심의 Ebstein씨 기형(삼첞판의 선천성 하방편위)

Abstract

A twenty-two years old cyanotic and dyspneic Korean male with clubbed fingers was diagnosed as an Ebstein's congenital cardiac anomaly clinically. For surgical palliation of these symptoms inflow of superior vena caval blood was shunted to the right pulmonary artery by Glenn's operation. Thirty hours post-operatively the patient expired with increasing cyanosis and dyspnea. Pre-operative diagnosis was confirmed by autopsy findings. On autopsy, the heart revealed a large ovoid-globular baggy shape, weighing 350grns. The dilated right atrium and ventricle were thin-walled and bulged prominently above the left atrium and ventricle. Multiple congenital anomalies of the heart and great cardiac vessels were observed, and included the followings: 1. Ebstein's malformation of the tricuspid valve, (downward displacement of the tricuspid valve in the right ventricle); The anterior leaflet of the tricuspid valve appeared to be near-normally formed and positioned to the annulus fibrosus. However. chordae tendineae of this valve leaflet extended to the septal part of the right ventricular wall without papillary muscles. Mal-shaped septal and posterior leaflets were not clearly distinguishable from each other, and posi: ioned apart from annulus fibrosus far downwards to the atrialized portion of the thin and dilated right ventricle, with rudimentary chordae and absent papillary muscles. 2. Absence of muscular trabeculation in the atrialized portion of the thin-walled right ventricle. 3. Persistence of foramen ovale. 4. Hypoplasia of the pulmonary artery and aorta. 5. Abnormally deep and wide sulcus coronarius. 6. Localized fibro-elastic changes of endocardium of the right ventricle. Aside from these multiple congenital anomalies of the heart and great vessels, dilatation of venae cavae, mild exudative pericarditis, marked generalized passive congestion of viscera, and clubbed fingers and toes were observed.