368例右室双出口的解剖特点与双心室矫治术

Anatomy variation and biventricular repair in 368 patients with Double Outlet Right Ventricle

目的通过分析右室双出口(DORV)的解剖类型、合并心脏畸形及双心室矫治方法 ,指导临床诊断,探讨个体化手术方案。方法回顾368例经双心室矫治术证实的DORV,分析手术诊断和矫治方法。结果 (1)解剖类型:室缺位于主动脉下95例,肺动脉下44例,双动脉下13例,远离两大动脉98例。大动脉位置关系正常40例,左右并列关系51例,右位型主动脉101例,左位型主动脉43例。主动脉骑跨142例,肺动脉骑跨36例,双动脉骑跨2例,两大动脉完全发自右室157例。(2)合并心脏畸形共862处,其中常见畸形依次为肺动脉狭窄223例,动脉导管未闭99例,房间隔缺损93例,右位主动脉弓68例,永存左上腔静脉64例,冠状动脉畸形60例,镜面右位心24例。(3)手术方法 :心室内隧道修补术(IVR)270例,大动脉调转术(ASO)59例,Rastelli术14例,改良REV术7例,Nikaidoh术7例,双动脉根部调转术(DRT)11例,同期行Senning术6例,Mustard术1例。结论 DORV解剖类型复杂多样,可合并多种畸形,有多种矫治方法。只有早期准确诊断DORV,才能在合适的手术时机,根据不同的解剖特点,制定最佳的个体化手术方案。

Objective We dissected the anatomy variation of DORV combined with cardiac anomalies or not and the method of biventricular repair to seek complementary for diagnosis and a personalized therapy. Methods We analyzed retrospectively the diagnosis and therapy of 368 patients with DORV confirmed by biventricular repair. Results The anatomy characteristics demonstrated that the subaortic VSD was found in 95 patients, subpulmonary VSD in 44 patients, doubly committed VSD in 13 patients and noncommitted VSD in 98 patients. The normal relationship of the great arteries was found in 40 patients, right position of the aorta in 51 patients, D-malposition of the aorta in 101 patients and L-malposition of the aorta in 43 patients. The aortic overriding was found in 142 patients, pulmonary overriding in 36 patients, both the great arteries overriding in 2 patients and both the great arteries deriving from the right ventricle in their entirety in 157 patients. There were 862 combined cardiac anomalies, including pulmonary stenosis in 223 cases, patent ductus arteriosus in 99 cases, atrial septal defect in 93 cases, right aortic arch in 68 cases, persistent left superior vena cava in 64 cases, coronary anomalies in 60 cases and mirror dextrocardia in 24 cases. Total 270 patients underwent intraventricular tunnel repair, 59 arterial switch operation, 14 Rastelli operation, 7 modified REV procedure, 7 Nikaidoh operation and 11 double-root translocation. Meanwhile, 6 patients underwent Senning operation additionally and 1 patient underwent Mustard operation additionally. Conclusions The anatomy variation of DORV was very complicated, might be combined with multiple anomalies and could be treated with different operations. The accuracy of early diagnosis could help to find the optimal therapy for different lesions of DORV.