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Does hypoplasia of one pulmonary artery preclude a definitive repair in pulmonary atresia,intact ventricular septum,and hypoplastic right ventricle?
Authors:Kiyoshi Suzuki  Katsuhiko Tatsuno  Shigekazu Mimori  Yasuo Murakami  Shouzaburoh Doi  Yukihiro Takahashi  Toshio Kikuchi  Katsuhiko Mori
Affiliation:(1) Department of Pediatric Cardiology, Sakakibara Heart Institute, 2-5-4 Yoyogi, Shibuya-ku, 151 Tokyo, Japan;(2) Department of Cardiac Surgery, Sakakibara Heart Institute, 2-5-4 Yoyogi, Shibuya-ku, 151 Tokyo, Japan;(3) Pediatric Department, Tokyo Medical and Dental University School of Medicine, 1-5-45 Yushima, Bunkyo-ku, 113 Tokyo, Japan
Abstract:
Summary Twins with pulmonary atresia, intact ventricular septum, and hypoplastic right ventricle associated with underdeveloped and discontinuous left pulmonary artery are described. Operations to connect the left pulmonary artery to the main pulmonary trunk, with subsequent biventricular repair assisted by bidirectional cavopulmonary anastomosis and atrial fenestration were performed. Follow-up catheterization proved spontaneous closure of the fenestration in one patient. Both patients have been in a satisfactory condition for 3 years since operation.
Keywords:Pulmonary atresia with intact ventricular septum  Hypoplasia of pulmonary artery  Twins with congenital heart disease  Biventricular repair  Bidirectional cavopulmonary anastomosis
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