-pulmonary artery collaterals. All patients were subject to a strategy of staged repair.
Sixty-four patients in this cohort underwent palliation in the newborn period at a median age of 1 week. Surgical palliation included systemic-to-pulmonary artery shunts, right ventricular outow patches, and banding of aorta-to-pulmonary artery collaterals to reduce high pressure and ow. Interventional cardiac catheterization procedures were performed to promote growth of the pulmonary arteries as necessary. Ninety-four patients underwent unifocalization at a median of 3.5 years (range, 6 months to 37 years). Fifty-eight (range, 1 to 34 years) of these 94 patients have proceeded to complete repair at a median of 7.2 years. Unifocalization was performed in 19 adults, and of this group, 8 patients have undergone uneventful complete repair. There was neither mortality nor signicant morbidity in this group. At a median follow-up of 60 months there were a total of 18 deaths for a 17% early and late mortality rate. Survival after initial palliation was 92%, after unifocalization 91%, and after complete repair 91%. There were 36 reoperations (12%) and 16 patients required catheter-based interventions after surgery. The mean right ventricle-to-left ventricle pressure ratio was 0.46. Nearly all the survivors are asymptomatic and do not exhibit any signs of exercise intolerance. Although there is some debate about whether a onestage repair is preferable in neonates and children, patients
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presenting as adults, with or without prior palliation, may be excellent candidates for the staged approach described above. We prefer a staged approach to one-stage correction in adults whose predominant blood supply to the lungs is from collaterals. In adults with a predominant blood supply from the true pulmonary arteries a one-stage repair may be utilized. The strategy of staged repair for patients with tetralogy of Fallot with major aorto pulmonary collaterals provides good functional results. The mortality rate and requirements for postoperative interventional cardiac catheterization with this approach are lower than published reports of single-stage repair. As patients age and mature they will require reoperations to replace right ventricle-to-pulmonary artery homografts and degenerative bioprostheses in the pulmonary position. Long-standing pressure and volume load on the right ventricle will lead to tricuspid regurgitation and right atrial enlargement that may predispose some patients to atrial arrhythmias.
Preoperative evaluation Transthoracic or transesophageal echocardiography usually delineates the site of systemic or pulmonary venous obstruction, ventricular function, and valvular regurgitation. In some cases angiography is also required. Magnetic resonance imaging has become a sensitive tool for assessing ventricular function and can be used to follow patients undergoing ventricular retraining. This allows for a rapid, noninvasive assessment of ventricular function during sequential banding so that the band can be loosened in the face of left ventricular failure.144 Operative procedure Reoperation for obstruction of either systemic veins or pulmonary veins can almost always be accomplished by incision of the site of obstruction and patching using a pericardial patch when it is available. Usually, with repair of the caval part of the bafe, the functional left atrium is also enlarged. For patients who present with right ventricular dysfunction and tricuspid valve regurgitation, the choice of therapy is more complex. If the major problem is tricuspid valve regurgitation in the presence of relatively well-preserved right ventricular function, we prefer to repair or replace the tricuspid valve. The results of tricuspid valve repair or replacement in suitable patients are generally good. Care must be taken to avoid conduction tissue that is very vulnerable at the junction of the septal and anterior leaets. If right ventricular function is signicantly depressed, the left ventricular function and the left ventricular outow tract (LVOT) and pulmonic valve are evaluated. If left ventricular function is good and there is no xed LVOT obstruction or pulmonic stenosis, the patient may be considered for left ventricle preparation and the arterial switch procedure. Preparation of the left ventricle requires PA banding to a pressure of 60 to 70% of systemic pressures initially and then delayed rebanding to systemic pressures. It should be noted that retraining the left ventricle in the mature heart is a longer process than in the neonate, and that there is less margin for error when placing a band in a fully septated heart. Six months to a year may be required to achieve this and to obtain a normal left ventricular wall thickness. Once this is achieved, the arterial switch operation may be performed. The atrial bafe is removed, and a new atrial septum is constructed in the anatomic position (Fig. 61-14). In adults, left ventricle preparation by successive tightening of a pulmonary artery band can be hazardous and ultimately unsuccessful with the onset of left ventricular failure. The outcomes from this approach have been quite variable and the overall experience is limited. If signicant left ventricular dysfunction or xed LVOT obstruction is identied, the patient may be considered for heart transplantation. Results The outcomes from reoperations to repair bafe obstructions in patients with previous Mustard and Senning procedures are quite good if the systemic right ventricular function is
Late Reoperations for Transposition of the Great Arteries

Anatomy and natural history Dextraposed transposition of the great arteries is characterized by atrioventricular concordance and associated ventriculoarterial discordance. Prior to the introduction of the arterial switch procedure in 1982, the Mustard and Senning procedures were the standard operations for dextraposed transposition. In the Mustard procedure, a pericardial bafe was used to redirect the systemic and pulmonary venous return. In the Senning procedure, the atrial septum and wall were used for the bafe. This allowed deoxygenated blood to be pumped to the pulmonary circulation and the oxygenated blood to be pumped to the systemic circulation. As a consequence of these operations, such patients have the morphologic right ventricle acting as the systemic ventricle, and the natural history of such anatomy is well documented. There is about 70 to 80% survival at 20 years; 10% of patients have symptomatic right ventricular dysfunction, and about 60% have dysfunction that becomes evident at exercise testing. Additionally, atrial arrhythmias are common, and many patients are in junctional rhythm at 10 years of follow-up. With either procedure, bafe obstruction can lead to a high incidence of vena caval obstruction or pulmonary venous obstruction requiring reoperation.141,142 Eventual morphologic right ventricular failure in these patients will require either transplantation or a staged procedure with left ventricular reconditioning and arterial switch. Sudden death has been identied as the most common cause of mortality in patients having undergone an atrial switch procedure.143 Most sudden death events occurred during exercise. Predictors of sudden death were found to be documented atrial brillation/utter and the presence of symptomatic arrhythmias or heart failure. Electrocardiogram, chest x-ray, and Holter monitoring were not predictive.
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venous deoxygenated blood within the ventricle. Pulmonary blood ow is supplied by either a true pulmonary artery (PA), a patent ductus arteriosus, systemic-to-pulmonary collaterals, or a surgical shunt. The PA may have pulmonary or subpulmonary stenosis or may have been surgically banded. With complete intracardiac mixing, pulmonary blood ow must be approximately 1.5 times the systemic blood ow (Qp:Qs 1.5), to achieve a systemic arterial oxygen saturation of 80 to 85%. This increased pulmonary blood ow results in a volume overload on the ventricle and the aorta. The clinical manifestations in patients with any form of single ventricle are directly related to the pulmonary blood ow and pulmonary artery pressure. These will be determined by the presence or absence of pulmonary stenosis and the pulmonary vascular resistance. Of note, over half of these patients will have some form of pulmonary stenosis or atresia.
Figure 61-14. Takedown of a Mustard bafe. After opening the anatomic right (functional left) atrium, the four pulmonary veins surrounded on three sides by the Mustard bafe are visible. The illustrated incision enters the atrial chamber that receives systemic venous blood, and when completed will expose both caval-atrial junctions and the four pulmonary veins entering a common atrial chamber. For venous return to the perfusion circuit, the superior and inferior venae cavae can be cannulated directly or via peripheral venous cannulas.
preserved.141,142,145157 Generally adolescents and adults are highly variable in their response to a staged conversion to an arterial switch procedure.148,153 This is a critical point since few patients have undergone atrial switch procedures for transposition in the last 20 years, and most new patients will be in the third decade of life and later. While no age cutoff has been identied, attempts to identify risk factors indicate that complex coronary anatomy, severe morphologic right ventricular failure, and supraventricular arrhythmias result in worse outcomes. In these patients cardiac transplantation as an initial therapy may be the preferred course.
Indications for surgery There are a few patients reported in the literature with single ventricle who have survived to adulthood without surgical intervention.23 Most patients will require intervention because of too much pulmonary blood ow, causing heart failure, or too little pulmonary blood ow, causing cyanosis. Patients are stratied according to their pulmonary artery pressure, pulmonary vascular resistance (PVR), ventricular function, and anatomic complexity into low-, medium-, and high-risk candidates for a Fontan procedure. In mediumand high-risk patients with elevated PA pressure, PVR, and impaired ventricular function, a bidirectional Glenn shunt is performed as a rst stage to a Fontan procedure or as longterm palliation until a heart transplant may be considered. Glenn shunt
PHYSIOLOGY:
Single Ventricle
Anatomy Single ventricle is a term that describes various forms of congenital heart disease characterized by malformation and hypoplasia of one ventricular chamber, resulting in a single functioning ventricle. The specic defects include tricuspid atresia, mitral atresia, unbalanced atrioventricular canal defects, severe forms of pulmonary atresia with intact ventricular septum, hypoplastic left heart syndrome, double inlet left and right ventricles, and absent ventricular septum (Holmes heart). Malposition or transposition of the great arteries is present in many of these patients. Physiology All of these patients have single-ventricle physiology with initial mixing of the systemic oxygenated blood and the
By connecting the end of the superior vena cava (SVC) to the superior aspect of the right PA, about one-third of the systemic venous return is diverted to the lungs for oxygenation. This is a more efcient shunt than a systemic-to-PA shunt and does not cause a volume overload on the ventricle. It is therefore better tolerated in the presence of impaired ventricular function. Provided the SVC pressure is 18 mm Hg or less, the elevation in SVC pressure is well tolerated.
As shown in Fig. 61-15, the Glenn shunt is performed without cardiopulmonary bypass in most patients by using an SVC-to-PA shunt. If additional procedures are required, such as the relief of subaortic obstruction, atrial septectomy, or atrioventricular valve repair, an open procedure on cardiopulmonary bypass is required. An additional source of pulmonary blood ow aims for a pulmonary-to-systemic blood ow ratio of 1 to 1.3, depending on the PVR. Either a banded pulmonary artery or a small systemic-to-PA shunt is used. The additional source of blood ow improves oxygenation at rest and with exercise, and may prevent late arteriovenous stula development in the lungs.
OPERATIVE PROCEDURE:
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Figure 61-15. Creation of a bidirectional Glenn shunt (superior vena cava to right pulmonary artery) using an extracorporeal shunt (with systemic heparin) to maintain superior vena caval ow into the right atrium. A deairing chamber and port are needed to prevent air entry into the heart.
OUTCOMES: The early mortality for an isolated Glenn shunt is low (1 to 4%), depending on factors such as the PVR and ventricular function.158161 Additional intracardiac procedures increase the risk. In the long term the Glenn shunt slowly loses its effectiveness due to the development of venous collaterals from the superior vena cava to the inferior vena cava.158,160,161 These can be coil embolized by catheter technique. If there is no additional source of pulmonary blood ow, there is usually severe desaturation on exercise and systemic-to-PA collaterals develop over time. Intrapulmonary arteriovenous stula can also develop in this situation, resulting in desaturation. In patients with no additional source of pulmonary blood ow and with severely impaired ventricular function, oxygenation can be improved with a controlled shunt by performing an axillary artery-to-vein stula.162
mm Hg, and no additional severe hemodynamic lesions that require prior attention, such as residual coarctation of the aorta, severe subaortic obstruction, or severe atrioventricular valve regurgitation. Generally, correctable lesions should be addressed at the time of the Glenn shunt or before completion of the Fontan procedure.
OPERATIVE PROCEDURE:
Modied Fontan procedure

