CAJ | 학술논문

目的评价合并内脏异位综合征先天性心脏病外科治疗的效果.方法 2008年9月至2013年10月,34例内脏异位综合征合并心脏畸形患儿(婴)行手术治疗46例次.男22例,女12例;年龄4个月～14岁,中位年龄22个月.右心房异构24例,左心房异构10例;1例为单纯室间隔缺损,33例伴复杂心内畸形,其中完全性房室间隔缺损26例,肺静脉异位引流18例,双上腔静脉17例,下腔静脉中断5例,三尖瓣闭锁4例,肺静脉梗阻4例,二尖瓣闭锁1例.32例采用单心室矫治手术,其中双向Glenn术13例次,肺动脉环缩术5例次,Fontan手术5例次,瓣膜置换术5例次,双侧双向Glenn术4例次,Kawashima手术3例次,中央分流术1例次;9例采用多种术式.结果住院死亡4例;随访1个月～5年,死亡2例,早、中期死亡比例17.6％(6/34),与同期103例未合并内脏异位综合征行单心室系列手术患儿(婴)早、中期死亡比例4.8％(5/103)差异显著(P＜0.05).结论单心室矫治系列手术是治疗内脏异位合并心内复杂畸形的主要手术方式,术后早、中期效果良好.
목적 평개합병내장이위종합정선천성심장병외과치료적효과.방법 2008년9월지2013년10월,34례내장이위종합정합병심장기형환인(영)행수술치료46례차.남22례,녀12례;년령4개월～14세,중위년령22개월.우심방이구24례,좌심방이구10례;1례위단순실간격결손,33례반복잡심내기형,기중완전성방실간격결손26례,폐정맥이위인류18례,쌍상강정맥17례,하강정맥중단5례,삼첨판폐쇄4례,폐정맥경조4례,이첨판폐쇄1례.32례채용단심실교치수술,기중쌍향Glenn술13례차,폐동맥배축술5례차,Fontan수술5례차,판막치환술5례차,쌍측쌍향Glenn술4례차,Kawashima수술3례차,중앙분류술1례차;9례채용다충술식.결과 주원사망4례;수방1개월～5년,사망2례,조、중기사망비례17.6％(6/34),여동기103례미합병내장이위종합정행단심실계렬수술환인(영)조、중기사망비례4.8％(5/103)차이현저(P＜0.05).결론 단심실교치계렬수술시치료내장이위합병심내복잡기형적주요수술방식,술후조、중기효과량호.
Objective To evaluate the results of surgical treatment for patients with heterotaxy syndrome and cardiac anomaly.Methods From September 2008 to October 2013,34 patients with heterotaxy syndrome underwent 46 cardiac operations at Guangzhou Women and Children Medical Center.22 were male,and 12 female.Mean age at operation was 22 months (4 months to 14 year).There were right atrial isomerism 24 cases,left atrial isomerism 10 cases.Only one had a single VSD,and others were all with complex cardiac anomaly,including complete atrioventricular canal defect 26 cases,tricuspid atresia 4 cases,mitral atresia 1 case,anomalous pulmonary venous connection 18 cases,pulmonary venous stenosis 4 cases,double superior vena cava 17 cases,and interrupted hepatic portion of the inferior vena cava 5 cases.Fontan procedure was performed in 5 patients,bidirectional Glenn in 13,Kawashima in 3,central shunt in 1,atrioventricular valve replacement in 5,and pulmonary artery banding in 5.Results There were 4 died during hospitalization.The follow-up duration was 1 month to 5 years.There were 2 died.The early to middle term mortality was 17.6％ (6/34).In the same period the mortality for 103 patients without heterotaxy syndrome underwent univentricular repair was 4.8％ (5/103).There was significant statistical difference (P ＜ 0.05).Conclusion Right heart bypass operation remains the preferred palliative procedure for patients with heterotaxy syndrome,and the eraly and middle term results were satisfied.