Pediatric Cardiology and Cardiac Surgery
Vol.26 No.5 2010 (392-399)

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Takako Miyazaki,1) Masaaki Yamagishi,1) Masafumi Yashima,1) Yoshinobu Maeda,1) Yusuke Yamamoto,1) Masaaki Koide,2) Koji Nomura,3) and Hitoshi Yaku4)

1)Department of Pediatric Cardiovascular Surgery, Children’s Research Hospital Kyoto Prefectural University of Medicine, Kyoto, 2)Department of Cardiovascular Surgery, Seirei Hamamatsu General Hospital, Shizuoka, 3)Department of Cardiovascular Surgery, Saitama Children’s Medical Center, Saitama, and 4)Department of Cardiovascular Surgery, Kyoto Prefectural University of Medicine, Kyoto


Background: We advocate one-stage unifocalization concomitant with palliative right ventricular outflow tract reconstruction (pRVOTR) for patients with major aortopulmonary collateral arteries (MAPCA) in pulmonary atresia with ventricular septal defects. The object of this study was to assess our surgical strategy for MAPCA.
Methods: From 2001 to 2009, through a median sternotomy, one-stage unifocalization under cardiopulmonary bypass was performed in 15 patients. The median age at the operation was 1.0 year. The central pulmonary arteries were created by using an autologous pericardium roll/patch. Definitive repair was performed in two patients, and staged repair with pRVOTR (right ventricular to pulmonary artery conduits with a diameter of 75% of the normal pulmonary annulus diameter) was performed in 13 patients. Before the operation, the surgical strategy for unifocalization of MAPCA and pulmonary reconstruction was planned using accurate spatial information from the differential color imaging provided by 3D CT angiography.
Results: There were no hospital deaths or late deaths; 14 patients achieved definitive repair, and 1 patient is waiting for further follow-up (average: 3.5 years, range: 6.2 months to 8.2 years). The pulmonary artery index and average SpO2 were 139.7±88.6 mm2/m2 and 79%, which initially and significantly increased to 398.5±128.2 mm2/m2 and 88% after unifocalization. The pulmonary arterial systolic pressure was 35.3±14.1 mmHg before definitive repair and decreased to 29.3±2.2 mmHg after definitive repair.
Conclusions: One-stage unifocalization and pRVOTR showed excellent results for all patients with MAPCA. Bilateral equal pulmonary blood flow was obtained and led to the growth of pulmonary arteries with this procedure. Plotting a thorough strategy is crucial for the successful treatment of MAPCA.