Cardiac Structural and Functional Alterations in Infants and Children With Biliary Atresia, Listed for Liver Transplantation

Abstract

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.

Bile acids induce arrhythmias: old metabolite, new tricks

“…These human experiments, which were faithfully reproduced in isolated adult mouse cardiomyocytes, further strengthen the long-held notion that high levels of circulating bile acids adversely affect myocardial cell biology. More importantly, these studies add clinical relevance to the animal studies which speculated a role for bile acids in the pathophysiology of cardiac dysfunction in cirrhosis,13 14 termed cirrhotic cardiomyopathy,15 16 which has a prevalence of ∼50% according to some studies 17 18. Electrocardiographic abnormalities, more specifically prolonged QT interval is a key derangement of cirrhotic cardiomyopathy and is known to cause sudden death in adults 19 20 and increases mortality risk in children21 with liver failure.…”

Current Issues in Pediatric Liver Transplantation

“…A study in children with biliary atresia listed for liver transplant concluded that cardiomyopathy is a prevalent condition in infants with end-stage cirrhotic liver disease due to biliary atresia (> 70%) compared with age-matched controls without liver disease. 11 Furthermore, children with preexisting abnormal echocardiography had longer intensive care unit and hospital stays after transplant. A mouse model of CC demonstrated rapid development of cardiac hypertrophy and altered function, including impaired inotropic response within 3 weeks of inducing biliary fibrosis.…”

Analysis of 83 consecutive liver transplants performed at a tertiary care reference hospital in the interior of the state of Sao Paulo

“…It should be pointed out that the systemic pressure levels of the two groups were similar to normal values, also considering that all patients were chronically taking a beta-blocker in order to reduce portal pressure while waiting on the transplant line. These patients are known to have peripheral systemic vasodilation and increased cardiac output in a compensatory manner, a fact that seems to have been effective in these patients 6,17,18 .…”