Ishibashi Nobuyuki, Shin'oka Toshiharu, Ishiyama Masakuni, Sakamoto Takahiko, Kurosawa Hiromi
Department of Cardiovascular Surgery, Heart Institute of Japan, Tokyo Women's Medical University, Tokyo, Japan.
Eur J Cardiothorac Surg. 2007 Aug;32(2):202-8. doi: 10.1016/j.ejcts.2007.04.022. Epub 2007 May 23.
Our treatment strategy for pulmonary atresia with ventricular septal defect (VSD) and major aortopulmonary collateral arteries is a staged repair that comprises the first complete unifocalization (UF) with 'unification' of intrapulmonary arteries and then the definitive repair. The purpose of this study is to evaluate the outcome of our staged repair strategy with complete UF and to determine the results of our current management strategy.
From 1982 to 2004, 113 consecutive patients were treated with staged repair at our institute. We evaluated the risk of definitive repair failure or death in the 3 years after definitive repair using logistic regression. Furthermore, we compared the early group (patients who underwent UF before December 1995) and the late group (patients who underwent UF after January 1996).
The mean follow-up interval was 8.8 years (0.8 months to 23.3 years), and Kaplan-Meier-estimated overall survival rates after first UF were 80.9, 73.8, and 69.9% at 5, 10, and 15 years, respectively. Survival in patients with an absent central pulmonary artery (PA) was significantly lower than in those with a central PA (p<0.05), and the factor that was significantly associated with definitive repair failure or death in the 3 years after definitive repair was central PA morphology (p<0.05). Higher mean PA pressure after UF was detected in patients with hypoplastic central PA, compared with those without hypoplastic PA (30.9 mmHg vs 23.3 mmHg, p<0.05). In the late group, age (in years) at first UF (3.9 vs 8.4, p<0.01), second UF (4.3 vs 9.2, p<0.01), and definitive repair (5.8 vs 9.1, p<0.01) was significantly younger than in early group, and the survival rate after first UF in the late group was 96.2 and 91.3% at 3 and 7 years, respectively. Systolic right ventricular pressure and the pressure ratio between the right and the left ventricles after definitive repair in the late group were significantly lower than in the early group (53.6 mmHg vs 75.0 mmHg, p<0.01; 61.7% vs 75.9%, p<0.05).
Hypoplastic central PA was a significant risk factor in this disease. The overall survival was improved by our current management strategy. Improved RV pressure after definitive repair appears to affect the long-term outcome.
我们针对室间隔缺损（VSD）合并主-肺动脉侧支动脉的肺动脉闭锁的治疗策略是分期修复，包括首次完全单源化（UF），即肺内动脉“统一”，然后进行最终修复。本研究的目的是评估我们采用完全UF的分期修复策略的结果，并确定我们当前管理策略的效果。
1982年至2004年，我院连续113例患者接受了分期修复治疗。我们使用逻辑回归评估最终修复后3年内最终修复失败或死亡的风险。此外，我们比较了早期组（1995年12月前接受UF的患者）和晚期组（1996年1月后接受UF的患者）。
平均随访时间为8.8年（0.8个月至23.3年），首次UF后采用Kaplan-Meier法估计的5年、10年和15年总生存率分别为80.9%、73.8%和69.9%。中央肺动脉（PA）缺如的患者生存率显著低于有中央PA的患者（p<0.05），与最终修复后3年内最终修复失败或死亡显著相关的因素是中央PA形态（p<0.05）。与中央PA无发育不全的患者相比，中央PA发育不全的患者UF后平均PA压力更高（30.9 mmHg对23.3 mmHg，p<0.05）。在晚期组中，首次UF时的年龄（岁）（3.9对8.4，p<0.01）、第二次UF时的年龄（4.3对9.2，p<0.01）和最终修复时的年龄（5.8对9.1，p<0.01）显著低于早期组，晚期组首次UF后3年和7年的生存率分别为96.2%和91.3%。晚期组最终修复后的收缩期右心室压力以及右心室与左心室之间的压力比显著低于早期组（53.6 mmHg对75.0 mmHg，p<0.01；61.7%对75.9%，p<0.05）。
中央PA发育不全是该疾病的一个重要危险因素。我们目前的管理策略提高了总体生存率。最终修复后右心室压力的改善似乎影响长期预后。
