先天性心脏病外科治疗中国专家共识(七):右心室双出口

Chinese expert consensus on surgical treatment of congenital heart disease(7): Double outlet right ventricle

右心室双出口是指一个大动脉全部和另一大动脉开口的大部分(>50%)起源于形态右心室,主动脉瓣与二尖瓣之间可存在或无纤维连续,是一种复杂心脏畸形,具有许多解剖学变异和外科治疗方式。其病变类型可以通过多种方式进行分类,目前比较常用的分型方法是参考室间隔缺损与两大动脉的关系进行分类。许多医疗中心报道了从单心室矫治到双心室修复的各种手术方法,但在诸多方面存在争议,国内外尚无右心室双出口诊疗的指南或者共识,因此我们制定了右心室双出口外科治疗中国专家共识,以期促进我国右心室双出口外科治疗的规范化。

Double outlet right ventricle(DORV)is a complex cardiac malformation with many anatomic variations and various approaches for surgical repair.DORV is mainly defined as the congenital heart disease with ventriculoarterial connection in which both pulmonary artery and aorta arising primarily(>50%)from the right ventricle,associated with continuity or discontinuity between the aorta and mitral valve.DORV can be subclassified by various ways.Now subclassification is usually performed according to the relationship between the ventricular septal defect(VSD)and the great arteries.Various approaches for surgical repair of DORV ranging from single ventricle palliation to biventricular repair are reported from many centers.However,the high-grade guideline of surgical management of DORV is still absent.Hence,we developed the Chinese expert consensus on DORV as the evidence for surgical strategies.