Abstract

Pulmonary atresia with an intact ventricular septum (PAIVS) is a rare congenital cardiac disease with various structural abnormalities. For patients with moderate right ventricular (RV) hypoplasia for whom the decision has not been made between biventricular and single ventricular repair, if possible, the ideal treatment choice is biventricular repair, by promoting the growth of the underdeveloped RV. Since RV sinus myectomy has been proposed, various surgical strategies have been introduced to promote RV growth. Herein, we described instances of RV sinus myectomy and the accompanying techniques for "RV overhaul." Furthermore, we introduced a current approach for small patients with PAIVS within an uncertain area.

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