INDICATIONS:
Because of the limited palliation provided by the Glenn shunt, patients who meet hemodynamic criteria should be considered for a Fontan procedure. The criteria include good ventricular function, ejection fraction 50% or higher, normal or close-to-normal PVR, PA pressure 20
Many different modications of the Fontan procedure have evolved to connect both the superior and inferior vena caval blood to the pulmonary arteries. Currently, the two most commonly performed operations are the lateral tunnel Fontan (Fig. 61-16) and the extracardiac Fontan (Fig. 61-17). The lateral tunnel has the advantage of not requiring warfarin anticoagulation in the majority of cases, and a fenestration can be easily included in the procedure. The extracardiac Fontan can be done without arresting the heart and is therefore associated with improved postoperative ventricular function. It can be fenestrated with a separate small shunt from the conduit to the atrium. Treatment with warfarin anticoagulation is indicated for at least 1 year and possibly for life to reduce the incidence of conduit thrombus. Because of the extensive atrial suture lines, arrhythmias and sick sinus syndrome may be more common with the lateral tunnel Fontan. A newer modication of the
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Figure 61-16. A lateral tunnel Fontan operation enlarges the right atrium and directs inferior caval ow into the right pulmonary artery using an end-to-side anastomosis to provide bidirectional ow into both pulmonary arteries. A snared purse-string suture adjusts the size of an atrial septal opening that is used to decompress caval pressures and to control the amount of right-to-left shunting.
extracardiac Fontan using bovine pericardium and the native atrial wall has recently been reported.1
OUTCOMES: The early mortality of the Fontan procedure in adults depends on selection of patients and the presence of risk factors. Reported series have an early mortality of 5%.163,9,21 Fontan patients may be candidates for pacemakers (10%) and reoperations for valvular (5%) and obstructive problems, and may require transplantation for deteriorating ventricular function.164 Protein-losing enteropathy can occur in 5 to 10% of patients.163,165,166
Late Reoperations after Modied and Classic Fontan Procedures

