Leung M P, Mok C K, Lee J, Lo R N, Cheung H, Chiu C
Department of Paediatrics, Grantham Hospital, University of Hong Kong, Aberdeen.
Am J Cardiol. 1993 Jun 1;71(15):1331-6. doi: 10.1016/0002-9149(93)90550-v.
To examine the impact on survival and clinical course of incorporating the morphologic classification of the right ventricle into the evolving management strategy for babies with pulmonary atresia and intact ventricular septum, the surgical results and follow-up status of the first 62 consecutive patients managed in this hospital between 1979 and 1990 were reviewed. Before 1984, all 23 babies from group I underwent primary right ventricular outflow reconstruction irrespective of right ventricular morphology and size. Since 1984, depending on the morphology and size of the right ventricle, 39 babies from group II had either closed transventricular pulmonary valvotomy (n = 31) or a shunt operation (n = 8). There were 10 hospital (43%) and 2 late deaths (total mortality 52%) in our group I patients. Three of the 11 long-term survivors had cyanosis at rest but none had any residual pressure gradient across the pulmonary outflow. Group II had 6 hospital (15%) and 4 late deaths (total mortality = 26%). Of the 29 long-term survivors, 9 had a second-stage right ventricular outflow reconstruction, 8 had balloon valvuloplasty and 2 had successful Fontan operation. At the latest follow-up, 5 children from this group have cyanosis at rest, 1 has a residual gradient (55 mm Hg) across the infundibulum, and 3 have right ventricular dysfunction. The hospital and total mortality for babies in group II was significantly lower than that in group I (p < 0.01). These data suggest that tailoring the treatment to the right ventricular anatomy results in a lower overall mortality although long term postoperative hemodynamic abnormalities are observed in both groups.
为研究将右心室形态学分类纳入不断发展的肺动脉闭锁合并完整室间隔患儿治疗策略中对生存及临床病程的影响，我们回顾了1979年至1990年间在本院连续治疗的首批62例患者的手术结果及随访情况。1984年前，I组的所有23例患儿均接受了初次右心室流出道重建术，而不考虑右心室的形态和大小。自1984年起，根据右心室的形态和大小，II组的39例患儿接受了经心室肺动脉瓣切开闭合术（n = 31）或分流手术（n = 8）。I组患者中有10例住院死亡（43%），2例晚期死亡（总死亡率52%）。11例长期存活者中有3例静息时出现青紫，但均无肺动脉流出道残余压力阶差。II组有6例住院死亡（15%），4例晚期死亡（总死亡率 = 26%）。29例长期存活者中，9例行二期右心室流出道重建术，8例行球囊瓣膜成形术，2例行成功的Fontan手术。在最近一次随访时，该组有5例患儿静息时出现青紫，1例漏斗部存在残余压力阶差（55 mmHg），3例有右心室功能障碍。II组患儿的住院死亡率和总死亡率显著低于I组（p < 0.01）。这些数据表明，根据右心室解剖结构调整治疗方案可降低总体死亡率，尽管两组术后均观察到长期血流动力学异常。
