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Re: A 'Persistent' Problem with Pulmonary Atresia and Intact Ventricular Septum

Dear Editor

We read with interest the case report by Codispoti et al, depicting an association of pulmonary atresia and intact inter-ventricular septum (PAIS) with persistent pulmonary hypertension of the newborn. In our study of 24 autopsied cases of PAIS, we found four cases (16.7%) with persistence of fetal circulation. All were a few days old with varying degrees of ventricular hypoplasia and tricuspid stenoses. As a protocol followed at our centre, sections of the lungs (average six) are studied in all autopsied congenital heart diseases and hence we feel that this association may not be as uncommon as suggested by the authors.

Taking into consideration the physiology of PAIS, the patent ductus arteriosus supplies the pulmonary arterial tree in a retrograde fashion and is responsible for the infrequency of pulmonary arterial hypoplasia and development of collaterals. However, with persistent pulmonary hypertension of the newborn, the retrograde blood flow would decrease and there may even be a right to left shunt at the ductal level. Hence such neonates will not only have a bad post operative course after correction as occurred in the authors' case but would also have higher pre-operative morbidity. It would, therefore, be important to consider them as a subset of PAIS.

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