Surgical reintervention following a Fontan procedure may be indicated for arrhythmias, cyanosis, exercise intolerance, protein-losing enteropathy, atrioventricular valve regurgitation, or failing Fontan circulation. Surgical procedures include pacemaker insertion, revision of Fontan, fenestration of Fontan, Maze procedure, repair or replacement of the atrioventricular valve, and heart transplantation.8 Patients who have had a right atrial-to-PA connection may develop massive right atrial enlargement.164,165,166 This can eventually result in atrial arrhythmias, thrombus formation, and thromboembolism. Supraventricular arrhythmias as well as atrioventricular node dysfunction are common, and preoperative evaluation by an electrophysiologist is usually
indicated. When the right atrium is enlarged, warfarin anticoagulation is indicated. Such patients should be considered for reoperation with conversion to either a lateral tunnel or to an extracardiac conduit with reduction of the enlarged right atrium, and a right-sided Maze procedure has resulted in improved NYHA functional class and reduced incidence of arrhythmias.7,22,24,25,167 Many will require a permanent pacemaker. If they do not meet the criteria for Fontan conversion, they could be considered candidates for a heart transplant. The long-term results after modied Fontan procedures have shown that a signicant number of patients require late reoperation. The highest incidence was for patients in whom a valved conduit was used.168,169 Older age at operation remains a risk factor for late death after the Fontan procedure. Revision of the atrioventricular valve closure in double-inlet ventricles or repair of the atrioventricular valve in tricuspid atresia is needed in more than 5% of patients. Adult patients with systemic single right ventricles have signicant risk for ventricular dysfunction with congestive heart failure and increased mortality.26 In addition to arrhythmias and reoperations, adult Fontan patients may also suffer from clinically silent pulmonary emboli and thromboembolic events that may reduce the quality of life and have severe long-term consequences.10,27 Protein-losing enteropathy occurs in about 10% of Fontan patients. It is characterized by a low serum albumin level, persistent ascites, and peripheral edema with or without
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Figure 61-17. Extracardiac Fontan with adjustable conduit-to-atrial connection.
diarrhea. It may be associated with a mortality up to 20%.163,164,166 It occurs more frequently in patients with heterotaxia or polysplenic syndromes, as well as in those with elevated pulmonary vascular resistance or abnormal systemic venous drainage. Some of these patients can be helped by transcatheter fenestration of the atrial septum or Fontan conduit with stenting.2 If they do not meet criteria for a Fontan revision, they should be evaluated for transplantation.170177 In many patients, protein-losing enteropathy will resolve following successful heart transplantation.
Ebstein Anomaly
Ebstein malformation is a rare congenital cardiac defect accounting for less than 1% of all congenital heart disease. The primary pathologic nding is an abnormal development of the tricuspid valve marked by a downward displacement of the septal and posterior leaets into the cavity of the right ventricle. This defect is characterized by a remarkable morphologic variability and a broad spectrum of clinical presentations. Consequently, the diagnosis may be made in symptomatic newborn infants, in children, or in adults.178,179 The degree of tricuspid regurgitation varies depending on the anatomic abnormality. If there is an atrial septal defect,
patients may present with cyanosis. If the atrial septum is intact, they may present with cardiomegaly, right-sided heart failure, or arrhythmias. Those patients with atrial or ventricular arrhythmias may present with episodes of syncope, near syncope, or recurrent palpitations. Paroxysmal supraventricular arrhythmias occur in 25 to 40% of patients and are most often found in teenagers or young adults.180 Aberrant right atrial to right ventricular tracts resulting in tachycardia (Wolff-Parkinson-White syndrome) occur in 10 to 18% of patients.181 Sudden death due to ventricular arrhythmias may occur in as many as 5 to 7% of patients. Likewise, patients with mild manifestations of the Ebstein malformation may present as late as the third or fourth decade of life with complaints of palpitations or mild exercise intolerance.182,183 While some patients may reach advanced age without serious clinical manifestations, most will eventually develop signicant symptoms.184 The most common causes of death are congestive heart failure, severe hypoxia, and cardiac arrhythmias.185 Anatomy Ebstein malformation is dened by a downward displacement of the annular attachments of the septal and posterior
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leaets of the tricuspid valve into the inlet portion of the right ventricle.186 This downward displacement of the leaets reduces the distal chamber of the right ventricle, leaving part of the ventricle above the valve as an extension of the right atrium. The atrialized right ventricle is variable in size and thickness, depending on the extent of downward displacement of the leaets. The entire wall of the right ventricle, both above and below the tricuspid valve, is often thin, dilated, and dysfunctional. In most patients, annular dilatation and malformation of the leaets result in moderate to severe insufciency of the tricuspid valve. It usually occurs in the pulmonary right ventricle, but can occur in the systemic right ventricle in patients with corrected transposition of the great arteries. An atrial septal defect or patent foramen ovale is present in more than 50% of patients, allowing predominantly right-to-left shunting at the atrial level. Physiology In patients with Ebstein malformation, the tricuspid valve is incompetent, but the degree varies depending on the anatomic features. In addition, there is some degree of functional impairment of the right ventricle. The atrialized right ventricle moves paradoxically with right atrial and right ventricular contractions. The net effect is reduced forward blood ow through the right ventricle and pulmonary arteries. The impaired lling of the functional right ventricle and the incompetence of the tricuspid valve both result in systemic venous hypertension. The right atrium and the atrialized right ventricle become dilated, often to extreme degrees. In patients with atrial septal defects, right-to-left shunting occurs, resulting in cyanosis. Both atrial and ventricular arrhythmias may contribute to impaired right ventricular function. Although the primary pathology involves the right ventricle, patients with Ebstein malformation may also demonstrate abnormal left ventricular geometry and function. The severity of left ventricular dysfunction is associated with the degree of displacement of the tricuspid valve, the size and dysfunction of the right ventricle, and the severity of paradoxical motion of the interventricular septum. Preoperative evaluation On a chest radiograph, the right border of the heart in the area of the right atrium is enlarged and there may be massive cardiomegaly. Typically, the shadow of the great vessels is narrow due to a small aorta and main pulmonary artery. Right atrial and right ventricular enlargement produce a globular shape to the heart shadow. The apical region of the left ventricle may be elevated from the diaphragm, as seen in right ventricular enlargement. Pulmonary vascularity may range from normal to signicantly decreased in the presence of an ASD. A cardiothoracic ratio greater than 0.65 has been shown to be a predictor of sudden death and is considered by some to be an indication for surgery. Echocardiography has evolved as the primary diagnostic tool for patients with Ebstein malformation. The preoperative
echocardiogram is helpful in predicting the ability to repair the valve. Echocardiography can dene the morphology of the tricuspid valve and the specic abnormalities of the leaets. Of the greatest importance are the length and mobility of the anterior leaet. In addition, the function, thickness, and size of the right and left ventricles can be assessed. Coexisting cardiac lesions can also be identied. Color ow Doppler allows a better assessment of tricuspid valve incompetence and the degree of shunting at the atrial level.187 If echocardiography is inadequate, magnetic resonance angiography imaging with three-dimensional reconstruction is useful for diagnostic purposes. Diagnostic cardiac catheterization should be avoided, as it is usually unnecessary and can result in arrhythmias. Currently, cardiac catheterization is reserved for patients with associated cardiac defects, previous shunt placements, possible pulmonary artery stenosis, or possible coronary artery disease. Therapeutic catheterization may be performed in older patients for identication and ablation of accessory conduction pathways prior to surgical intervention. Indications for surgery The indications for surgical intervention in patients with Ebstein malformation include the following: functional NYHA class III or IV symptoms; signicant or progressive cyanosis; decline in exercise tolerance; severe cardiomegaly (cardiothoracic ratio 0.65); associated cardiac anomalies (including right ventricular outow tract obstruction); refractory atrial or ventricular arrhythmias; and a history of paradoxical embolus. With the improved outcomes and a greater ability to repair the valve, there is a trend to perform early repair in the presence of severe tricuspid regurgitation and atrial enlargement. Operative procedure The goals of surgical intervention in patients with Ebstein malformation are to increase pulmonary blood ow, minimize tricuspid insufciency, reduce or eliminate right-to-left shunting, optimize right ventricular function, and reduce or eliminate arrhythmias. Ideally, the tricuspid valve can be repaired, which may avoid valve replacement with a bioprosthetic valve and the need for future valve replacements. Patients with preoperative Wolff-Parkinson-White syndrome are treated by catheter ablation prior to the surgery. If the anterior leaet is adequate in size and is not extensively bound down by muscular attachments, repair is almost always possible. Two main techniques of repair have been described. Danielson and colleagues were the rst to demonstrate the ability to repair these valves and avoid replacement.188 Repair includes plication of the atrialized right ventricle back to the true annulus and an annuloplasty (Fig. 61-18). Carpentier and associates described a technique in which the atrialized right ventricle is plicated perpendicular to the valve annulus toward the apex of the heart.189 The displaced leaets are detached from the right ventricle at their
