Cyanotic congenital heart disease

Classification of congenital heart diseases

Group I : Group II: Group III: Left to right shunts Right to lefts shunts Obstructive lesions

Left to right shunts

Atrial Septal Defect Ventricular Septal Defect Patent Ductus Arteriosus

Right to Left Shunts

1) Tetralogy of Fallot
2) 3) Tricuspid atresia Ebsteins anomaly

4) Transposition of Great Vessels 5) Truncus Arteriosus 6) Total Anomalous Pulmonary Venous Return (TAPVR)

Obstructive Lesions
Aortic stenosis Coarctation of the Aorta Pulmonic Stenosis

Cyanotic heart disease

Right to Left Shunt

Who is this guy?

TIENNE-LOUIS ARTHUR FALLOT!

a French physician, 1888 Fallot accurately described in detail the four anatomical characteristics of tetralogy of Fallot.

Tetralogy OF Fallot
Most common cyanotic heart disease!

75%!

TOF
4 component!
Imagine this is a HEART!

TOF
1) Vetricular Septal Defect

TOF
1) Vetricular Septal Defect 2) Pulmonic Stenosis

TOF
1) Vetricular Septal Defect 2) Pulmonic Stenosis 3) Overriding of dextroposed aorta

TOF
1) 2) 3) 4) Vetricular Septal Defect Pulmonic Stenosis Overriding of dextroposed aorta Right Ventricular hypertrophy

TOF
1) 2) 3) 4) Vetricular Septal Defect Pulmonic Stenosis Overriding of dextroposed aorta Right Ventricular hypertrophy
Concentric R ventricular hypertrophy without cardiac enlargement

TOF
1) 2) 3) 4) Vetricular Septal Defect Pulmonic Stenosis Overriding of dextroposed aorta Right Ventricular hypertrophy
Concentric R ventricular hypertrophy without cardiac enlargement

Increase in right ventricular pressure*

RV and LV pressures becomes identical

RV and LV pressures becomes identical

There is little or no L to R shunt

Hence, VSD is silent

RV and LV pressures becomes identical

There is little or no L to R shunt

Right ventricle into pulmonary artery across pulmonic stenosis producing ejection systolic murmur

Hence, VSD is silent

RV and LV pressures becomes identical

There is little or no L to R shunt

Hence, the more severe the pulmonary stenosis

Hence, the more severe the pulmonary stenosis The BIGGER the Left to RIGHT shunt

Hence, the more severe the pulmonary stenosis The BIGGER the Left to RIGHT shunt Less flow into the pulmonary artery

Hence, the more severe the pulmonary stenosis The BIGGER the Left to RIGHT shunt Less flow into the pulmonary artery Shorter the ejection systolic murmur

Hence, the more severe the pulmonary stenosis The BIGGER the Left to RIGHT shunt Less flow into the pulmonary artery Shorter the ejection systolic murmur More cynosis because of less flow to the lung!

Hence,
Severity of cyanosis is directly proportional to the severity of pulmonic stenosis Intensity of the systolic murmur is inversely related to the severity of pulmonic stenosis

Congestive failure never occur* because

Right ventricle is effectively decompressed because of the ventricular septal defect.

* exception

Congestive failure never occur* because

Right ventricle is effectively decompressed 1) Anemia because of the ventricular septal defect.
2)Infective Endocarditis 3)Systemic hypertension 4)Unrelated myocarditis complicating TOF 5)Aortic or pulmonary valve regurgitation

* exception

Pulmonary obstruction results in delayed P2

Pulmonary artery pressure reduce Pulmonary obstruction results in delayed P2

P2 become soft or inaudible

Pulmonary artery pressure reduce Pulmonary obstruction results in delayed P2

(Second Sound) S2= A2 + P2 Since P2 is inaudible, hence S2 = A2 + P2 [S2 is single sound]

P2 become soft or inaudible

Aorta is displace anteriorly too, A2 become LOUD!

