Background: Cirrhotic cardiomyopathy, characterized by low systemic vascular resistance, is increasingly recognized as a complication of end stage liver disease. Yet optimal strategies to screen and stratify patients based on risk are not defined, especially in young children awaiting liver transplant (LTX). Failure to recognize occult cardiac disease may increase morbidity and mortality. We describe and analyze a comprehensive cardiac screening program in a single center cohort of children awaiting LTX.

Methods: All patients (n=28) received a LTX from 8/2012 to 8/2014. Before LTX, patients underwent evaluation by a pediatric cardiologist with expertise in heart failure, all patients received an echocardiogram and 19/28 an EKG.

Results: The primary diagnoses were biliary atresia (n=17), hepatoblastoma (n=5) and other (n=6); age at LTX was 2.4±2.6 yrs (range 0.4 to 9.4); PELD score at allocation was 27±10 (range 2-40). Nine patients were Status 1A or Status 1B (5 with hepatoblastoma). No patient had significant congenital heart disease. Thirteen patients had a prolonged QTc. The mean QTc was 433±22 msec (range 386-470 msec). Only 20% of patients with hepatoblastoma had a prolonged QTC compared to 71% of patients with cirrhotic liver disease. Left atrial volume (LA) was moderately to severely increased in 13 subjects. The mean LA volume was 32.2 ± 12.6 ml/m2 (range 15.0-67.4 ml/m2). No patient with hepatoblastoma had an increased LA volume compared to 62% of patients with cirrhosis. Ejection fraction (EF) was normal in all patients (mean 62.5±4).

Conclusions: Echo assessment of EF inadequately describes cardiovascular (CV) disease in children with cirrhosis awaiting LTX. More subtle findings, including increased LA volume, a marker of increased left atrial pressure/volume, and prolonged QTc, suggest disease-specific markers of CV dysfunction are needed. Furthermore, given EF alone lacks sensitivity in assessing CV disease, it likely underestimates CV risk as well. A strategy to develop a validated CV risk assessment for children awaiting LTX is indicated.

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