심의 Ebstein씨 기형(삼첞판의 선천성 하방편위)
Ebstein's Anomaly: Congenital Downward Displacement of Tricuspid Valve.

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이영린; 최연수; 손이석; 장해원; 정준; 이동식; 홍창애; 이영균; 김종술; 이성수; 백성연; 김용일
Issue Date
서울대학교 의과대학
Seoul J Med 1961;2(1):99-105
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
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
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
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.
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College of Medicine/School of Medicine (의과대학/대학원)Dept. of Medicine (의학과)The Seoul Journal of MedicineThe Seoul Journal of Medicine Vol. 02 No.1 (1961)
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