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Figure 61-18. Danielsons repair of the tricuspid valve in Ebstein anomaly.
base and attached to the true annulus (Fig. 61-19). We perform an annuloplasty using a glutaraldehyde-xed strip of pericardium. The redundant right atrium wall and appendage are excised, and the ASD is closed. In patients with a large preoperative right-to-left shunt and a thinned-out underdeveloped right ventricle, a snare-controlled adjustable atrial septal defect may be used to allow continued controlled right-to-left shunting until the right ventricle recovers. The ASD can then be closed using the snare, which is exposed under local anesthesia. In addition, electrophysiologic mapping for localization of accessory pathways may be performed in patients with arrhythmias. A recently described technique of posterior annular plication without plication of the atrialized right ventricle or repositioning the tricuspid valve has been successful in
non-neonatal patients.2 Further experience and follow-up will be needed to determine the usefulness and efcacy of this type of tricuspid repair in adults with Ebstein anomaly. For tricuspid valve replacement, we prefer to use a porcine bioprosthetic valve. In the absence of atrial arrhythmias or severe atrial wall thickening, this allows anticoagulation with aspirin only. Generally, tissue valves are preferred in the tricuspid position because of the risk of thrombosis of a right-sided mechanical valve in a low-pressure setting. The right atrial Maze procedure is a modication of the Maze procedure and has been used to treat atrial arrhythmias in patients with Ebstein malformation. This procedure may reduce or eliminate atrial arrhythmias by preventing reentry conduction at the atrial level (Fig. 61-20).
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Figure 61-19. Carpentiers repair of the tricuspid valve in Ebstein anomaly.
Outcomes Results of surgical repair for Ebstein anomaly in children and adults continue to improve. Patients who present at a younger age with associated defects or severe symptoms have a worse prognosis. Those patients presenting in early adulthood for surgical repair have a much better prognosis. Danielson and colleagues at the Mayo Clinic currently have a surgical experience of more than 500 patients with Ebstein malformation.190 The data have been analyzed for the rst 312 patients who had surgical intervention dating back to 1972. The ages in this series range from 9 months to
71 years, with a mean age of 20.7 years. There were no neonates in this group. In 43% a tricuspid valve repair was successful, and in 53% a bioprosthesis was used to replace the tricuspid valve. Approximately 4% of patients underwent a Fontan reconstruction or other procedures. There were 20 hospital deaths (6.4% early mortality) in this series. Forty-four patients had accessory conduction pathways (Wolff-Parkinson-White syndrome) and underwent successful pathway ablation as part of their repair. Fifteen patients underwent right-sided Maze procedures for control of atrial dysrhythmias and four underwent ablation of the atrioventricular
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Figure 61-20. Injury to the conduction system may be avoided during tricuspid valve replacement by suturing a triangular patch of pericardium over the atrioventricular (AV) node and triangle of Koch.
node for re-entry tachycardia. There were 24 late deaths (7.3%). Seventeen of the 135 patients (12.6%) who underwent valve repair required reoperation for valve regurgitation 1.5 to 18 years later (mean, 8.7 years). Eight bioprosthetic valves required replacement 1 to 16 years after implantation. Follow-up of those patients evaluated more than a year after operation determined that 93% were in NYHA functional class I or II. Other authors have recently reported similar results using different repair techniques in a smaller series of patients.191,192 The addition of a right atrial Maze procedure to the repair is often successful in reducing or eliminating atrial arrhythmias. Furthermore, the addition of epicardial mapping and intraoperative ablation of accessory pathways at the time of repair has also been successful.1,7,193,194 The durability of a porcine bioprosthesis for tricuspid valve replacement in these patients has been quite favorable, with freedom from reoperation of 97% at 5 years and 81% at 15 years.195
systemic and pulmonary venous return can be corrected. Deformities of the pulmonary artery can be repaired. This may require quite extensive repair, particularly in some patients after the Fontan procedure. Dextrocardia and transposition may be challenging, but with current reconstructive techniques, transplantation is almost always possible.204208,216,217 Preoperative evaluation General evaluation is as for all heart transplant recipients. Cardiopulmonary exercise testing, and pulmonary, dental, and psychosocial evaluations are routine. Pulmonary vascular resistance must be carefully assessed; the cutoff is at 4 to 6 Wood units or a transpulmonary gradient of 12 to 14 mm Hg. Panel-reactive antibody screening is essential, as most patients have had previous surgery and blood transfusions. If these values are higher than 10%, then prospective crossmatching is preferable. Chest wall collaterals may increase left-sided return, particularly after previous surgery or cyanosis, and therefore it is usual to oversize donors by 20%. Some larger aortopulmonary or veno-venous collaterals may be coil embolized preoperatively.218 Renal and liver function and reserve require careful evaluation. Renal function may be compromised due to a low-output state, as well as renal vein congestion from elevated central venous pressure. Ideally, pretransplant creatinine clearance should be at least 40 mL/min.219 Cardiac hepatopathy is poorly understood. Our experience is that these patients are at high risk for liver dysfunction due to frequent right ventricular failure or Fontan physiology. Many of these potential recipients will have normal liver function tests but abnormal liver histology. Preoperative histology may be useful to rule out cirrhosis. Centrilobular brosis may be seen on occasion.220,221 Our experience has revealed that noninvasive imaging may underestimate the possibility
Heart Transplantation
Adults with congenital heart disease may not be amenable to palliation or repair due to severe ventricular dysfunction or pulmonary vascular disease. They may be candidates for heart, lung, or heart-lung transplantation. In addition, patients previously repaired may deteriorate and may have no other options.196211 Other reparative options are always considered prior to choosing heart transplantation as a surgical treatment. In this regard, there have been several reports of one and onehalf ventricle repairs in cases of right ventricular pathology.212215 In symptomatic patients who are NYHA class III or IV and who have an acceptable pulmonary vascular resistance, heart transplantation is usually feasible. Anomalies of
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of cardiac cirrhosis. Even though this is rare, it is an important contraindication to heart transplantation. Two to three hours of cardiopulmonary bypass time will unmask poor liver reserve. This is especially true in the third decade of life. Postoperatively, one may observe increasing total bilirubin with minimal rise in other tests of liver function. Total bilirubin may rise as high 35 to 40 mg/dL before returning to normal levels over a period of as many as 3 to 4 weeks.222 Such a clinical course may be explained by a compromised liver preoperatively, to which is added an ischemic insult. Such livers require high ows during cardiopulmonary bypass and are thought to poorly tolerate hypothermia.223 As described below, this may not always be possible. Operative procedure It is important to anticipate the recipient anatomy. For example, in tricuspid atresia dextraposed transposition is common, and the aorta may be immediately behind the sternum. A computed tomography scan is obtained preoperatively to assess the retrosternal structures. It is rarely necessary to institute bypass before opening the sternum. Aprotinin is used in all cases and it is important to plan correct timing for the arrival of the donor heart, since redo sternotomy in these patients is more complex than usual. Meticulous hemostasis is essential prior to starting cardiopulmonary bypass, as postoperative coagulopathy is common. If needed,
