Restrictive Cardiomyopathy in Patient with Extrahepatic Biliary Atresia

Title
Restrictive Cardiomyopathy in Patient with Extrahepatic Biliary Atresia
Authors
Hong, Y.J.
Keywords
bile ducts, extrahepatic, cardiomyopathy
Issue Date
2001-06
Publisher
KOREAN ACAD MEDICAL SCIENCES
Abstract
The most commonly associated anomalies in patients with extrahepatic biliary atresia are cardiovascular, digestive and splenic defects. Of the cardiovascular anomalies, there are very few reports of biliary atresia with cardiomyopathy. We report the first case of a child with extrahepatic biliary atresia and restrictive cardiomyopathy. The patient was a 13-month-old boy diagnosed with extrahepatic biliary atresia at the age of 2 months, when he underwent laparotomy for definite diagnosis. Hepatic portoenterostomy was performed after confirmative cholangiogram. Recently, he developed severe cough and dyspnea, and his respiratory symptoms worsened. Chest radiograph showed cardiomegaly. Two-dimensional echocardiography showed marked biatrial enlargement. On M-mode echocardiogram, a slight increase in left ventricular dimension was seen in early diastole with a relatively good left Ventricular function. Mitral inflow Doppler tracing showed an increased E-velocity (1.1 m/sec) with decreased deceleration time (75 m/sec), and increased YA ratio (0.33). He was diagnosed as having restrictive cardiomyopathy with characteristic echocardiographic features.
URI
http://dspace.inha.ac.kr/handle/10505/20216
ISSN
1011-8934
Appears in Collections:
Medical School/College of Medicine (의학전문대학원/의과대학) > Medical Science (의학) > Journal Papers, Reports(의학 논문, 보고서)
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