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Clinical Consequences of Cardiomyopathy in Children With Biliary Atresia Requiring Liver Transplantation
Author(s) -
Gorgis Noelle M.,
Kennedy Curtis,
Lam Fong,
Thompson Kathleen,
CossBu Jorge,
Akcan Arikan Ayse,
Nguyen Trung,
Hosek Kathleen,
Miloh Tamir,
Karpen Saul J.,
Penny Daniel J.,
Goss John,
Desai Moreshwar S.
Publication year - 2019
Publication title -
hepatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 5.488
H-Index - 361
eISSN - 1527-3350
pISSN - 0270-9139
DOI - 10.1002/hep.30204
Subject(s) - medicine , biliary atresia , liver transplantation , liver disease , cardiomyopathy , receiver operating characteristic , population , cardiology , cohort , intensive care unit , model for end stage liver disease , transplantation , retrospective cohort study , ventricular assist device , gastroenterology , heart failure , environmental health
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.