patients are cooled to 22 to 24C in order to minimize the pulmonary venous return, which can be torrential. This can warm the donor heart, and after the aortic anastomosis is completed, may wash out the preservation solution if the aortic root is not vented. A vent in the left atrium is therefore used to collect this return. Anomalies in systemic or pulmonary venous return and the pulmonary arteries are reconstructed prior to bringing the donor heart onto the eld. We apply intracardiac cooling to the left ventricle using a catheter passed through the left atrial anastomosis into the left ventricle apex. Generally, we prefer a bicaval anastomosis (Figs. 61-21 and 61-22). Outcomes Due to previous surgeries, anatomic complexity, borderline pulmonary vascular resistance, hepatic congestion, and other factors, these patients (particularly the Fontan patients) are at higher risk for transplantation. Between 1984 and 2002, 30 adults and adolescents with congenital heart disease have undergone transplantation at our institution. Close to 50% of the patients had single-ventricle physiology, and on average all had had at least two previous sternotomies. Age range was 13 to 49 years old. The early mortality for this high-risk group was 18%. The single-ventriclephysiology patients are probably the highest-risk recipients. Quaegebeur, Addonizio, and Hsu and colleagues have reported a cohort of 35 recipients with 28% early mortality.
Figure 61-21. Heart transplantation after total cavopulmonary connection in a patient with bilateral superior venae cavae. The innominate vein from the donor is used to reconstruct the left superior vena cava. Alternatively (not shown), if the left superior vena cava is too short, a synthetic graft can be used to route the left superior vena caval blood along the coronary sinus of the donor heart into the recipient native inferior vena cava.
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8. Durack DT: Prevention of infective endocarditis. N Engl J Med 1995; 332:38. 9. Freed MD: Infective endocarditis in the adult with congenital heart disease. Cardiol Clin 1993; 11:589. 10. Perloff JK, Marelli AJ, Miner PD: Risk of stroke in adults with cyanotic congenital heart disease. Circulation 1993; 87:1954. 11. Brili SV, Barberis VI, Karamitros IA, et al: Mild cyanosis due to coexistence of congenitally corrected transposition of the great arteries and Gerbode-type defect. Cardiology 2006; 105:41. 12. Simko LC, McGinnis KA: What is the perceived quality of life of adults with congenital heart disease and does it differ by anomaly? J Cardiovasc Nurs 2005; 20:206. 13. Suds K, Matsumura M, Matsumoto M: Shunt obstruction after air travel in an adult patient with cyanotic congenital heart disease. Cardiology 2005; 103:142. 14. Siegel MJ, Bhalla S, Gutierrez FR, et al: MDCT of postoperative anatomy and complications in adults with cyanotic heart disease. AJR Am J Roentgenol 2005; 184:241. Review. 15. Mavroudis C, Deal BJ, Backer CL: Surgery for arrhythmias in children. Int J Cardiol 2004; 97(Suppl 1):39. 16. Walker F, Mullen MJ, Woods SJ, et al: Acute effects of 40% oxygen supplementation in adults with cyanotic congenital heart disease. Heart 2004; 90:1073. 17. Rogozinska-Zawislak A, Kozak J: [Congenital pulmonary arteriovenous stula coexisting with atrial septal defect. A case report]. Kardio Pol 2004; 60:63. Polish. 18. Berdat PA, Immer F, Pfammatter JP, et al: Reoperations in adults with congenital heart disease: analysis of early outcome. Int J Cardiol 2004; 93:239. 19. Pekdemir H, Gokhan Cin V, Necdet Akkus M, et al: Cyanotic tetralogy of Fallot with its infective endocarditis complication on the tricuspid and pulmonary. Circ J 2004; 68:178. 20. Ullrich NJ, Urion DK: Transient global amnesia in a young adult with cyanotic heart disease. Pediatr Neurol 2003; 29:334. 21. Rao V, Van Arsdell G, David T, et al: Aortic valve repair for adult congenital heart disease: a 22-year experience. Circulation 2000; 102:III40. 22. Overgaard C, Harrison D, Siu S, et al: Outcome of previous tricuspid valve operation and arrhythmias in adult patients with congenital heart disease. Ann Thorac Surg 1999; 68:2158. 23. Sabet H, Edwards W, Tazelaar H, Daly R: Congenitally bicuspid aortic valves: a surgical pathology study of 542 cases (1991 through 1996) and a literature review of 2,715 additional cases. Mayo Clin Proc 1999; 74:14. 24. Van Nooten GJ, Caes F, Tacymarrs Y, et al: Tricuspid valve replacement: postoperative and long-term results. J Thorac Cardiovasc Surg 1995; 110:672. 25. Milgalter E, Laks H: Use of a pericardial patch to bridge the conduction tissue during tricuspid valve replacement. Ann Thorac Surg 1991; 52:1337. 26. Kuckarczuk J, Cohen M, Rhodes L, et al: Epicardial atrial pacemaker lead placement after multiple cardiac operations. Ann Thorac Surg 2001; 71:2057. 27. Cohen M, Vetter V, Wernovsky G, et al: Epicardial pacemaker implantation and follow-up in patients with a single ventricle after the Fontan operation. J Thorac Cardiovasc Surg 2001; 121:804. 28. Ramesh V, Gaynor J, Shah M, et al: Comparison of left and right atrial epicardial pacing in patients with congenital heart disease. Ann Thorac Surg 1999; 68:2314. 29. Epstein M, Walsh E, Saul J, et al: Long-term performance of bipolar epicardial atrial pacing using an active xation bipolar endocardial lead. Pacing Clin Electrophysiol 1998; 21:1098. 30. Roberts AD, Sett S, Leblanc J, Sanatani S: An alternate technique to pacing in complex congenital heart disease: assessment of the left thoracotomy approach. Can J Cardiol 2006; 22:481. 31. Chintala K, Forbes TJ, Karpawich PP: Effectiveness of transvenous pacemaker leads placed through intravascular stents in patients with congenital heart disease. Am J Cardiol 2005; 95:424.
Figure 61-22. Heart transplant in a patient with dextrocardia, right-sided arch, and interrupted inferior vena cava with azygos continuity who previously had undergone a Fontan procedure. Ao aorta; Az azygos vein; HV hepatic vein; IVC inferior vena cava; PA pulmonary artery; RA right atrium; SVC superior vena cava.
Similar results have been reported by Dark and associates. The group in Bergamo has reported a survival of 86% for heart transplantation in patients with previous Fontan operations.224226
References
1. Moodie D: Diagnosis and management of congenital heart disease in the adult. Cardiol Rev 2001; 9:276. 2. Brickner M, Hillis L, Lange R: Congenital heart disease in adults. N Engl J Med 2000; 324:256. 3. Webb CL, Jenkins KJ, Karpawich PP, et al: Collaborative care for adults with congenital heart disease. Circulation 2002; 105:2318. 4. Therrien J, Webb G: Clinical update on adults with congenital heart disease. Lancet 2003; 362:1942. 5. Chessa M, Cullen S, Deaneld J, et al: The care of adult patients with congenital heart defects: a new challenge. Lancet 2003; 362:1305. 6. Deaneld J, Thaulow E, Warnes C, et al, and the Task Force on the Management of Grown Up Congenital Heart Disease, European Society of Cardiology: Management of grown up congenital heart disease. Eur Heart J 2003; 24:1035. 7. Perloff JK: Congenital Heart Disease in Adults. Philadelphia, WB Saunders, 1996; p 21.
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32. Cohen MI, Rhodes LA, Spray TL, Gaynor JW: Efcacy of prophylactic epicardial pacing leads in children and young adults. Ann Thorac Surg 2004; 78:197; discussion 202. Review. 33. Walker F, Siu SC, Woods S, et al: Long-term outcomes of cardiac pacing in adults with congenital heart disease. J Am Coll Cardiol 2004; 43:1894. 34. Donit A, Bonvicini M, Placci A, et al: Surgical treatment of secundum atrial septal defect in patients older than fty years. Ital Heart J 2001; 2:428. 35. Jemielity M, Dyszkiewicz W, Paluszkiewicz L, et al: Do patients over forty years of age benet from surgical closure of atrial septal defects? Heart 2001; 85:300. 36. Landzberg M: Closure of atrial septal defects in adult patients: justication of the tipping point. J Intervent Cardiol 2001; 14:267. 37. Moodie D, Sterba R: Long-term outcomes excellent for atrial septal defect repair in adults. Cleve Clin J Med 2000; 67:591. 38. Gatzoulis M, Redington A, Somerville J, Shore D: Should atrial septal defects in adults be closed? Ann Thorac Surg 1996; 61:657. 39. Oakley C: Closure of atrial septal defect in adult life. Cardiologia 1996; 41:31. 40. Perloff J: Surgical closure of atrial septal defect in adults. N Engl J Med 1995; 8:513. 41. Steele PM, Fuster V, Cohen M, et al: Isolated atrial septal defect with pulmonary vascular obstructive disease: long-term followup and prediction of outcome after surgical correction. Circulation 1987; 76:1037. 42. Campbell M: Natural history of atrial septal defect. Br Heart J 1970; 32:820. 43. Craig RJ, Selzer A: Natural history and prognosis of atrial septal defect. Circulation 1968; 37:805. 44. Ad N, Birk E, Barak J, et al: A one-way valved atrial septal patch: a new surgical technique and its clinical application. J Thorac Cardiovasc Surg 1996; 111:841. 45. Stulak JM, Dearani JA, Puga FJ, et al: Right-sided Maze procedure for atrial tachyarrhythmias in congenital heart disease. Ann Thorac Surg 2006; 81:1780. 46. Deal BJ, Mavroudis C, Backer CL: Beyond Fontan conversion: Surgical therapy of arrhythmias including patients with associated complex congenital heart disease. Ann Thorac Surg 2003; 76:542. 47. Kirklin JW, Barratt-Boyes BG: Atrial septal defect and partial anomalous pulmonary venous connection, in Kirklin JW, BarrattBoyes BG (eds): Cardiac Surgery. New York, Churchill-Livingstone, 1993; p 620. 48. Kirklin JW, Barratt-Boyes BG: Atrioventricular canal defect, in Kirklin JW, Barratt-Boyes BG (eds): Cardiac Surgery. New York, Churchill-Livingstone, 1993; p 718. 49. Horvath KA, Burke RP, Collins JJ, Cohn LH: Surgical treatment of adult atrial septal defect: early and long-term results. J Am Coll Cardiol 1992; 20:1156. 50. Murphy JG, Gersh BJ, McGoon MD, et al: Long-term outcome after surgical repair of isolated atrial septal defect. N Engl J Med 1990; 323:1645. 51. Attenhofer Jost CH, Connolly HM, Danielson GK, et al: Sinus venosus atrial septal defect: long-term postoperative outcome for 115 patients. Circulation 2005; 112:1953. 52. Lloyd TR, Rao S, Beekman RH, et al: Atrial septal defect occlusion with the buttoned device (a multi-institutional U.S. trial). Am J Cardiol 1994; 73:286. 53. Rome JJ, Keane JF, Perry SB, et al: Double-umbrella closure of atrial defects. Circulation 1990; 82:751. 