Restrictive cardiomyopathy in a patient with extrahepatic biliary atresia.
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 at...
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| Format: | Online |
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Korean Academy of Medical Sciences
2001
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| Online Access: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3054757/ |
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pubmed-3054757 |
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pubmed-30547572011-03-15 Restrictive cardiomyopathy in a patient with extrahepatic biliary atresia. Jeong, S. J. Choe, Y. H. Hong, Y. J. Research Article 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. Korean Academy of Medical Sciences 2001-06 /pmc/articles/PMC3054757/ /pubmed/11410702 Text en |
| repository_type |
Open Access Journal |
| institution_category |
Foreign Institution |
| institution |
US National Center for Biotechnology Information |
| building |
NCBI PubMed |
| collection |
Online Access |
| language |
English |
| format |
Online |
| author |
Jeong, S. J. Choe, Y. H. Hong, Y. J. |
| spellingShingle |
Jeong, S. J. Choe, Y. H. Hong, Y. J. Restrictive cardiomyopathy in a patient with extrahepatic biliary atresia. |
| author_facet |
Jeong, S. J. Choe, Y. H. Hong, Y. J. |
| author_sort |
Jeong, S. J. |
| title |
Restrictive cardiomyopathy in a patient with extrahepatic biliary atresia. |
| title_short |
Restrictive cardiomyopathy in a patient with extrahepatic biliary atresia. |
| title_full |
Restrictive cardiomyopathy in a patient with extrahepatic biliary atresia. |
| title_fullStr |
Restrictive cardiomyopathy in a patient with extrahepatic biliary atresia. |
| title_full_unstemmed |
Restrictive cardiomyopathy in a patient with extrahepatic biliary atresia. |
| title_sort |
restrictive cardiomyopathy in a patient with extrahepatic biliary atresia. |
| description |
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. |
| publisher |
Korean Academy of Medical Sciences |
| publishDate |
2001 |
| url |
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3054757/ |
| _version_ |
1611444910876000256 |