Clinical Consequences of Cardiomyopathy in Children With Biliary Atresia Requiring Liver Transplantation

Noelle M. Gorgis, Curtis Kennedy, Fong Lam, Kathleen Thompson, Jorge Coss-Bu, Ayse Akcan Arikan, Trung Nguyen, Kathleen Hosek, Tamir Miloh, Saul J. Karpen, Daniel J. Penny, John Goss, Moreshwar S. Desai

Research output: Contribution to journalArticlepeer-review

12 Scopus citations


Cirrhotic cardiomyopathy (CCM), a comorbidity of end-stage cirrhotic liver disease, remains uncharacterized in children, largely because of a lack of an established pediatric definition. The aim of this retrospective cohort analysis is to derive objective two-dimensional echocardiographic (2DE) criteria to define CCM associated with biliary atresia (BA), or BA-CCM, and correlate presence of BA-CCM with liver transplant (LT) outcomes in this population. Using receiver operating characteristic (ROC) curve analysis, optimal cut-off values for left ventricular (LV) geometrical parameters that were highly sensitive and specific for the primary outcomes: A composite of serious adverse events (CSAE) and peritransplant death were determined. These results were used to propose a working definition for BA-CCM: (1) LV mass index (LVMI) ≥95 g/m 2.7 or (2) relative wall thickness of LV ≥0.42. Applying these criteria, BA-CCM was found in 34 of 69 (49%) patients with BA listed for LT and was associated with increased multiorgan dysfunction, mechanical and vasopressor support, and longer intensive care unit (ICU) and hospital stays. BA-CCM was present in all 4 waitlist deaths, 7 posttransplant deaths, and 20 patients with a CSAE (P < 0.01). On multivariable regression analysis, BA-CCM remained independently associated with both death and a CSAE (P < 0.01). Utilizing ROC analysis, LVMI was found to be a stronger predictor for adverse outcomes compared with current well-established markers, including Pediatric End-Stage Liver Disease (PELD) score. Conclusion: BA-CCM is highly sensitive and specific for morbidity and mortality in children with BA listed for LT. 2DE screening for BA-CCM may provide pertinent clinical information for prioritization and optimal peritransplant management of these children.

Original languageEnglish (US)
Pages (from-to)1206-1218
Number of pages13
Issue number3
StatePublished - Mar 2019
Externally publishedYes

ASJC Scopus subject areas

  • Hepatology


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