CAJ | 학술논문

目的探讨肺动脉闭锁伴室间隔缺损(PA/VSD)姑息手术方法选择、二期根治手术时机及方法.方法 2004年4月至2008年7月,上海交通大学医学院附属上海儿童医学中心50例PA/VSD患儿进行二期根治手术.初期姑息手术体肺分流7例,其中改良Blaloek.Taussig分流术5例,中央分流术2例;流出道重建43例,其中心包补片扩大10例、心包管道4例和Gore-tex管道连接29例.手术间隔时间7-49(20±10)个月.二期手术体肺分流切断7例;所有患儿进行VSD修补、但补片开窗6例;心包加宽流出道和肺动脉成形42例,更换外管道8例;主肺侧支动脉(MAPCAS)单源化1例,结扎或心导管封堵4例,保持开放或旷置4例.结果早期死亡2例,病死率4%;术后残余分流3例,残余梗阻3例,完全传导阻滞1例,手足徐动症1例,急性肾衰竭3例.随访3个月～4年,无死亡和并发症发生.结论依据患儿肺动脉形态和临床状况个体化选择姑息手术方法.二期手术保证右心室流出道及肺动脉血流通畅、右室高压患儿VSD补片开窗以及对MAPCAS恰当处理,是降低PA/VSD分期手术病死率和并发症率的关键.
목적 탐토폐동맥폐쇄반실간격결손(PA/VSD)고식수술방법선택、이기근치수술시궤급방법.방법 2004년4월지2008년7월,상해교통대학의학원부속상해인동의학중심50례PA/VSD환인진행이기근치수술.초기고식수술체폐분류7례,기중개량Blaloek.Taussig분류술5례,중앙분류술2례;류출도중건43례,기중심포보편확대10례、심포관도4례화Gore-tex관도련접29례.수술간격시간7-49(20±10)개월.이기수술체폐분류절단7례;소유환인진행VSD수보、단보편개창6례;심포가관류출도화폐동맥성형42례,경환외관도8례;주폐측지동맥(MAPCAS)단원화1례,결찰혹심도관봉도4례,보지개방혹광치4례.결과 조기사망2례,병사솔4%;술후잔여분류3례,잔여경조3례,완전전도조체1례,수족서동증1례,급성신쇠갈3례.수방3개월～4년,무사망화병발증발생.결론 의거환인폐동맥형태화림상상황개체화선택고식수술방법.이기수술보증우심실류출도급폐동맥혈류통창、우실고압환인VSD보편개창이급대MAPCAS흡당처리,시강저PA/VSD분기수술병사솔화병발증솔적관건.
Objective To determine the choice of palliative procedures, timing and techniques of second-stage operations. Methods Between April 2004 to July 2008, 50 consecutive patients with pulmonary atresia with ventricular septal defect ( PA/VSD) underwent two-stage operation. Palliative procedures included modified Blaloclc-Taussig shunt (n - 5 ) , central shunt ( n = 2 ) , pericardia] patch enlargement (n = 10), pericardia! tube (n =4) and Gore-Tex conduit (n =29). The interval period was 7-49 months (20. 0 ± 10. 0 months) . In the second stage, a surgical shunt was interrupted in 7 cases. Ventricular septal defect was closed in all patients, but fenestrated ventricular septal defect patch was used in 6 cases. Right ventricular outlet tract ( RVOT) was widened with pericardial patch in 42 cases and conduit exchange in 8 cases. Aortopulmonary collateral arteries ( MAPCASs) unifocalization ( n = 1) , ligation or transcatheter occlusion with embolization coils ( n = 4 ) and maintaining open or untreated (n = 4 ). Results Death occurred in 2 and the mortality rate was 4% . Postoperative complications included residual shunt (n = 3 ) , residual obstruction ( n = 3 ) , complete AV block ( n = 1) , athetosis (n = 1 ) and acute renal failure (n = 3). Neither death nor complication was reported during a follow-up period of 3 months to 4 years. Conclusion A palliative procedure should be individualized to the patient's morphology of central pulmonary artery and clinical status of a patient. Right ventricular outlet tract reconstruction, pulmonary arterioplasty, fenestration of VSD patch in baby with suprasystemic right ventricular pressure and appropriate interventions with MAPCASs are key to decrease the mortality and morbidity of staged operations for PA/ VSD.