Lacour-Gayet F
The Children's Hospital Heart Institute, Denver, CO 80218, USA.
Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2008:39-43. doi: 10.1053/j.pcsu.2007.12.003.
Intracardiac repair of double outlet right ventricle (DORV) remains controversial. DORV is a particular mode of ventriculo-arterial connection and not a specific congenital heart disease. It can exhibit a wide spectrum of anatomic and physiologic variations. This heterogeneity has naturally led to controversies over the anatomical definition, classification schemes, and the techniques for surgical repair. From a surgical standpoint, the functional classification that was adopted together by the Society of Thoracic Surgeons (STS), The European Association of Cardio-Thoracic Surgery (EACTS) and the Association of the European Pediatric Cardiologists (AEPC), provides useful information to understand the anatomical variations and the choice of the surgical technique. The lesions that remain a surgical challenge are those where "200% of the great vessels" arise from the right ventricle. Namely: DORV-nc-VSD and DORV-AVSD-heterotaxy. Both of these lesions have traditionally been indications for single-ventricle palliation. In our series, there was one death in 15 of these challenging patients (6.7% mortality) following bi-ventricular repair. Two patients required a one and a half repair. Long-term results of biventricular repair of complex DORV are not yet available. These data would be crucial to validate the intracardiac repair technique."
右心室双出口（DORV）的心内修复仍存在争议。DORV是心室-动脉连接的一种特殊模式，而非特定的先天性心脏病。它可表现出广泛的解剖和生理变异。这种异质性自然导致了在解剖学定义、分类方案及手术修复技术方面的争议。从外科角度来看，由美国胸外科医师协会（STS）、欧洲心胸外科学会（EACTS）和欧洲儿科心脏病学家协会（AEPC）共同采用的功能分类，为理解解剖变异和手术技术的选择提供了有用信息。仍具手术挑战的病变是那些“200%的大血管”起源于右心室的情况。即：DORV-无室间隔缺损（nc-VSD）和DORV-房室间隔缺损（AVSD）-内脏异位。传统上，这两种病变均为单心室姑息治疗的指征。在我们的系列研究中，15例此类具有挑战性的患者在双心室修复后有1例死亡（死亡率6.7%）。2例患者需要进行一期半修复。复杂DORV双心室修复的长期结果尚无报道。这些数据对于验证心内修复技术至关重要。
