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 transplantation and will correlate with mortality and post-operative 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 to age- and sex-matched infants without cardiac or liver disease (controls). Length of stay and correlation with 2DE results were assessed. Results Compared to controls, children with BA had significant increases in multiple 2DE parameters, notably left ventricle (LV) wall thickness (23% increase), 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 under-recognized condition likely contributes to the prolongation of post-transplant hospitalization.

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The definitions for the various ventilator-associated conditions are good predictors of outcomes in children and adults and are amenable to automated surveillance. Based on the study findings, we suggest consideration for shifting the focus of surveillance for ventilator-associated events from only pneumonia to a broader range of complications.