Koji Kagisaki, Toshikatsu Yagihara, Ikuo Hagino, Shuichi Shiraishi, Fumiaki Shikata, Yuki Nakamura, Junjiro Kobayashi, Soichiro Kitamura; Cardiovascular Surgery, National Cardiovascular Center, Suita, Japan
Objective: We introduced staged unifocalization through thoracotomy in 1985 and complete unifocalization through median sternotomy in 1992 for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA/VSD/MAPCA). However, late outcome of the unifocalized pulmonary vasculature is still controversial. We assessed the long-term outcome after definitive repair for PA/VSD/MAPCA in the patients who have more than 10-year follow-up after the first unifocalization.Methods: Among 65 patients who underwent first unifocalization from 1985 to 1996 (62: through thoracotomy, 3: through median sternotomy), definitive repair was performed in 50 patients including 2 one stage repair. Age at definitive repair ranged from 1 to 34 years (7.0 ± 7.9 years). Each patient had 1 - 7 MAPCAs (mean 3.8 ± 1.5). The number of the unifocalized pulmonary vascular segments was 9 - 18 (mean 15.8 ± 2.4). Right ventricular outflow tract was reconstructed with a valved conduit in 44 patients and with a transannular patch in 6 patients.
Results: There were 5 hospital deaths and 9 late deaths. The follow-up period ranged from 7.3 to 21 years (mean: 12.6 ± 4.0). Actuarial survival at 5, 10 and 15 years was 80%, 73% and 69%, respectively. Early postoperative (1 year) mean pulmonary arterial pressure was significantly higher in patients with late deaths than in survivors (44.2±23.7 vs. 22.7±8.5 mmHg p=0.001). Pulmonary arterial pressure was correlated with unifocalized pulmonary segments (r2=0.17, p=0.024). Fifteen patients underwent re- right ventricular outflow tract reconstruction at 9.5 ± 3.4 years after definitive repair. Three patients underwent aortic valve replacement. Actuarial freedom from reoperation at 5, 10 and 15 years was 98%, 72% and 68%, respectively. Serial change of mean pulmonary arterial pressure, pulmonary vascular resistance, right ventricular end-diastolic volume and right ventricular ejection fraction in long-term survivors revealed acceptable pulmonary vasculature and good right ventricular function (shown in the table).
Conclusion: The number of the unifocalized pulmonary segments correlated with the mortality and pulmonary vascular resistance after definitive repair for PA/VSD/MAPCA. Performance of unifocalized pulmonary vasculature and right ventricular function were acceptable in the majority of long-term survivors for more than 10-year follow-up.
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