Right ventricle-pulmonary artery shunt in first-stage palliation of hypoplastic left heart syndrome
Right ventricle-pulmonary artery shunt in first-stage palliation of hypoplastic left heart syndrome
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DOI：
10.1016/s0022-5223(02)73575-7
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发表时间：
2003-08-01
影响因子：
6
通讯作者：
Ohtsuki, S
中科院分区：
文献类型：
--
作者：
Sano, S;Ishino, K;Ohtsuki, S
目的：通过体肺分流引起的肺过度循环是诺伍德手术后早期死亡的主要原因之一。为了避免这种致命的并发症，我们在左心发育不良综合征的一期姑息治疗中建立了右心室-肺动脉分流术。1998年2月至2002年2月，连续19名6至57天的婴儿，（中位数，9天），体重1.6至3.9 kg（中位数，3.0 kg），接受了改良的诺伍德手术，右心室-肺动脉分流。该手术包括通过直接吻合近端主肺动脉和小右心室切开术与主肺动脉远端残端之间的无瓣膜聚四氟乙烯分流术进行主动脉重建。分流管直径为4 mm者5例，5 mm者14例。结果：所有患者均顺利完成手术，无肺血管阻力控制措施。有17名幸存者（89%），包括3名体重小于2 kg的患者。两例晚期死亡是由于右心室-肺动脉分流道阻塞。13例患者在平均间隔6个月后接受了11期Glenn手术，2例住院死亡。迄今为止，已在4名患者中完成了III期Fontan手术。总生存率为62%（13/19）。在最后一次随访（第1阶段后3-48个月）时，右心室缩短分数范围为26%至43%（n = 13，平均值为33%hosclero.Conclusion：没有精细的术后管理，以控制肺血管阻力，改良诺伍德程序使用右心室-肺动脉分流提供了一个稳定的体循环，以及足够的肺血流量。这种新的手术可能对患有左心发育不良综合征的低出生体重儿特别有益。
Objective: Pulmonary overcirculation through a systemic-pulmonary shunt has been one of the major causes of early death after the Norwood procedure. To avoid this lethal complication, we constructed a right ventricle-pulmonary shunt in first-stage palliation of hypoplastic left heart syndrome.Methods: Between February 1998 and February 2002, 19 consecutive infants, aged 6 to 57 days (median, 9 days) and weighing 1.6 to 3.9 kg (median, 3.0 kg), underwent a modified Norwood operation with the right ventricle-pulmonary artery shunt. The procedure included aortic reconstruction by direct anastomosis of the proximal main pulmonary artery and a nonvalved polytetrafluoroethylene shunt between a small right ventriculotomy and a distal stump of the main, pulmonary artery. The size of the shunt used was 4 mm in 5 patients and 5 mm in 14.Results: All patients were managed without any particular manipulation to control pulmonary vascular resistance. There were 17 survivors (89%), including 3 patients weighing less than 2 kg. Two late deaths occurred due to obstruction of the right ventricle-pulmonary artery shunt. Thirteen patients underwent a stage 11 Glenn procedure after a mean interval of 6 months, with 2 hospital deaths. To date, a stage III Fontan procedure has been completed in 4 patients. Overall survival was 62% (13/19). Right ventricular fractional shortening at the last follow-up (3-48 months after stage 1) ranged from 26% to 43% (n = 13, mean, 33%).Conclusion: Without delicate postoperative management to control pulmonary vascular resistance, the modified Norwood procedure using the right ventricle-pulmonary shunt provides a stable systemic circulation as well as adequate pulmonary blood flow. This novel operation may be particularly beneficial to low-birth-weight infants with hypoplastic left heart syndrome.