Features of Cirrhotic Cardiomyopathy Early in the Lives of Infants With Biliary Atresia Correlate With Outcomes Following Kasai Portoenterostomy

Abstract

Cirrhotic cardiomyopathy (CCM), detected during two‐dimensional echocardiography (2DE), is prevalent in patients with biliary atresia (BA) awaiting transplant. Whether CCM occurs early in the lives of infants with BA is unknown. The aim of this study was to explore the incidence and consequence of CCM in patients with BA, focusing on the earliest ages when 2DE was performed. A cohort of 78 patients with BA at a single center underwent 2DE (median age = 132 days) during the first year of life. Left ventricular mass index (LVMI) to upper limit of normal (ULN) ratio ≥ 1.0 was present in 60% of patients who never underwent Kasai portoenterostomy (KPE; n = 15), 49% with nondraining KPE (n = 41), and 21% with draining KPE (n = 19). Patients with a draining KPE (median age at 2DE = 72 days) had a lower LVMI/ULN ratio (0.75 [interquartile range [IQR] 0.70, 0.91]) compared to those with a nondraining KPE (0.99 [IQR 0.78, 1.17] median age of 141 days; P = 0.012). In those whose 2DE was performed within 7 days of KPE (n = 19, median age of 61 days), the LVMI/ULN ratio was lower in those with a future draining KPE (0.73 [IQR 0.66, 0.75]) compared to the group with a future nondraining KPE (1.03 [IQR 0.88, 1.08], P = 0.002). Logistic regression modeling revealed LVMI/ULN ratio ≥ 1.0 as a predictor of KPE outcome, with an odds ratio of 16.7 (95% confidence interval 1.36‐204; P = 0.028) for a future nondraining KPE compared to those with a LVMI/ULN ratio < 1.0. Conclusion: 2DE early in the lives of patients with BA revealed features of CCM that correlated with future outcomes. If validated in a multicenter study, this could lead to 2DE as a useful clinical tool in the care of infants with BA.

Elevated bile acids are associated with left ventricular structural changes in biliary atresia

“…A recent study shows that abnormalities in LV geometry are detected in early infancy and are associated with poor outcomes after Kasai porto-enterostomy (KPE). 12 Despite advances in the fields of cardiology and hepatology, the only current treatment option to reverse CCM is an LT. 4 The status quo in advancing care in this domain stems from a lack of understanding of the causative factors that lead to the development of CCM in BA. A deeper insight into metabolites that link liver dysfunction with structural and functional aberrations in the heart is needed to understand the pathogenesis of CCM if overall outcomes are to be optimized.…”

Subclinical cardiac abnormalities in children with biliary atresia correlate with outcomes after liver transplantation

“…In our study, we found that elevated LVMI and RWT can predict poor outcomes after liver transplantation. And a recent study suggests that abnormal 2DE features are present early in life in patients with BA and usually occur at the age when BA is diagnosed ( 29 ). We surmise that if we compared these 2DE parameters to the whole pool of patients on a liver transplant waiting list, this extrahepatic feature could optimize organ allocation and direct clinical care in the future.…”

Beyond Varices: Complications of Cirrhotic Portal Hypertension in Pediatrics

“…115 121 A recent study, interestingly, identified CCM early in the course of BA and found the ratio of LVMI to its upper limit of normal was a predictor of post-Kasai outcome. 125 Despite these differences in survival, studies found CCM is associated with higher ICU length of stay as well as multiorgan dysfunction, mechanical ventilation, and the need for pressor support prior to LT. 115 116 121 124 Adult guidelines recommend routine TTE at regular intervals before and after LT ( Table 2 ). 117 Approaches to surveillance for and monitoring of CCM in children are not well defined.…”