三尖瓣下移畸形合并难治性右心衰竭及下肢溃疡1例

Ebstein's anomaly with refractory right-sided heart failure and leg ulcers: a case report

三尖瓣下移畸形又称Ebstein畸形,是一种较为罕见的先天性心脏病,主要病理改变为:三尖瓣(主要隔瓣和后瓣)不附着在瓣环上,而是螺旋形下移至右心室,同时瓣膜及瓣下结构异常。由于右室发育不全及功能障碍和三尖瓣关闭不全导致的右心容量负荷增加,表现为劳累后气急乏力、心悸、发绀和心力衰竭。本文就三尖瓣下移畸形合并难治性右心衰竭及下肢溃疡1例进行报告。

Ebstein malformation is a congenital heart disease characterized pathologically by displacement of the septal leaflet of the tricuspid valve towards the apex of the right ventricle of the heart. Hypoplasia, dysfunction of the right ventricle and tricuspid regurgitation cause an increased volume load of the right heart and result in the clinical manifestations of chest tightness, shortness of breath and fatigue after activities, palpitation, cyanosis and heart failure. We report a case of Ebstein＇s anomaly with refractory right heart failure and leg ulcers.