대동맥판막 및 승모판막 폐쇄증을 동반한 심내막 섬유탄성증
Endocardial Fibroelastosis with Aortic and Mitral Valve Atresia
이정용, 박현철,
소속 상세정보
이정용 ( )
조선대학교 의과대학 정형외과학교실
박현철 ( )
가톨릭대학 의학부 병리학교실
KMID : 0357919800140040079
Abstract
Endocardial fibroelastosis is a disease of obscure etiology and pathogenesis but its
gross and microscopic findings have been well known.
We have experienced 8-month-old male infant who is diagnosed clinically transposition
of great vessel with atrial septal defect.
Postmortem examination was performed 96 hours after death. The heart weighed
130gm(normal 35 gm), and was globular in shape. Left ventricle was completely
occuluded by aortric and mitral atresia. Left ventricular endocardial surface was pearly
white in color and measured up to 2 mm in thickness.
Microscopically, left ventricle showed fibroelastic thickening of endocardium.
The left atrium was opened to right atrium through patent formen ovale. Aortic arch is
very hypoplastic but its main branches and coronary artery were branched out in
normal position, and connected with pulmonary artery by widened ductus arteriosus. The
other chambers and valvular morphology of heart were free from remarkable findings.
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