Restrictive Cardiomyopathy in a Patient with Extrahepatic Biliary Atresia.
10.3346/jkms.2001.16.3.363
- Author:
Su Jin JEONG
1
;
Yon Ho CHOE
;
Young Jin HONG
Author Information
1. Department of Pediatrics, Inha University Hospital, Inha University College of Medicine, Inchon. cyh@smc.samsung.co.kr
- Publication Type:Case Report
- Keywords:
Bile Ducts, Extrahepatic;
Biliary Atresia;
Cardiomyopathy, Restrictive
- MeSH:
Bile Ducts, Extrahepatic/*abnormalities;
Biliary Atresia/*complications/physiopathology/radiography/ultrasonography;
Cardiomyopathy, Restrictive/*complications/physiopathology/radiography/ultrasonography;
Human;
Infant;
Lung/radiography;
Male;
Radiography, Thoracic
- From:Journal of Korean Medical Science
2001;16(3):363-365
- CountryRepublic of Korea
- Language:English
-
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 E/A ratio (0.33). He was diagnosed as having restrictive cardiomyopathy with characteristic echocardiographic features.
