Pediatric Cardiology and Cardiac Surgery
Vol.25 No.2 2009 (146-149)

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Yasuyuki Toyoda, Mitsuhiro Kimura, Takako Nishino, Shunji Uchita , and Yorikazu Harada
Department of Cardiovascular Surgery, Nagano Children’s Hospital, Nagano, Japan


We report a rare case of isolated pulmonary vein atresia in a 3-year-old girl who visited our hospital with a complaint of recurrent hemoptysis of undetermined origin. She had no other cardiac anomaly except for patent ductus arteriosus (PDA), which was closed by clip at 3 months of age. Enhanced computed tomography (CT) and cardiac catheterization demonstrated left pulmonary vein atresia at the drainage into the left atrium. Cardiac catheterization showed high left pulmonary capillary wedged pressure (PCWP) of 26 mmHg. We diagnosed high PCWP due to the pulmonary vein atresia that caused repeated hemoptysis. She underwent surgical repair for pulmonary vein atresia by anastomosis between the left pulmonary veins and the left atrial appendage under cardiopulmonary bypass. She was free from hemoptysis following the repair.