Pediatric Cardiology and Cardiac Surgery
Vol.27 No.2 2011 (69-75)

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Masaaki Yamagishi

Department of Pediatric Cardiovascular Surgery, Children’s Research Hospital, Kyoto Prefectural University of Medicine, Kyoto, Japan.


Because of the wide spectrum of right ventricular morphology, the optimal management of infants with pulmonary atresia with an intact ventricular septum (PA/IVS) still remains controversial. Sinusoidal communication and right-ventricle-dependent coronary circulation have a significant impact on the surgical outcome. The presence of right-ventricle-dependent coronary circulation is a contraindication for right ventricle decompression. The initial palliation for PA/IVS consists of pulmonary valvotomy, with or without modified Blalock-Taussig (BT) or BT shunt only. For definitive surgery, biventricular repair is performed in patients with more than 70% of the normal size of the right ventricle and tricuspid annular diameter. The Fontan procedure or one-and-a-half repair is recommended in patients with mild to moderate hypoplasia of the right ventricle and tricuspid annular diameter (30% to 70% of normal). The Fontan procedure should be performed in patients when the right ventricle and tricuspid annular diameter is less than 30% of the normal size.