Transposition of the Great Arteries

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Transposition of the Great Vessels

most common cyanotic congenital heart lesion that presents in neonates ventriculoarterial discordance
artery arises from the morphologic left

Hallmark:

aorta arises from the morphologic right ventricle ventricle

pulmonary

Survival

rate: > 90%

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Major Classifications
depend

on the relationship of the great arteries to each other and/or the infundibular morphology d-TGA

1.

60% of the patients aorta is anterior and to the right of the pulmonary artery

2.

l-TGA

aorta is anterior and to the left of the pulmonary artery 6/30/12

Primary Anatomic Subtypes

1. 2. 3.

TGA with intact ventricular septum TGA withVSD TGA with VSD and left ventricular outflow tract obstruction TGA with VSD and pulmonary vascular obstructive disease

4.

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Pathophysiology
pulmonary results

and systemic circulations function in parallel, rather than in series in a deficient oxygen supply to the tissues and an excessive right and left ventricular workload with prolonged survival unless mixing of oxygenated and deoxygenated blood occurs at some anatomic level

incompatible

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common anatomic sites for mixing of oxygenated and deoxygenated blood in transposition of the great arteries:
1. 2. 3.

ASD VSD PDA

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Manifestations

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History
usually

born at term apparent within hours of birth

cyanosis clinical

course and manifestations depend on the extent of intercirculatory mixing and the presence of associated anatomic lesions

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TGA with Intact Ventricular Septum

Prominent

and progressive central cyanosis within the first 24 hours of life than cyanosis, the physical examination is often unremarkable

Other

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TGA with Large VSD

Cyanosis

may be mild initially; usually more apparent with stress or crying right ventricular impulse grade 3-4/6 holosystolic murmur rumble may be present
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increased

prominent third

heart sound rhythm

mid-diastolic gallop

Hepatomegaly

TGA with VSD and LV Outflow Tract Obstruction

extreme

cyanosis at birth, proportional to the degree of left ventricular (pulmonary) outflow tract obstruction single, or narrowly split, diminished second heart sound 2-3/6 systolic ejection murmur is rare

grade

Hepatomegaly

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TGA with VSD and Pulmonary Vascular Obstructive Disease

progressive No

cyanosis, despite an early successful palliative procedure murmur (despite the ventricular septal defect) or early short systolic ejection sounds are heard heart sound: often single, with increased intensity later childhood or adolescence, a highpitched, blowing, early decrescendo diastolic murmur of pulmonary insufficiency and a blowing apical murmur of mitral insufficiency are evident 6/30/12

second In

Imaging studies

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Chest X-ray
may egg

appear normal

on a string" appearance (1/3 of patients) with increased pulmonary arterial vascular markings = VSD

cardiomegaly

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Echocardiography
bifurcating

pulmonary artery arising posteriorly from the left ventricle in the parasternal long-axis view short-axis view: relationship of the great arteries to one another

parasternal

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Cardiac Catheterization
usually

reserved for the subgroup of patients for whom echocardiography does not adequately delineate the anatomy confirm or better delineate suspected coronary artery abnormalities and additional ventricular septal defects

may

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treatment

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Medical Care
maintaining
promote increase promote

ductal patency with continuous intravenous (IV) prostaglandin E1infusion

pulmonary blood flow left atrial pressure

left-to-right intercirculatory mixing at the atrial level

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Medical Care
Cardiac

catheterization

indicated

for a balloon atrial septostomy in severely hypoxemic patients with an inadequate atrial level communication and insufficient mixing atrial septostomy is used to increase the atrial level shunt and to improve mixing.

balloon

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Medical Care
metabolic

acidosis should be corrected with fluid replacement and bicarbonate administration ventilation may be necessary if pulmonary edema develops in concert with severe hypoxemia repair or palliation early in life

Mechanical

surgical

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Surgical Care
depends
age

on:
of associated congenital cardiac lesions

of the patient at presentation

presence

experience

of the cardiothoracic surgeon with a given surgical technique

Most

full-term neonates with uncomplicated transposition of the great arteries can undergo an arterial switch procedure in one operation, with minimal mortality

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TGA with Intact Ventricular Septum

Arterial
ideal

switch procedure
repair ventriculoarterial concordance

procedure

anatomic

establishes not

recommended when coronary artery translocation may not be feasible (intramural coronary artery)

Atrial level switch

Senning or Mustard procedure surgical and short-term morbidity and mortality

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lower

TGA with VSD

arterial

switch procedure with VSD closure intracardiac repair

Rastelli-type
for

large and nonrestrictive VSD

done

when the coronary artery anatomy makes an arterial switch operation inadvisable

If

the infant has excessive congestive heart failure

reparative

surgery or, if not feasible, band/ligate the main pulmonary artery and place an aortopulmonary shunt during the newborn period to restrict pulmonary blood flow
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TGA with VSD and LV Outflow Tract Obstruction

arterial

switch operation may not be feasible due to pulmonary (left ventricular outflow tract) stenosis or atresia intracardiac repair
If

Rastelli

the VSD is nonrestrictive and not too remote from the aorta repair until the infant is older and larger may be preferable

delaying

placing an aortopulmonary shunt during the newborn period may be necessary to establish adequate pulmonary blood flow while waiting
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TGA with VSD and Pulmonary Vascular Obstructive Disease

these

patients might not be appropriate surgical candidates because of the progressive increase in pulmonary vascular resistance. is a small subgroup of patients whose conditions are not often diagnosed until after a palliative or reparative procedure is performed

this

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Prognosis
depends overall overall

on the specific anatomic substrate and type of surgical therapy used survival rate following arterial switch operation is 90%. mortality rate following an atrial level switch is low morbidity associated with systemic (right) ventricular dilatation and failure, systemic atrioventricular (tricuspid) valve regurgitation, and atrial bradyarrhythmias and tachyarrhythmias is significant
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long-term

Thank you!

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