儿童右室双出口的外科治疗体会

Surgical treatment experience of double outlet right ventricle in children

目的总结儿童右室双出口的外科治疗体会。方法2009年2月至2017年3月共手术治疗87例患儿，男性48例，女性39例，年龄10d至18岁，平均（30．0±44．4）个月，体重3．17-47（11．1±8．8）k。根据STS-EACTS数据库分类：VSD型43例，TOF型7例，TGA型9例，VSD远离大动脉型28例。共经历手术100例次，非体外循环下手术30例次，体外循环下手术70例次。体外循环时间32-234（103．3±49．3）min，主动脉阻断时间12-180（59．6±37．2）min。VSD型手术方法包括心室内隧道补片修补33例，PA Banding术5例，B-T分流术1例，双向Glenn术7例，改良Fontan术1例，术后出现右心室流出道狭窄行右心室流出道重建术3例。TOF型手术方法为心室内隧道补片修补±右室流出道疏通术。TGA型手术方法均为大动脉调转术，同期行IAA（A）矫治术I例，术后出现右心室流出道狭窄行右心室流出道重建术4例。VSD远离大动脉型手术方法包括B-T分流术4例，PA Banding术5例，双向Glenn术17例，改良Fontan术4例。术后呼吸机时间4-360（46．4±60．6）h，监护室时间6h至21d，平均（127．2±81．6）h，住院时间11-39（23．3±12．1）d。结果100例次手术术后呼吸机时间平均（46．4±60．6）h，监护室时间平均（127．2±81．6）h，住院时间平均（23．3±12．1）d。84例患儿顺利存活。住院死亡3例，手术死亡率3％，1例B-T分流术后低氧血症，1例术后低心排综合征，1例术后心功能不全、肾功能不全及肝功能不全，经腹透及血液滤过治疗后并发颅内出血伴昏迷，家长要求放弃治疗而死亡。其余患儿效果良好。随访12个月至5年，无晚期死亡病例。结论DORV疾病谱广，病种复杂，术前需充分评估病情，根据患儿的解剖特点和年龄制订个体化的手术方案，可以获得比较满意的手术效果。

Objective To explore surgical treatment experience of double outlet right ventricle in children. Methods From February 2009 to March 2017, 87 patients with DORV underwent surgical repair at this center. Among the 87 patients, 48 were males and 39 were females. There ages ranged from 10 days to 18 years old, mean age （30.0±44.4）months. Their weight ranged from 3.17-47 kg （11.1±8.8）kg. According to STS-EACTS international nomenclature： 43 patients were DORV associated with ventricular septal defect （VSD）, 7 were DORV with tetralogy of Fallot （TOF）, 9 were DORV with transposition of the great arteries （TGA）, and 28 were DORV with remote VSD. Total surgery times were 100, 70 cases underwent extracorporeal circulation, the extracorporeal circulation time ranged from 32-234 （103.3±49.3）minutes, the aorta blocking time ranged from 12-180 （59.6± 37.2）minutes. 30 cases were underwent non extracorporeal circulation. The surgical methods of DORV associated with ventricular septal defect（VSD） including the heart tunnel patch repair in 33 cases; the PA Banding operation in 5 cases; the B-T bypass operation in 1 case; the bidirectional Glenn operation in 7 cases; the Modified Fontan operation in 1 case; ventricle outflow tract reconstruction after surgery in 3 cases. The surgical methods of DORV with tetralogy of Fallot（TOF） were heart tunnel patch repair plus ventricle outflow tract reconstruction. The surgical method of DORV with transposition of the great arteries（TGA ） was switch surgery, 1 case underwent IAA （A） surgical rectifying at the same time, ventricle outflow tract reconstruction after surgery in 4 cases. The surgical methods of DORV with remote VSD including B-T bypass operation in 4 cases; the PA Banding operation in 5 cases; the bidirectional Glenn operation in 17 cases; the modified Fontan operation in 4 cases. The Postoperative respiratory machine time ranged from 4-360 （46.4±60.6）hours, the time in CICU ranged from 6 hours to 21 days, mean time（127.2±81.6）hours, the discharge time ranged from 11-39（23.3±12.1）days. Results Of the 100 cases, the postoperative respiratory machine time ranged from 4-360 （46.4±60.6）hours, the time in CICU ranged from 6 hours to 21 days, mean time（ 127.2±81.6）hours, the discharge time ranged from 11-39（23.3±12.1）days. 84 cas- es were survived, 3 patients died after surgery, the operation mortality is 3%. 1 case underwent hypoxemia after B-T bypass operation; 1 case underwent low cardiac output syndrome postoperative; 1 case underwent cardiac insufficiency, renal insufficiency, and hepatic insufficiency postoperative, raised intracranial hemorrhage and coma after treatment of peritoneal dialysis and hemofiltration, the parents requested treatment and die. The rest of the patients with good effect, follow up for 12 months to 5 years, no late deaths. Conclusion DORV has numerous of ariations and complexity, need to fully evaluate the condition preoperative, to formulate individual operation plan according to the anatomical characteristics and the age of the patients, it can obtain satisfactory surgery results.