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Published on: August 13, 2014
Cardiac structural and functional alterations in infants and children with biliary atresia, listed for liver
Moreshwar S Desai1, Shabier Zainuer, Curtis Kennedy
1Section of Pediatric Critical Care, Department of Pathology, Baylor College of Medicine, Houston, Texas, USA.
Insights
Cirrhotic cardiomyopathy is common in infants with biliary atresia, affecting over 70%. This cardiac condition in infants with liver disease may prolong hospital stays after liver transplants.
Area of Science:
- Pediatric Cardiology
- Hepatology
- Transplant Surgery
Background:
- Cirrhotic cardiomyopathy is known in adults but understudied in infants.
- Biliary atresia (BA) is a common cause of pediatric cirrhosis requiring liver transplantation.
Purpose of the Study:
- To investigate cirrhotic cardiomyopathy features in infants with BA awaiting liver transplant.
- To determine if these cardiac abnormalities correlate with mortality and postoperative complications.
Main Methods:
- Retrospective review of two-dimensional echocardiography (2DE) in 40 infants with BA.
- Comparison with age- and sex-matched healthy controls.
- Assessment of correlation between 2DE findings, intensive care unit (ICU) and hospital length of stay (LOS).
Main Results:
- Infants with BA showed significantly increased left ventricle wall thickness, LV mass index, and LV shortening fraction compared to controls.
- Over 70% of infants with BA exhibited features of cirrhotic cardiomyopathy, including hyperdynamic contractility and altered LV geometry.
- Abnormal 2DE findings correlated with longer ICU and hospital stays post-liver transplantation.
Conclusions:
- Cardiomyopathy is highly prevalent in infants with end-stage liver disease due to BA.
- This underrecognized cardiac condition likely contributes to prolonged hospitalization after liver transplantation.
Background & Aims:
Cirrhotic liver diseases are associated with abnormalities in cardiac geometry and function in adults (cirrhotic cardiomyopathy) but rarely explored in cirrhotic infants or children. We proposed that features of cirrhotic cardiomyopathy are present in infants with cirrhosis due to biliary atresia (BA) as early as the time of evaluation for liver transplant and will correlate with mortality and postoperative morbidity.
Methods:
Two-dimensional echocardiography (2DE) of infants with BA (n=40; median age, 8 months), listed for transplantation at the Texas Children's Hospital from 2004 to 2010, were reviewed and compared with age- and sex-matched infants without cardiac or liver disease (controls). Length of stay and correlation with 2DE results were assessed.
Results:
Compared with controls, children with BA had significant increases in multiple 2DE parameters, notably left ventricle wall thickness (23% increase), left ventricular (LV) mass indexed to body surface area (51% increase), and LV shortening fraction (8% increase). Overall, features of cirrhotic cardiomyopathy were observed in most infants (29/40; 72%); 17 had hyperdynamic contractility, and 24 had altered LV geometry. After liver transplantation (33), infants with abnormal 2DE results had longer stays in the intensive care unit (median, 6 vs 4 days) and the hospital (21 vs 11 days) compared with infants who had normal 2DE reports. On univariate analysis, the length of hospital stay correlated with LV mass index.
Conclusions:
Cardiomyopathy is a prevalent condition in infants with end-stage cirrhotic liver disease due to BA (>70%). This underrecognized condition likely contributes to the prolongation of posttransplant hospitalization.
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