Pulmonary artery pressure reduce Pulmonary obstruction results in delayed P2

 Ascending aorta in TOF is large, results aortic ejection click

 Diastolic interval is clear No S3 No S4

Concentric right ventricular hypertrophy reduce the distensibility of the right ventricle during diastole

Concentric right ventricular hypertrophy reduce the distensibility of the right ventricle during diastole

a waves become prominent in JVP* *but not too tall

Clinical Picture
Symptomatic any time after birth Paroxysmal attacks of dyspnea
Anoxic spells Predominantly after waking up Child cry Dyspnea Blue Lose conscious Convulsion Frequency varies from once a few days to many attack everyday

tet spell
lethal, unpredictable episodes The mechanism
spasm of the infundibular septum, which acutely worsens the RV outlet obstruction.

 Dyspnea on exertion Exercise intolerance

 Sitting posture squatting

Compensatory mechanism Squatting increases the peripheral vascular resistance, which diminishes the right-to-left shunt increases pulmonary blood flow.

 Cyanosis during feeding

Poor feeding fussiness, tachypnea, and agitation. Birth weight is low. Growth is retarded. Development and puberty may be delayed.

 Rarely, patient remain asymptomatic into adult life.

Physical examination
Clubbing + Cyanosis (Variable) Squatting position Scoliosis Common bulging left hemithorax

 Prominent a waves JVP Normal heart size

Mild parasternal impulse

Systolic trill (30%)

 S1 normal S2 single
only A2 heard P2 soft & delayed: INAUDIBLE

Murmur
Shunt murmur (VSD) absent Flow murmur: Ejection systolic, the smaller the flow the shorter the murmur

Ejection aortic click

 Retinal engorgement Hemoptysis

ECG
ECG

ECG
ECG

ECG
ECG
wiLLiam moRRow

ECG
Right axis deviation (+120 to +150) Right or combined ventricular hypertrophy Right atrial hypertrophy Partial or complete right bundle branch block (especially true of patients after surgical repair)

 Coeur en sabot (boot-shaped heart) secondary to uplifting of the cardiac apex from RVH and the absence of a normal main pulmonary artery segment

 Normal heart size due to the lack of pulmonary blood flow and congestive heart failure

 Decreased pulmonary vascularity

 Right atrial enlargement Right-sided aortic arch (20-25% of patients) with indentation of leftward-positioned tracheobronchial shadow

Echocardiography
Reveals a large VSD overriding aorta variable degrees of right ventricular outflow tract (RVOT) obstruction

Course and Complication

1) Each anoxic spell is potentially fatal 2) Polycytemia
1) Cerebrovascular thrombosis

3) Anoxic infaction of CNS

1) Neurological complication

4) LUNG is an awesome filter.

1) Bypassing it may not be a good idea! 2) TOF, venous blood from gut, peripheral system by pass the lung and re-enter circulation 3) Hence TOF can cause:
1) Brain Abcess 2) Infective endocarditis 3) Paradoxical embolism

Management of anoxic spell

1) Knee chest position 2) Humified O2 3) Be careful not to provoke the child
1) Especially you are bad at gaining IV access 2) Ask for help from someone more experience 3) Permit the baby to remain with mother

4) Morphine 0.1 -0.2 mg/Kg Subcutaneous 5) Correct acidosis Sodium Bicarb IV

6) Propanolol
1) 0.1mg/kg/IV during spells 2) 0.5 to 1.0 mg/kg/ 4-6hourly orally

7) Vasopressors: Methoxamine IM or IV drip 8) Correct anemia 9) GA is the last resort

Palliative Surgery
Blalock-Taussig shunt Pott procedure Waterston shunt

Blalock Taussig Shunt

Subclavian artery Pulmonary artery anastomosis

Modified Blalock Taussig Shunt

Goretex graft

Surgical Palliation

 Palliative operation prolong life Increase exercise tolerance

Definitive operation
Closing the VSD Resecting infundibular 90% can return almost normal life after operation Complication:
RBBB Residual VSD Residual Pulmonary stenosis Pulmonary regurgitation (pulmonary valve excised) Risk 5%

