- 期刊
三尖瓣Ebstein畸形之三病例報告
Ebstein's Malformation of the Tricuspid Valve - Report of Three Cases
摘要
三尖瓣閉鎖不全或狹窄所致的先天性心臟病不多，其中以Ebstein異常最多，佔所有先天性心臟病約千分之五，其病理上的特徵有：(1)瓣膜組織過長〈redundancy〉。(2)中隔瓣及後瓣〈septal and posterior cuspid〉附黏於右心室壁，形成瓣膜固定點向下位移。因此；產生三尖瓣閉鎖不全及狹窄的情形出現，導致右心房擴大，右心室機能不全的現象。另外也常伴有右束枝阻滯，B型Wolff-Parkinson-White 〈W–P –W〉症候群及其他心律不整。但這些病人在接受心導管攝影檢查時，可能因心律不整而致死亡；加上最近的非侵襲性的M型及雙相超音波心圖均有其特徵。因此超音波心圖已成為快速且正確診斷本症的利器。本文提出三例報告並討論如下。
關鍵字
無資料並列摘要
Ebstein’s anomaly is a rare congenital heart disease. An over all frequency of 0.5% of all congenital heart disease has been reported The basic anatomic abnormality consists of displacement of the septal and posterior leaflets of the tricuspid valve from the atrioventricular annulus resulting in atrialization of a portion of the right ventricle. Sometimes, the anterior tricuspid leaflet is also found abnormal and characterized by large and whiplike leaflet on echocardiogram. Recently we have collected three cases of Ebstein;s anomaly diagnosed by cardias catheterization and/or echocardiography. The patients are an 8-year-old boy, a 14-year-old female neonate. Only the neonate had cyanosis. The two boys had specific triple gallop rhythm at left parasternal border. All the cases had cardiomegaly with cardiothoracic ratio being 55%, 58% and 94% respectively. The lung markings decreased in the chest X-ray films. The two boys had complete right bundle branch block. The neonate had incomplete right bundle block and biventricular hypertrophy as shown on the electrocardiogram. The parasternal long axis view of the 2-D echocardiogram demonstrated the whiplike movement of the anterior tricuspid leaflet during systolic and diastolic movements. The apical four chamber view revealed (1) downward displacement of the septal tricuspid leaflet with displacement index being 39 mm/m2, 31 mm/m2 and 110 mm/m2 respectively (≧8 mm/m2 in Ebstein’s anomaly8), (2) atrialized right ventricle, enlarged right atrium and small functioning right ventricle, (3) tethering and immobilization of the anterior tricuspid leaflet. M-mode echocardiographies of these patients revealed 80 msec, 60 msec and 75 msec delay of tricuspid valve closure on mitral closure respectively. The two boys had undergone catheterization studies which revealed atrialized right ventricle based on intracardiac EKG. The right bentriculogram and right anterior oblique 30° view revealed (1) leftward displacement of the tricuspid valve from atrioventricular annulus to the left side of vertebral spine, (2) aneurismal dilatation of right atrium, (3) right ventricle dividing into atrialized right ventricle. The neonate expired on the 2lst day of birth because of heart failure. The two boys are currently of heart failure. The two boys are currently follow-up at our outpatient clinic.