CAJ | 학술논문

目的探讨儿童Ebstein畸形的外科治疗方法.方法回顾性分析儿童Ebstein畸形的外科治疗.自1992年8月至2010年8月共外科治疗Ebstein畸形31例,其中男13例,女18例,年龄6个月至15岁,平均(6.9±1.6)岁,体重5.5～53kg,平均(19.6±4.7)kg.心功能(NYHA)Ⅰ级9例,Ⅱ级15例,Ⅲ级7例.31例均经彩色多普勒超声心动图确诊,隔瓣、后瓣下移1.5~6.5cm,6例伴不同程度前瓣下移.三尖瓣轻度反流4例,三尖瓣中度反流16例,重度反流11例.本组2例无合并畸形,合并房间隔缺损20例,卵圆孔未闭9例,室间隔缺损3例,肺动脉瓣狭窄2例,三尖瓣狭窄2例,动脉导管未闭1例.手术方法全组均在体外循环下行三尖瓣成形术,23例应用Carpentier法,Carpentier分类A型1例,B型16例,C型6例,其中5例加行双向格林术,1例留有3mm房间隔缺损;8例应用Danielson法,Carpentier分类A型2例,B型6例,其中2例加行双向格林术,并对两种方法进行对比分析.结论 Carpentier法术后无死亡,术后心功能均为Ⅰ级,复查超声心动图示9例三尖瓣无明显反流,13例轻度反流,1例中度反流.Danielson法死亡1例,死亡原因为术后心律失常、持续低氧血症;术后存活病例心功能均为Ⅰ级,3例三尖瓣无明显反流,3例轻度反流,2例中度反流;1例患儿并发Ⅲ度房室传导阻滞,术后3个月转为窦性心律.结论 Ebstein畸形的变异较大,治疗须慎选手术方法,Carpentier法较为理想,同时根据三尖瓣结构和右室功能决定是否加行双向格林术.
목적 탐토인동Ebstein기형적외과치료방법.방법 회고성분석인동Ebstein기형적외과치료.자1992년8월지2010년8월공외과치료Ebstein기형31례,기중남13례,녀18례,년령6개월지15세,평균(6.9±1.6)세,체중5.5～53kg,평균(19.6±4.7)kg.심공능(NYHA)Ⅰ급9례,Ⅱ급15례,Ⅲ급7례.31례균경채색다보륵초성심동도학진,격판、후판하이1.5~6.5cm,6례반불동정도전판하이.삼첨판경도반류4례,삼첨판중도반류16례,중도반류11례.본조2례무합병기형,합병방간격결손20례,란원공미폐9례,실간격결손3례,폐동맥판협착2례,삼첨판협착2례,동맥도관미폐1례.수술방법전조균재체외순배하행삼첨판성형술,23례응용Carpentier법,Carpentier분류A형1례,B형16례,C형6례,기중5례가행쌍향격림술,1례류유3mm방간격결손;8례응용Danielson법,Carpentier분류A형2례,B형6례,기중2례가행쌍향격림술,병대량충방법진행대비분석.결론 Carpentier법술후무사망,술후심공능균위Ⅰ급,복사초성심동도시9례삼첨판무명현반류,13례경도반류,1례중도반류.Danielson법사망1례,사망원인위술후심률실상、지속저양혈증;술후존활병례심공능균위Ⅰ급,3례삼첨판무명현반류,3례경도반류,2례중도반류;1례환인병발Ⅲ도방실전도조체,술후3개월전위두성심률.결론 Ebstein기형적변이교대,치료수신선수술방법,Carpentier법교위이상,동시근거삼첨판결구화우실공능결정시부가행쌍향격림술.
Objective To study the surgical treatment for Ebstein's anomaly in children.Methods From August 1992 to August 2010,there were 31 cases of Ebstein's anomaly in children underwent surgical repair,including thirteen males and eighteen females,aged from 6 months to fifteen years[mean(6.9±1.6) years] and the weight was from 5.5 to 53.5 kg[mean(19.6±4.7)kg].Eight patients underwent Danielson repair of tricuspid valve,among these 2 patients were added bidirectional Glenn operation;23 patients underwent Carpentier repair of tricuspid valve,among these 5 patients were added bidirectional Glenn operation.The results of 31 cases of Ebstein's anomaly in children were retrospectively analyzed.Results There was no postoperative death with Carpentier repair of tricuspid valve.All survivals were in New York Heart Association class Ⅰ.Postoperative tricuspid regurgitation was markedly improved,echocardiography showed disappearance of tricuspid incompetence in 9 patients,mild incompetence in 13 and moderate incompetence in 1.There was one postoperative death with Danielson repair of tricuspid valve,and main causes of death were tachyarrhythmia and continuance hypoxia.All survivals were in New York Heart Association class Ⅰ.Postoperative echocardiaography showed disappearance of tricuspid incompetence in 3 patients,mild competence in 3 and moderate mild competence in 2. No reoperation for residual or recurrent tricuspid incompetence was required in all survivals. Conclusions In spite of the variable degree of Ebstein's anomaly present,the majority of patients can undergo a surgical repair.Tricuspid reconstruction should be the first choice of surgical procedures whenever possible in children.Carpentier repair of tricuspid valve is the best procedure at present.The bidirectional Glenn could be adopted according to the function of tricuspid valve and right ventricle.