54. Hein R, Buscheck F, Fischer E, et al: Atrial and ventricular septal defects can safely be closed by percutaneous intervention. J Intervent Cardiol 2005; 18:515. Review. 55. Holzer R, Cao QL, Hijazi ZM: Closure of moderately large atrial septal defect with a self-fabricated fenestrated Amplatzer septal occluder in an 85-year-old patient with reduced diastolic elasticity of the left ventricle. Catheter Cardiovasc Intervent 2005; 64:513; discussion 519. Khositiseth A, Cabalka AK, Sweeney JP, et al: Transcatheter Amplatzer device closure of atrial septal defect and patent foramen ovale in patients with presumed paradoxical embolism. Mayo Clin Proc 2004; 79:35. Casselman FP, Dom H, De Bruyne B, et al: Thoracoscopic ASD closure is a reliable supplement for percutaneous treatment. Heart 2005; 91:791. Jemielity M, Perek B, Paluszkiewicz L, Dyszkiewicz W: Results of surgical repair of ostium primum atrial septal defect in adult patients. J Heart Valve Dis 2001; 10:525. Burke RP, Horvath K, Landzberg M, et al: Long-term follow-up after surgical repair of ostium primum atrial septal defects in adults. J Am Coll Cardiol 1996; 27:696. El-Najdawi EK, Driscoll DJ, Puga FJ, et al: Operation for partial atrioventricular septal defect: a forty-year review. J Thorac Cardiovasc Surg 2000; 119:880. Bergin ML, Warnes CA, Tajik AJ, Danielson GK: Partial atrioventricular canal defect: long-term follow-up after initial repair in patients or 40 years old. J Am Coll Cardiol 1995; 25:1189. Murashita T, Kubota T, Oba J, et al: Left atrioventricular valve regurgitation after repair of incomplete atrioventricular septal defect. Ann Thorac Surg 2004; 77:2157. Michielon G, Stellin G, Rizzoli G, et al: Left atrioventricular valve incompetence after repair of common atrioventricular canal defects. Ann Thorac Surg 1995; 60(6 Suppl):S604. Tlaskal T, Hucin B, Marek J, et al: Individualized repair of the left atrioventricular valve in spectrum of atrioventricular septal defect. J Cardiovasc Surg (Torino) 1997; 38:233. Ten Harkel AD, Cromme-Dijkhuis AH, Heinerman BC, et al: Development of left atrioventricular valve regurgitation after correction of atrioventricular septal defect. Ann Thorac Surg 2005; 79:607. Alexi-Meskishvili V, Hetzer R, Dahnert I, et al: Results of left atrioventricular valve reconstruction after previous correction of atrioventricular septal defects. Eur J Cardiothorac Surg 1997; 12:460. Capouya ER, Laks H, Drinkwater DC Jr, et al: Management of the left atrioventricular valve in the repair of complete atrioventricular septal defects. J Thorac Cardiovasc Surg 1992; 104:196; discussion 201. Mace L, Dervanian P, Houyel L, et al: Surgically created doubleorice left atrioventricular valve: a valve-sparing repair in selected atrioventricular septal defects. J Thorac Cardiovasc Surg 2001; 121:352. Lai YQ, Luo Y, Zhang C, Zhang ZG: Utilization of double-orice valve plasty in correction of atrioventricular septal defect. Ann Thorac Surg 2006; 81:1450. Poirier NC, Williams WG, Van Arsdell GS, et al: A novel repair for patients with atrioventricular septal defect requiring reoperation for left atrioventricular valve regurgitation. Eur J Cardiothorac Surg 2000; 18:54. McGrath LB, Kirklin JW, Soto B, Bargeron LM Jr.: Secondary left atrioventricular valve replacement in atrioventricular septal (AV canal) defect: a method to avoid left ventricular outow tract obstruction. J Thorac Cardiovasc Surg 1985; 89:632. Mora BN, Daebritz SH, del Nido PJ: Atrioventricular canal defects, in Selke FW, del Nido PJ, Swanson SJ (eds): Sabiston and Spencer Surgery of the Chest, Vol. II, 7th ed. Philadelphia, Elsevier, 2005; p 1963. Gurbuz AT, Novick WM, Pierce CA, Watson DC: Left ventricular outow tract obstruction after partial atrioventricular septal defect repair. Ann Thorac Surg 1999; 68:1723. Van Arsdell GS, Williams WG, Boutin C, et al: Subaortic stenosis in the spectrum of atrioventricular septal defects. Solutions may be complex and palliative. J Thorac Cardiovasc Surg 1995; 110:1534; discussion 1541.
56.
57.
58.
59.
60.
61.
62.
63.
64.
65.
66.
67.
68.
69.
70.
71.
72.
73.
74.
1460
99. Bauer M, Alexi-Meskishvili V, Bauer U, et al: Benets of surgical repair of coarctation of the aorta in patients older than fty years. Ann Thorac Surg 2001; 72:2060. 100. Marx G: Repaired aortic coarctation in adults: not a simple congenital heart defect. J Am Coll Cardiol 2000; 35:1003. 101. Bouchart F, Dubar A, Tabley A, et al: Coarctation of the aorta in adults: surgical results and long-term follow-up. Ann Thorac Surg 2000; 70:1483. 102. Aris A, Subirana T, Ferres P, Torner-Soler M: Repair of aortic coarctation in patients more than fty years of age. Ann Thorac Surg 1999; 67:1376. 103. Kirklin JW, Barratt-Boyes BG: Coarctation of the aorta and interrupted aortic arch, in Kirklin JW, Barratt-Boyes BG (eds): Cardiac Surgery. New York, Churchill-Livingstone, 1993; p 1263. 104. Almeida de Oliviera SA, Lisboa AF, Dallan LAO, et al: Extraanatomic aortic bypass for repair of aortic arch coarctation via sternotomy: midterm clinical and magnetic resonance imaging results. Ann Thorac Surg 2003; 76:1962. 105. Arakelyan V, Spiridonov A, Bockeria L: Ascending-to-descending aortic bypass via right thoracotomy for complex (re-) coarctation and hypoplastic aortic arch. Eur J Cardiothorac Surg 2005; 27:815. 106. Cohen M, Fuster V, Steele PM, et al: Coarctation of the aorta: long-term follow-up and prediction of outcome after surgical correction. Circulation 1989; 80:840. 107. Presbitero P, Demarie D, Villani M, et al: Long term results (1530 years) of surgical repair of aortic coarctation. Br Heart J 1987; 57:462. 108. Walhout R, Lekkerkerker J, Ernst S, et al: Angioplasty for coarctation in different aged patients. Am Heart J 2002; 144:180. 109. Rosenthal E: Stent implantation for aortic coarctation: the treatment of choice in adults? J Am Coll Cardiol 2001; 38:1524. 110. Hellenbrand WE, Allen HD, Golinko RJ, et al: Balloon angioplasty for aortic recoarctation: results of valvuloplasty and angioplasty of congenital anomalies registry. Am J Cardiol 1990; 65:793. 111. Zabal C, Attie M, Buendia-Hernandez A, Garcia-Montes JA: The adult patient with native coarctation of the aorta: balloon angioplasty or aortic stenting? Heart 2003; 89:77. 112. Carr JA: The results of catheter-based therapy compared with surgical repair of adult coarctation. J Am Coll Cardiol 2006; 47:1101. 113. Harrison D, McLaughlin P, Lazzam C, et al: Endovascular stents in the management of coarctation of the aorta in the adolescent and adult: one year follow up. Heart 2001; 85:561. 114. Therrien J, Siu S, McLaughlin P, et al: Pulmonary valve replacement in adults late after repair of tetralogy of Fallot: are we operating too late? J Am Coll Cardiol 2000; 36:1670. 115. Oechslin E, Harrison D, Harris L, et al: Reoperation in adults with repair of tetralogy of Fallot: indications and outcomes. J Thorac Cardiovasc Surg 1999; 118:245. 116. Dittrich S, Vogel M, Dahnert I, et al: Surgical repair of tetralogy of Fallot in adults today. Clin Cardiol 1999; 22:460. 117. Rammohan M, Airan B, Bham A, et al: Total correction of tetralogy of Fallot in adults: surgical experience. Int J Cardiol 1998; 63:121. 118. Van der Weijden P, Baur L, Kool L, et al: Embolization as a treatment of life-threatening haemoptysis in an adult with tetralogy of Fallot with pulmonary atresia. Int J Card Imaging 1998; 14:123. 119. Nollert G, Fischlein T, Bouterwek S, et al: Long-term results of total repair of tetralogy of Fallot in adulthood: 35 years follow-up in 104 patients corrected at the age of 18 or older. Thorac Cardiovasc Surg 1997; 45:178. 120. Jonsson H, Ivert T, Jonasson R, et al: Work capacity and central hemodynamics thirteen to twenty-six years after repair of tetralogy of Fallot. J Thorac Cardiovasc Surg 1995; 110:416. 121. Rosenthal A: Adults with tetralogy of Fallotrepaired, yes: cured, no. N Engl J Med 1993; 9:655. 122. Pome G, Rossi C, Colucci V, et al: Late reoperations after repair of tetralogy of Fallot. Eur J Cardiothorac Surg 1992; 6:31.
75. Kidd L, Driscoll DJ, Gersony WM, et al: Second natural history study of congenital heart defects: results of treatment of patients with ventricular septal defects. Circulation 1993; 87(Suppl I):I38. 76. Glen S, Burns J, Bloomeld P: Prevalence and development of additional cardiac abnormalities in 1448 patients with congenital ventricular septal defects. Heart 2004; 90:1321. 77. Rhodes LA, Keane JF, Keane JP, et al: Long follow-up (to 43 years) of ventricular septal defect with audible aortic regurgitation. Am J Cardiol 1990; 66:340. 78. Kirklin JW, Barratt-Boyes BG: Ventricular septal defect, in Kirklin JW, Barratt-Boyes BG (eds): Cardiac Surgery. New York, Churchill-Livingstone, 1993; p 800. 79. Ikawa S, Shimazaki Y, Nakano S, et al: Pulmonary vascular resistance during exercise late after repair of large ventricular septal defects: relation to age at the time of repair. J Thorac Cardiovasc Surg 1995; 109:1218. 80. Hornberger LK, Sahn DJ, Krabill KA, et al: Elucidation of the natural history of ventricular septal defects by serial Doppler color ow mapping studies. J Am Coll Cardiol 1989; 13:1111. 81. Rahko P: Doppler echocardiographic evaluation of ventricular septal defects in adults. Echocardiography 1993; 10:517. 82. OLaughlin MP, Mullins CE: Transcatheter occlusion of ventricular septal defect. Cathet Cardiovasc Diagn 1989; 17:175. 83. Lock JE, Locherman JT, Jeane JF, et al: Transcatheter umbrella closure of congenital heart defects. Circulation 1987; 73:593. 84. Moller JH, Patton C, Varco RL, Lillehei W: Late results (3035 years) after operative closure of isolated ventricular septal defect from 1954 to 1960. Am J Cardiol 1991; 68:1491. 85. Campbell M: Natural history of persistent ductus arteriosus. Br Heart J 1968; 30:4. 86. Ho A, Tan P, Yang M, et al: The use of multiplane transesophageal echocardiography to evaluate residual patent ductus arteriosus during video-assisted thoracoscopy in adults. Surg Endosc 1999; 13:975. 