In this review, we approach the main morphologic and developmental aspects of the congenital cardiovascular malformation known as tetralogy of Fallot with pulmonary atresia. It is recognized that pulmonary atresia associated with a deficient ventricular septation can occur in several situations. However, the tetralogy presentation in particular, with frequent but not invariable concomitance of systemic-to-pulmonary collateral arteries supplying the lungs entirely or in part, poses surgical challenges. The variations in the morphology of such collateral arteries and the intracardiac anatomy are discussed.
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