移行型心内膜垫缺损(附11例手术病例报告)

TRANSITIONAL ATRIO-VENTRICULARSEPTAL DEFFCT(T-AVSD)(REPORT OF 11 SURGICAL CASES)

报告移行型心内膜垫缺损１１例。男性８例。女性３例，平均年龄１２．１岁，平均体重２７．３ｋｇ。术前多普勒彩色超声心动图正确诊断只有３例（２７％）。解剖发现有左右房室瓣瓣环未完全分隔，左右房室瓣叶基本形成；二尖瓣大瓣均有明确瓣裂，同时并有严重的三尖瓣发育不全，有明显的二尖瓣和三尖瓣关闭不全；由于二尖瓣大瓣与三尖瓣隔瓣均附着在室间隔上缴同一水平面，而产生类似完全型心内膜垫畸形的血流动力学改变；手术重点针对原发孔房缺和左房室瓣关闭不全进行修复，对三尖瓣的改变可不作处理。围术期死亡２例，后期合并症死亡１例，均为术后心衰。

his paper reviews 11 surgical cases which were recognized as transitional atrio-ventric- ular septal defect (T-AVSD) . Eight were male and 3 female , with average age of 12. 1 years and average body weight of 27. 3 kg. Pre-operative colour dopplar echocardiography diagno- sis of T-AVSD was only in 3 cases - (27% ). Anatomic features in them were : discrete left and right annili , independently formed mitral and tricuspid valves ; with sharp cleft in anteri- or leaflet of mitral valve and malformation of tricuspid valve. Two septal leaflets attached to the summit of ventricular septal on the same level and regurgitations were very nearby, which made the same hemodynamic change as the complete type. The surgical principle for them was to correct primary atrial defect and left atrio-ventricular valve without special pro- cedure for the tricuspid valve. Three died in the post-operation (27%) due to heart failure.