87. Schrader R, Kadel C, Cieslinski G, et al: Non-thoracotomy closure of persistent ductus arteriosus beyond age 60 years. Am J Cardiol 1993; 72:1319. 88. Yoshiyuki T, Matsumoto M, Sugita T: Optimal treatment for adult patent ductus arteriosus. Ann Thorac Surg 2001; 72:2186. 89. Toda R, Moriyama Y, Yamashita M, et al: Operation for adult patent ductus arteriosus using cardiopulmonary bypass. Ann Thorac Surg 2000; 70:1935. 90. Arora R: Patent ductus arteriosus: catheter closure in the adult patient. J Intervent Cardiol 2001; 14:255. 91. Latson LA: Residual shunts after transcatheter closure of patent ductus arteriosus: a major concern or benign techno-malady? [editorial comment]. Circulation 1991; 84:2591. 92. Bridges ND, Perry SB, Parness I, et al: Transcatheter closure of a large patent ductus arteriosus with the clamshell septal umbrella. J Am Coll Cardiol 1991; 18:1297. 93. Hosking MCK, Benson LN, Musewe N, et al: Transcatheter occlusion of the persistently patent ductus arteriosus. Circulation 1991; 84:2313. 94. Chessa M, Carrozza M, Butera G, et al: The impact of interventional cardiology for the management of adults with congenital heart defects. Cathet Cardiovasc Interv 2006; 67:258. 95. Roques F, Hennequin JL, Sanchez B, Ridarch A: Aortic stent-graft for patent ductus arteriosus in adults: the aortic exclusion technique. Ann Thorac Surg 2001; 71;1708. 96. Ozmen J, Granger EK, Robinson D, White GH: Operation for adult patent ductus arteriosus using an aortic stent-graft technique. Heart Lung Circ 2005; 14:54. 97. Hazama S, Sakamoto I, Yamachika S, Ariyoshi T: Endovascular surgery using an original occluder for patent ductus arteriosus in an adult patient. Jpn J Thorac Cardiovasc Surg 2005; 53:58. 98. Attenhofer Jost CH, Schaff HV, Connolly HM, et al: Spectrum of Reoperations after repair of aortic coarctation: Importance of an individualized approach because of coexistent cardiovascular disease. Mayo Clin Proc 2002; 77:646.
1461
123. Pacico AD: Reoperations after repair of tetralogy of Fallot, in Stark J, Pacico AD (eds): Reoperations in Cardiac Surgery. Berlin, Springer-Verlag, 1989; p 171. 124. Stewart S, Alexson C, Manning J: Long-term palliation with the classic Blalock-Taussig shunt. J Thorac Cardiovasc Surg 1988; 96:117. 125. Harrison D, Harris L, Siu S, et al: Sustained ventricular tachycardia in adult patients late after repair of tetralogy of Fallot. J Am Coll Cardiol 1997; 30:1368. 126. Cullen S, Celermajer DS, Franklin RCG, et al: Prognostic signicance of ventricular arrhythmia after repair of tetralogy of Fallot: a 12-year prospective study. J Am Coll Cardiol 1994; 23:1151. 127. Garson A, Nihill MR, McNamara DG, Cooley DA: Status of the adult and adolescent after repair of tetralogy of Fallot. Circulation 1979; 59:1232. 128. Kirklin JW, Devloo RA: Hypothermic perfusion and circulatory arrest for surgical correction of tetralogy of Fallot with previously constructed Potts anastomosis. Dis Chest 1961; 39:87. 129. Presbitero P, Demarie D, Aruta E, et al: Results of total correction of tetralogy of Fallot performed in adults. Ann Thorac Surg 1988; 46:297. 130. Zahka KG, Horneffer PJ, Rowe SA, et al: Long-term valvular function after total repair of tetralogy of Fallot: relation to ventricular arrhythmias. Circulation 1988; 78(Suppl III):III-14. 131. Lillehei CW, Varco RL, Cohen M, et al: The rst open heart corrections of tetralogy of Fallot: a twenty-six to thirty-one year follow-up of 106 patients. Ann Surg 1986; 204:490. 132. Deaneld JF, McKenna WJ, Presbitero P, et al: Ventricular arrhythmia in unrepaired and repaired tetralogy of Fallot: relation to age, timing of repair, and haemodynamic status. Br Heart J 1984; 52:77. 133. Uretzky G, Puga FJ, Danielson GK, et al: Reoperation after correction of tetralogy of Fallot. Circulation 1982; 66(Suppl I):I-202. 134. Fuster V, McGoon DC, Kennedy MA, et al: Long-term evaluation (twelve to twenty-two years) of open heart surgery for tetralogy of Fallot. Am J Cardiol 1980; 46:635. 135. Marelli A, Perloff J, Child J, Laks H: Pulmonary atresia with ventricular septal defect in adults. Circulation 1994; 89:243. 136. Reichenspurner H, Netz H, Uberfuhr P, et al: Heart-lung transplantation in a patient with pulmonary atresia and ventricular septal defect. Ann Thorac Surg 1994; 57:210. 137. Permut LC, Laks H: Surgical management of pulmonary atresia with ventricular septal defect and multiple aortopulmonary collaterals, in Karp RB, Laks H, Wechsler AS (eds): Advances in Cardiac Surgery. St. Louis, Mosby Year Book, 1999; p 75. 138. Trivedi KR, Karamlou T, Yoo SJ, et al: Outcomes in 45 children with ductal origin of the distal pulmonary artery. Ann Thorac Surg 2006; 81:950. 139. Ko Y, Nakamura Y, Nomura K, et al: Bidirectional cavopulmonary shunt using the azygos vein. Jpn J Thorac Cardiovasc Surg 2005; 53:213. 140. Duncan BW, Mee RB, Prieto LR, et al: Staged repair of tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries. J Thorac Cardiovasc Surg 2003; 126:694. 141. Webb GD, McLaughlin PR, Gow RM, et al: Transposition complexes. Cardiol Clin 1993; 11:651. 142. Turina M, Siebenmann R, Nussbaumer P, Senning A: Long-term outlook after atrial connection of transposition of great arteries. J Thorac Cardiovasc Surg 1988; 95:828. 143. Kammeraad JAE, van Deurzen CHM, Sreeram N, et al: Predictors of sudden cardiac death after Mustard or Senning repair for transposition of the great arteries. J Am Coll Cardiol 2004; 1:44:1095. 144. Poirier NC, Yu J, Brizard CP, Mee RBB: Long-term results of left ventricular reconditioning and anatomic correction for systemic right ventricular dysfunction after atrial switch procedures. J Thorac Cardiovasc Surg 2004; 127:975. 145. Kirklin JW, Blackstone EH, Tchervenkov CI, et al: Clinical outcomes after the arterial switch operation for transposition of the great arteries. Circulation 1995; 109:289. 146. Hechter S, Webb G, Fredriksen P, et al: Cardiopulmonary exercise performance in adult survivors of the Mustard procedure. Cardiol Young 2001; 11:407. 147. Fredrikson P, Chen A, Veldtman G, et al: Exercise capacity in adult patients with congenitally corrected transposition of the great arteries. Heart 2001; 85:191. 148. Padalino M, Stellin G, Brawn W, et al: Arterial switch operation after left ventricular retraining in the adult. Ann Thorac Surg 2000; 70:1753. 149. Cetta F, Bonilla J, Lichtenberg R, et al: Anatomic correction of dextrotransposition of the great arteries in a 36-year-old patient. Mayo Clin Proc 1997; 72:245. 150. Connelly M, Liu P, Williams W, et al: Congenitally corrected transposition of the great arteries in the adult: functional status and complications. J Am Coll Cardiol 1996; 27:1238. 151. Presbitero P, Somerville J, Rabajoli F, et al: Corrected transposition of the great arteries without associated defects in adult patients: clinical prole and follow up. Br Heart J 1995; 74:57. 152. Serraf A, Roux D, Lacour-Gayet F, et al: Reoperation after the arterial switch operation for transposition of the great arteries. J Thorac Cardiovasc Surg 1995; 110:892. 153. Cochrane AD, Karl TR, Mee RB: Staged conversion to arterial switch for late failure of the systemic right ventricle. Ann Thorac Surg 1993; 56:854. 154. Chang AC, Wernovsky G, Wessel DL, et al: Surgical management of late right ventricular failure after Mustard or Senning repair. Circulation 1992; 86(suppl II):II-140. 155. Stark J: Reoperations after Mustard and Senning operations, in Stark J, Pacico AD (eds): Reoperations in Cardiac Surgery. Berlin, Springer-Verlag, 1989; p 187. 156. Peterson RJ, Franch RH, Fajman WA, Jones RH: Comparison of cardiac function in surgically corrected and congenitally corrected transposition of the great arteries. J Thorac Cardiovasc Surg 1988; 96:227. 157. Quaegebeur M, Rohmer J, Brom AG: Revival of the Senning operation in the treatment of transposition of the great arteries. Thorax 1977; 32:517. 158. Elizari A, Somerville J: Experience with the Glenn anastomosis in the adult with cyanotic congenital heart disease. Cardiol Young 1999; 9:257. 159. Bruckheimer E, Bulbul Z, Hellenbrand W, et al: Takedown of Glenn shunts in adults with congenital heart disease: technique and long-term follow-up. J Thorac Cardiovasc Surg 1997; 113:607. 160. Jonas RA: Indications and timing for the bidirectional Glenn shunt versus the fenestrated Fontan circulation. J Thorac Cardiovasc Surg 1994; 108:522. 161. Kopf GS, Laks H, Stansel HC, et al: Thirty-year follow-up of superior vena cava-pulmonary artery (Glenn) shunts. J Thorac Cardiovasc Surg 1990; 100:662. 162. Glenn WL, Fenn JE: Axillary arteriovenous stula: a means of supplementing blood ow through a cava-pulmonary artery shunt. Circulation 1972; 46:1013. 163. Stamm C, Friehs I, Mayer J, et al: Long-term results of the lateral tunnel Fontan operation. J Thorac Cardiovasc Surg 2001; 121:28. 164. Harrison D, Liu P, Walters J, et al: Cardiopulmonary function in adult patients late after Fontan repair. J Am Coll Cardiol 1995; 26:1016. 165. Podzolkov V, Zaets S, Chiaureli M, et al: Comparative assessment of Fontan operation in modications of atriopulmonary and total cavopulmonary anastomoses. Eur J Cardiothorac Surg 1997; 11:458. 166. Driscoll DJ, Offord KP, Feldt RH, et al: Five- to fteen-year followup after Fontan operation. Circulation 1992; 85:469. 167. Deal BJ, Mavroudis C, Backer CC, et al: Comparison of anatomic isthmus block with the modied right atrial Maze procedure for late atrial tachycardia in Fontan patients. Circulation 2002; 106:575.
1462