Transposition of Great Areries (TGA)

Aorta originating from the right ventricle, and pulmonary artery originating from the left ventricle Accounts for 5-7% of all congenital heart disease

TGA
Survival is dependent on the presence of mixing between the pulmonary and systemic circulation Atrial septal defect is essential for survival 50% of patients have a VSD Usually presents in the first day of life with profound cyanosis More common in boys

TGA
Exam : cyanosis in an otherwise healthy looking baby Loud S2 ( aorta is anterior ) CXR : Egg on side Narrow mediastinum

TGA .. Acute Management

PGE-1 with no supplemental O2
Maintain ductus arteriosus patency, this will increase the effective pulmonary blood flow, and thence increase the left atrial pressure, therefore inhance the left to right shunt at the atrial level

Balloon atrial septostomy

Life saving procedure in the presence of inadequate atrial septal defect

TGA .. Surgical Management

Arterial switch
with re-implantation of the coronary artery to the new aortic site.

Atrial switch :
the old style surgery Redirecting the pulmonary and systemic venous return to result in a physiologically normal state The right ventricle remains the systemic ventricle Rarely needed

Truncus Arteriosus
The presence of a common trunk that supply the systemic, pulmonary and coronary circulation Almost always associated with VSD 1.2-2.5% of all congenital heart disease

Truncus Arteriosus
There are different anatomical tupes of truncus arteriosus This is relevant for surgical repair

Truncus Arteriosus
Generally patients have increased pulmonary blood flow Degree of cyanosis is mild and may not be evident clinically until late stage with pulmonary vascular disease Presenting feature is congestive heart failure (tachypnia, hepatomegally)

Truncus Arteriosus
Exam is significant for
Single S2 Ejection click of the abnormal truncal valve Systolic murmur of truncal valve stenosis if present Diaastolic murmur of truncal valve insufficiency Gallop

CXR : Cardiomegally , increased pulmonary circulation

Managment
Acute management
No O2 to minimize pulmonary blood flow Diuretics Afterload reduction to inhance systemic blood flow

Surgical management: complete repair with VSD closure and conduit placement between the right ventricle and pulmonary arteries Long term problems :
truncal valve dysfunction RV conduit obstruction

Trcuspid Atresia
Complete absence of communication between the right atrium and right ventricle About 3 % of congenital heart disease

Tricuspid Atresia
There is an obligate interatrial communication Usually associated with VSD The pulmonary blood flow is dependent on the size of the VSD Pulmonary blood flow can be increased or decreased causing variable presenting symptoms If there is no VSD ( also called Hypoplastic right ventricle) the pulmonary blood flow is dependent on the PDA

Tricuspid Atresia- presentation

The presentation will depend on the amount of pulmonary blood flow
If the PBF is decreased, the main presenting symptom is cyanosis If the PBF is increased the presentation is that of congestive heart failure

CXR will also reflect the amount of pulmonary blood flow

Tricuspid Atresia- EKG

Very characterestic : Left axis deviation

Management
PBF
Decreased
PGE-1, and minimal supplemental O2 to maintain ductal patency

Increased
No O2 Afterload reduction Diuretics

Surgical Management
Single ventricle paliation First stage : to establish a reliable source of PBF
Aorta to pulmonary artery shunt ( BT shunt) Pulmonary arterial banding in cases of increased PBF

Second stage: Glenn Anastomosis ( superior vena cava to pulmonary artery Third stage : Fontan anastomosis ( Inferior vena cava to pulmonary artery

Total Anomalous Pulmonary Venous Return (TAPVR)

TAPVR- Infracardiac

Radiography

Infracardiac type

Thank You