Left ventricular global longitudinal strain is predictive of outcomes in children with biliary atresia

Background Cirrhotic cardiomyopathy (CCM) in adults is now defined to include left ventricular (LV) global longitudinal strain (GLS) as a marker of subclinical systolic dysfunction.1 In children with biliary atresia (BA), cardiac involvement has been linked to adverse peri-transplant outcomes,2 but data on the significance of LVGLS in paediatric cirrhosis are sparse. Purpose This study aimed to assess the prevalence of abnormal LV GLS in children with BA listed for liver transplantation (LT) and its association with clinical outcomes. Methods We performed a retrospective study and included consecutive paediatric patients with BA listed for LT (2013–2021) at a quaternary paediatric institution. Patients with a pre-LT echocardiogram and adequate apical views for GLS measurement were included. GLS >-18% (between 0% and -18%) were considered abnormal.1,3 Primary outcomes included waitlist decompensation and overall mortality (death from listing to one year post-LT). Secondary outcome measures were ventilator-free days (VFD), post-operative ICU length of stay (LOS) and hospital LOS. Results Among 125 patients with BA listed for LT, 83 met the inclusion criteria (median age: 10 months [6, 17], 62% female). The median age at LT was 13 months [9, 24], including 42% (35/83) with waitlist decompensation and 14% (12/83) overall mortality (1 pre-LT and 11 post-LT). The median pre-LT LV GLS was -19.5% [-17, -23] and 31% (26/83) had abnormal LV GLS (>-18%). Patients with abnormal GLS had higher rates of waitlist decompensation (61% [16/26] vs. 33% [19/57]; OR = 3.2, 95% CI: 1.2, 8.3; p=0.019) and mortality (31% [8/26] vs. 7% [4/57]; OR = 5.9, 95% CI: 11.6, 21.9; p=0.007). VFD, ICU LOS, and hospital LOS did not differ significantly different between groups (Table 1). Abnormal GLS remained independently associated with mortality after adjusting for age, PELD score, and LV mass index (OR 23; 95% CI: 1.3-416; p = 0.03). Conclusions Abnormal LV GLS >-18% was present in 31% of children with BA listed for LT and was associated with waitlist decompensation and independently predictive of mortality. These findings highlight the potential role of GLS in evaluating CCM in paediatric cirrhosis. Further studies are warranted to refine CCM definition and improve risk stratification in paediatric cirrhosis.