191. Chauvaud S, Berrebi A, dAttellis N, et al: Ebsteins anomaly: repair based on functional analysis. Eur J Cardiothorac Surg 2003; 23:525. 192. Chen JM, Mosca RS, Altmann K, et al: Early and medium-term results for repair of Ebsteins anomaly. J Thorac Cardiovasc Surg 2004; 127:990; discussion 998. 193. Khositseth A, Danielson GK, Dearani JA, et al: Supraventricular tachyarrhythmias in Ebstein anomaly: management and outcome. J Thorac Cardiovasc Surg 2004; 128:826. 194. Greason KL, Dearani JA, Theodoro DA, et al: Surgical management of atrial tachyarrhythmias associated with congenital cardiac anomalies: Mayo Clinic experience. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2003; 6:59. 195. Kiziltan HT, Theodoro DA, Warnes CA, et al: Late results of bioprosthetic tricuspid valve replacement in Ebsteins anomaly. Ann Thorac Surg 1998; 66:1539. 196. Churgh R, Marelli D, Child J, et al: Heart transplantation in adolescents and adults with congenital heart disease. Presentation at the Annual Meeting of the American College of Cardiology, Atlanta, Ga, March 2002. 197. Pigula F, Gandhi S, Ristich J, et al: Cardiopulmonary transplantation for congenital heart disease in the adult. J Heart Lung Transplant 2001; 20:297. 198. Stoica S, McNeil K, Perreas K, et al: Heart-lung transplantation for Eisenmenger syndrome: early and long-term results. Ann Thorac Surg 2001; 72:1887. 199. Lamour J, Addonizio L, Galantowicz M, et al: Outcome after orthotopic cardiac transplantation in adults with congenital heart disease. Circulation 1999; 100:II200. 200. Speziali G, Driscoll D, Danielson G, et al: Cardiac transplantation for end-stage congenital heart defects: the Mayo Clinic experience. Mayo Cardiothoracic Transplant team. Mayo Clin Proc 1998; 73:923. 201. Mendeloff E, Huddleston C: Lung transplantation and repair of complex congenital heart lesions in patients with pulmonary hypertension. Semin Thorac Cardiovasc Surg 1998; 10:144. 202. Fann J, Wilson M, Theodore J, Reitz B: Combined heart and single-lung transplantation in complex congenital heart disease. Ann Thorac Surg 1998; 65:823. 203. Carpentier A, Levy F, Mettauer B, et al: Successful sequential double lung transplantation and cardiac repair for aortopulmonary window: technical and functional advantages over heart-lung transplantation. Transplant Proc 1996; 28:2878. 204. Lupinetti F, Bolling S, Bove E, et al: Selective lung or heart-lung transplantation for pulmonary hypertension associated with congenital cardiac anomalies. Ann Thorac Surg 1994; 57: 1545. 205. Bando K, Armitage JM, Paradis IL, et al: Indications for and results of single, bilateral, and heart-lung transplantation or pulmonary hypertension. J Thorac Cardiovasc Surg 1994; 108:1056. 206. Lupinetti FM, Bolling SF, Bove EL, et al: Selective lung or heartlung transplantation for pulmonary hypertension associated with congenital cardiac anomalies. Ann Thorac Surg 1994; 57:1545. 207. Sarris GE, Smith JA, Shumway NE, et al: Long-term results of combined heart-lung transplantation: the Stanford experience. J Heart Lung Transplant 1994; 13:940. 208. Pearl JM, Laks H, Drinkwater DC: Cardiac transplantation following the modied Fontan procedure. Transplant Sci 1992; 2:1. 209. Spray TL, Mallory GB, Canter CE, et al: Pediatric lung transplantation for pulmonary hypertension and congenital heart disease. Ann Thorac Surg 1992; 54:216. 210. Madden B, Radley-Smith R, Hodson M, et al: Medium-term results of heart and lung transplantation. J Heart Lung Transplant 1992; 11:S241. 211. Mayer JE, Perry S, OBrien P, et al: Orthotopic heart transplantation for complex congenital heart disease. J Thorac Cardiovasc Surg 1990; 99:484. 212. Reddy VM, McElhinney DB, Silverman NH, et al: Partial biventricular repair for complex congenital heart defects: an intermedi-
168. Gentles T, Gauvreau K, Mayer J, et al: Functional outcome after the Fontan operation: factors inuencing late morbidity. J Thorac Cardiovasc Surg 1997; 114:392. 169. Koutlas T, Harrison K, Bashore T, et al: Late conduit occlusion after modied Fontan procedure with classic Glenn shunt. Ann Thorac Surg 1996; 62:258. 170. Veldtman G, Nishimoto A, Siu S, et al: The Fontan procedure in adults. Heart 2001; 86:330. 171. Fredriksen P, Therrien J, Veldtman G, et al: Lung function and aerobic capacity in adult patients following modied Fontan procedure. Heart 2001; 85:295. 172. Dore A, Somerville J: Right atrioventricular extracardiac conduit as a Fontan modication: late results. Ann Thorac Surg 2000; 69:181. 173. Gates R, Laks H, Drinkwater D, et al: The Fontan procedure in adults. Ann Thorac Surg 1997; 63:1085. 174. Gelatt M, Hamilton RM, McCrindle BW, et al: Risk factors for atrial tachyarrhythmias after the Fontan operation. J Am Coll Cardiol 1994; 24:173. 175. Kirklin JW, Barratt-Boyes BG: Tricuspid atresia and the Fontan operation, in Kirklin JW, Barratt-Boyes BG (eds): Cardiac Surgery. New York, Churchill-Livingstone, 1993; p 1055. 176. Giannico S, Corno A, Marino B, et al: Total extracardiac right heart bypass. Circulation 1992; 86(Suppl II):II-110. 177. Laks H, Pearl JM, Haas GS, et al: Partial Fontan: advantages of an adjustable interatrial communication. Ann Thorac Surg 1991; 52:1084. 178. Celermajer DS, Bull C, Till JA, et al: Ebsteins anomaly: presentation and outcome from fetus to adult. J Am Coll Cardiol 1994; 23:170. 179. Kirklin JW, Barratt-Boyes BG: Ebsteins malformation, in Kirklin JW, Barratt-Boyes BG (eds): Cardiac Surgery. New York, Churchill-Livingstone, 1993; p 1105. 180. Hebe J: Ebsteins anomaly in adults. Arrhythmias: diagnosis and therapeutic approach. Thorac Cardiovasc Surg 2000; 48:214. 181. Pressley JC, Wharton JM, Tang ASL, et al: Effect of Ebsteins anomaly on short- and long-term outcome of surgically treated patients with Wolff-Parkinson-White syndrome. Circulation 1992; 86:1147. 182. Attie F, Rosas M, Rijlaarsdam M, et al: Analytical reviews of internal medicine, dermatology, neurology, pediatrics and psychiatry: the adult patient with Ebstein anomaly. Medicine (Baltimore) 2000; 79:27. 183. Saxena A, Fong LV, Tristam M, et al: Late noninvasive evaluation of cardiac performance in mildly symptomatic older patients with Ebsteins anomaly of tricuspid valve: role of radionuclide imaging. J Am Coll Cardiol 1991; 17:182. 184. Attie F, Rosas M, Rijlaarsdam M, et al: The adult patient with Ebstein anomaly. Outcome in 72 unoperated patients. Medicine (Baltimore) 2000; 79:27. 185. Gentles TL, Calder L, Clarkson PM, Neutze JM: Predictors of long-term survival with Ebsteins anomaly of the tricuspid valve. Am J Cardiol 1992; 69:377. 186. Frescura C, Angelini A, Daliento L, Thiene G: Morphological aspects of Ebsteins anomaly in adults. Thorac Cardiovasc Surg 2000; 48:203. 187. Oechslin E, Buchholz S, Jenni R: Ebsteins anomaly in adults: Doppler-echocardiographic evaluation. Thorac Cardiovasc Surg 2000; 48:209. 188. Mair DD, Seward JB, Driscoll DJ, Danielson GK: Surgical repair of Ebsteins anomaly: selection of patients and early and late operative results. Circulation 1985; 72:70. 189. Carpentier A, Chauvaud S, Mace L, et al: A new reconstructive operation for Ebsteins anomaly of the tricuspid valve. J Thorac Cardiovasc Surg 1988; 96:92. 190. Attenhofer Jost CH, Connolly HM, Edwards WD, et al: Ebsteins anomalyreview of a multifaceted congenital cardiac condition. Swiss Med Wkly 2005; 135:269.
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ate option for complicated anatomy or functionally borderline right complex heart. J Thorac Cardiovasc Surg 1998; 116:21. Chowdhury UK, Airan B, Sharma R, et al: One and a half ventricle repair with pulsatile bidirectional Glenn: results and guidelines for patient selection. Ann Thorac Surg 2001; 71:1995. Chowdhury UK, Airan B, Talwar S, et al: One and one-half ventricle repair: results and concerns. Ann Thorac Surg 2005; 80:2293. Stellin G, Vida VL, Milanesi O, et al: Surgical treatment of complex cardiac anomalies: the one and one half ventricle repair. Eur J Cardiothorac Surg 2002; 22:1043. Rabago G, Copeland J, Rosapepe F, et al: Heart-lung transplantation in situs inversus. Ann Thorac Surg 1996; 62:296. Carrel T, Neth J, Pasic M, et al: Should cardiac transplantation for congenital heart disease be delayed until adult age? Eur J Cardiothorac Surg 1995; 8:462. Perry SB, Radtke W, Fellows KE, et al: Coil embolization to occlude aortopulmonary collateral vessels and shunts in patients with congenital heart disease. J Am Coll Cardiol 1989; 13:100. Odim J, Wheat J, Laks H, et al: Peri-operative renal function and outcome after orthotopic heart transplantation. J Heart Lung Transplant 2006; 25:162. 220. Myers RP, Cerini R, Sayegh R, et al: Cardiac hepatopathy: clinical, hemodynamic, and histologic characteristics and correlations. Hepatology 2003; 37:393. 221. Dichtl W, Vogel W, Dunst KM, et al: Cardiac hepatopathy before and after heart transplantation. Transpl Int 2005; 18:697. 222. Wadia Y, Etheridge W, Smart F, et al: Pathophysiology of hepatic dysfunction and intrahepatic cholestasis in heart failure and after left ventricular assist device support. J Heart Lung Transplant 2005; 24:361. 223. Hashimoto K, Sasaki T, Hachiya T, et al: Superior hepatic mitochondrial oxidation-reduction state in normothermic cardiopulmonary bypass. J Thorac Cardiovasc Surg 2001; 121:1179. 224. Jayakumar KA, Addonizio LJ, Kichuk-Chrisant MR, et al: Cardiac transplantation after the Fontan or Glenn procedure. J Am Coll Cardiol 2004; 44:2065. 225. Carey JA, Hamilton JR, Hilton CJ, et al: Orthotopic cardiac transplantation for the failing Fontan circulation. Eur J Cardiothorac Surg 1998; 14:7; discussion 13. 226. Gamba A, Merlo M, Fiocchi R, et al: Heart transplantation in patients with previous Fontan operations. J Thorac Cardiovasc Surg 2004; 127:555.
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CHAPTER

Adult Congenital Heart Disease

GENERAL MANAGEMENT Preoperative Evaluation

Procedures Requiring Reoperation

SPECIFIC CONGENITAL MALFORMATIONS Atrial Septal Defects

Ventricular Septal Defects

Patent Ductus Arteriosus

Coarctation of the Aorta

Late Reoperations for Transposition of the Great Arteries

Modied Fontan procedure

Late Reoperations after Modied and Classic Fontan Procedures

Figure 61-17. Extracardiac Fontan with adjustable conduit-to-atrial connection.

Figure 61-18. Danielsons repair of the tricuspid valve in Ebstein anomaly.

Figure 61-19. Carpentiers repair of the tricuspid valve in Ebstein anomaly.

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