Cardiac structural and functional alterations in infants and children with biliary atresia, listed for liver transplantation

Gastroenterology. 2011 Oct;141(4):1264-72, 1272.e1-4. doi: 10.1053/j.gastro.2011.06.082. Epub 2011 Jul 14.


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.

Publication types

  • Research Support, N.I.H., Extramural

MeSH terms

  • Biliary Atresia / complications
  • Biliary Atresia / mortality
  • Biliary Atresia / surgery*
  • Cardiomyopathies / diagnostic imaging
  • Cardiomyopathies / etiology*
  • Cardiomyopathies / physiopathology
  • Case-Control Studies
  • Child, Preschool
  • Female
  • Heart Ventricles / diagnostic imaging
  • Heart Ventricles / physiopathology
  • Humans
  • Infant
  • Intensive Care Units
  • Length of Stay
  • Liver Cirrhosis / etiology
  • Liver Cirrhosis / surgery*
  • Liver Transplantation* / adverse effects
  • Liver Transplantation* / mortality
  • Male
  • Myocardial Contraction
  • Risk Assessment
  • Risk Factors
  • Texas
  • Time Factors
  • Treatment Outcome
  • Ultrasonography
  • Ventricular Dysfunction, Left / diagnostic imaging
  • Ventricular Dysfunction, Left / etiology*
  • Ventricular Dysfunction, Left / physiopathology
  • Ventricular Function, Left
  • Ventricular Remodeling
  • Waiting Lists* / mortality