Congenital Heart Disease part 7


Heart Illustration


Pulmonary atresia with ventricle septal defect, sometimes also called tetralogi of Fallot with pulmonary atresia, shares the intracardiac anatomy of tetralogy but lacks a direct communication between the right ventricle and pulmonary artery.  Patients with pulmonary artery and ventricle septal defect have a good size right ventricle, and from this perspective are suitable biventricular repair, major problems with pulmonary artery are common and determine both clinical presentation and management (the complexity of the pulmonary vascular bed may make repair unattractive or impossible).

Early clinical presentation varies between cyanosis (for patients with reduced or threathened pulmonary blood flow), failure to thrive, and/or exertional dyspnoea, to heart failure (for patients with execessive pulmonary blood flow through large major aortic pulmonary collaterals, who may develop segmental pulmonary arterial hypertension with time).

When pulmonary blood flow is is duct dependent, profound cyanosis and cardiovascular collapse ensue as the…

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