CAJ | 학술논문

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。
삼첨판하이기형우칭Ebstein기형，시일충교위한견적선천성심장병，주요병리개변위：삼첨판（주요격판화후판）불부착재판배상，이시라선형하이지우심실，동시판막급판하결구이상。유우우실발육불전급공능장애화삼첨판관폐불전도치적우심용량부하증가，표현위로루후기급핍력、심계、발감화심력쇠갈。본문취삼첨판하이기형합병난치성우심쇠갈급하지궤양1례진행보고。
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.