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Restrictive cardiomyopathy in a patient with extrahepatic biliary atresia
S J Jeong1, Y H Choe, Y J Hong
1Department of Pediatrics, Inha University Hospital, Inha University College of Medicine, Inchon, Korea.
Insights
This study reports the first case of extrahepatic biliary atresia in a child with restrictive cardiomyopathy, a rare cardiovascular complication. Early diagnosis and management are crucial for affected infants.
Area of Science:
- Pediatric Cardiology
- Hepatology
- Congenital Anomalies
Background:
- Extrahepatic biliary atresia commonly presents with digestive and splenic defects.
- Cardiovascular anomalies are less frequent but significant comorbidities.
- Cardiomyopathy is a rare finding in conjunction with biliary atresia.
Observation:
- A 13-month-old boy with diagnosed extrahepatic biliary atresia developed severe respiratory distress.
- Chest radiography revealed cardiomegaly, and echocardiography showed biatrial enlargement.
- Distinct echocardiographic findings indicated restrictive cardiomyopathy.
Findings:
- The patient presented with symptoms of heart failure, including cough and dyspnea.
- Echocardiography demonstrated specific features of restrictive cardiomyopathy, such as increased E-velocity and decreased deceleration time.
- This case represents the first documented instance of restrictive cardiomyopathy associated with extrahepatic biliary atresia.
Implications:
- Highlights the importance of considering cardiac evaluation in infants with biliary atresia.
- Suggests a potential link between biliary atresia and specific types of cardiomyopathy.
- Underscores the need for further research into the co-occurrence of these conditions and their management.
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.