title: Cardiac structural and functional alterations in infants and children with biliary atresia, listed for liver transplantation.
abstract: OBJECTIVE
  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.
authors.affiliation: Section of Pediatric Critical Care, Department of Pathology, Baylor College of Medicine, Houston, Texas, USA.
authors.forename: Moreshwar S
authors.initials: MS
authors.lastname: Desai
authors.forename: Shabier
authors.initials: S
authors.lastname: Zainuer
authors.forename: Curtis
authors.initials: C
authors.lastname: Kennedy
authors.forename: Debra
authors.initials: D
authors.lastname: Kearney
authors.forename: John
authors.initials: J
authors.lastname: Goss
authors.forename: Saul J
authors.initials: SJ
authors.lastname: Karpen
mesh_terms: D001656:Biliary Atresia / Q000150:complications / Q000401:mortality / Q000601:surgery*; D009202:Cardiomyopathies / Q000000981:diagnostic imaging / Q000209:etiology* / Q000503:physiopathology; D016022:Case-Control Studies; D002675:Child, Preschool; D005260:Female; D006352:Heart Ventricles / Q000000981:diagnostic imaging / Q000503:physiopathology; D006801:Humans; D007223:Infant; D007362:Intensive Care Units; D007902:Length of Stay; D008103:Liver Cirrhosis / Q000209:etiology / Q000601:surgery*; D016031:Liver Transplantation / Q000009:adverse effects / Q000401:mortality; D008297:Male; D009200:Myocardial Contraction; D018570:Risk Assessment; D012307:Risk Factors; D013781:Texas; D013997:Time Factors; D016896:Treatment Outcome; D014463:Ultrasonography; D018487:Ventricular Dysfunction, Left / Q000000981:diagnostic imaging / Q000209:etiology* / Q000503:physiopathology; D016277:Ventricular Function, Left; D020257:Ventricular Remodeling; D014850:Waiting Lists / Q000401:mortality