https://www.ncbi.nlm.nih.gov/pubmed/30076624 Biliary atresia

Hepatology. 2018 Aug 3. doi: 10.1002/hep.30204. [Epub ahead of print]
Clinical Consequences of Cardiomyopathy in Children with Biliary Atresia Requiring LiverTransplantation.
Gorgis NM1, Kennedy C1, Lam F1, Thompson K1, Coss-Bu J1, Arikan AA1,2, Nguyen T1, Hosek K3, Miloh T3, Karpen SJ4, Penny DJ5, Goss J6, Desai MS1.
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Abstract

Cirrhotic Cardiomyopathy (CCM), a comorbidity of end-stage cirrhotic liver disease, remains uncharacterized in children, largely due to a lack of an established pediatric definition. The aim of this retrospective cohort analysis is to derive objective 2-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 cutoff values for left ventricular (LV) geometric parameters that were highly sensitive and specific for the primary outcomes: a composite of serious adverse events (CSAE) and peri-transplant death were determined. These results were used to propose a new working definition for BA-CCM: 1) left ventricular mass index (LVMI) ≥ 95 g/m2.7 ; or 2) relative wall thickness of LV ≥ 0.42. Applying this criteria, BA-CCM was found in 34 of 69 (49%) patients with BA listed for LT and was associated with increased multi-organ dysfunction, mechanical and vasopressor support and longer ICU and hospital stays. BA-CCM was present in all 4 waitlist deaths, 7 post-transplant 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 to current well-established markers, including PELD.

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 peri-transplant management of these children. This article is protected by copyright. All rights reserved.