(378) Assessment of Left Ventricular Systolic and Diastolic Function in Children With Biliary Atresia

Introduction: Cirrhotic cardiomyopathy (CCM) is a recognized entity in adults and cardiac decompensation is a leading cause of mortality among adults after TIPS procedure. It is characterized as a syndrome of high output cardiac failure with progressive diastolic dysfunction. Despite evidence suggesting that the incidence of cirrhotic liver disease in children is rising and a cause for decompensation and death, literature on cirrhotic cardiomyopathy in children is sparse. Characterization of CCM and further establishing a link of CCM to poor outcomes could provide valuable insight into its onset and progression.

Methods: The study was approved by the Baylor College of Medicine Institutional Review Board. Retrospective review of biliary atresia (BA) patients listed for transplant without history of cardiac disease were included. Evaluation of LV strain parameters from echocardiograms obtained proximate to the time of transplantation . Left ventricular global longitudinal strain (GLS) was measured as a less load-dependent indicator of systolic cardisc function and LV diastolic strain (DS) assessed diastolic function. Results are reported as median (IQR).

Results: A total of 98 patients with 112 exams within 18 months (0.25 (0.12 to 0.53)) and demonstrated GLS of -0.20 (-0.23 to -0.17) and DS of 1.07 (-0.02 to 1.82). Of these studies 34.8% demonstrated abnormal GLS. Among studies in patients who died (9) a significant association between mortality and GLS (p = 0.08) or DS (p = 0.63) was not detected using Wilcoxon Rank-Sum test. Comparing GLS in categories of normal, borderline, and decreased demonstrated p = 0.07 using Fisher’s Exact test.

Conclusions: In this cohort of pediatric patients, an association between mortality and CCM as characterized by GLS and DS was not identified. This may reflect sample size limitations particularly with regard to the number of patients who died, as well as better survival overall for pediatric patients with BA. This study demonstrated the assessment of strain in this population and CCM was not associated with mortality in this observed cohort. Further investigation are warranted into reasons for potential differences in observed mortality between this pediatric cohort and adult populations.