室间隔完整型肺动脉闭锁的个体化手术治疗

目的 探讨室间隔完整型肺动脉闭锁(PA/IVS)的个体化手术治疗.方法 1997年至2005年上海儿童医学中心外科治疗PA/IVS病例52例.(1)右心室依赖冠状动脉循环(RVDCC)组:共6例,均进行单心室修补.(2)非RVDCC组:46例.右心室重度发育不良(三尖瓣Z值＜-4)者13例,进行单心室修补.右心室轻、中度发育不良(三尖瓣Z值＞-4)者33例,除1例危重新生儿进行体肺分流外,另32例患儿均先进行右心室减压手术,右心室减压术后循环和血氧满意,完成双心室修补6例;对出现循环不稳定和/或低氧血症者,新生儿、小婴儿(0～2个月)增加体肺分流术13例;年龄≥3个月以上者增加腔肺分流术13例(1.5心室修补).结果 RVDCC组初期手术死亡1例,非RVDCC组初期手术死亡7例,总病死率15%.成功获得终期手术26例,占50%.随访1～10年,死亡3例.结论 依据患儿冠状动脉解剖、右心室发育程度和生长潜能以及手术中血流动力学和血氧情况,个体化地选择相应的手术方法,有利于提高患儿生存率.

Individualized surgical management of pulmonary atresia with intact ventricular septum

OBJECTIVE: To delineate individualized surgical management strategy for the optimal management of pulmonary atresia with intact ventricular septum (PA/IVS). METHODS: Between 1997 to 2005, 52 consecutive patients with PA/IVS, 31 male and 21 female, aged 2-9, divided into 2 groups according if right ventricle-dependent coronary circulation (RVDCC) existed, underwent individualized surgical treatment. The patients in Group I (with: RVDCC, n = 6) underwent single ventricle repair. Forty-six patients were in Group II (without RVDCC), 13 of which with severe right ventricular hypoplasia (tricuspid valve Z value < -4)underwent single ventricle repair and 33 of which were with mild to moderate right ventricular hypoplasia (Z value > -4). One critical neonate underwent systemic- pulmonary artery shunt. The other 32 of the 33 patients received right ventricular decompression procedures firstly, and 6 of them presented optimal hemodynamics and oxygenation and achieved biventricular repair. If the patients presented unstable hemodynamics or hypoxemia after right ventricular decompression procedure, an additional shunt was added. 13 young infants (0-2 months) underwent additional systemic-pulmonary artery shunt, and 13 patients (> 3 months-old) underwent additional bidirectional cavopulmonary shunt (1.5 ventricular repair)The patients were followed up for 1-10 years. RESULTS: There were 1 early death in Group I and 7 early deaths in Group II. The total early mortality was 15%. A successful definitive repair was achieved in 26 cases (50%). Follow-up reported 3 late deaths. CONCLUSION: Individualized surgical management based on the presence of RVDCC, right ventricular hypoplasia grading, right ventricular growth potential, hemodynamic situation, and oxygen saturation after the decompression procedure is helpful to improve the surgical results of PA/IVS