ABSTRACT
Pulmonary atresia with intact ventricular septum (PA/IVS) is difficult to manage. The prognosis for this rare malformation (approximately 1:22000 live births) has improved, partly related to the use of prostaglandin (PGE1) infusion to maintain the duct-dependent pulmonary circulation. Another important contribution is a better understanding of right ventricle (RV) morphology and its use in the management strategy. The ideal approach is to restore continuity between RV and pulmonary artery (PA), when the RV is adequate for supporting the pulmonary circulation. This also decompresses the hypertensive RV and may promote growth of the hypoplastic cavity. Precise management in an individual patient will be governed by the varying degrees of RV hypertrophy/cavity hypoplasia, tricuspid valve (TV) dysplasia and RV-dependent coronary circulation.
