Clinical Consequences of Cardiomyopathy in Children With Biliary Atresia Requiring Liver Transplantation

Gorgis, Noelle; Kennedy, Curtis; Lam, Fong; Thompson, Kathleen; Coss‐Bu, Jorge A.; Akcan-Arikan, Ayse; Nguyen, Trung; Hosek, K; Miloh, Tamir; Karpen, Saul J.; Penny, Daniel J.; Goss, John A.; Desai, Moreshwar S.

doi:10.1002/hep.30204

Cited by 30 publications

(46 citation statements)

References 40 publications

(108 reference statements)

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“…They concluded that CCM‐associated cardiac changes in pediatric patients with cirrhotic liver disease are frequent, mild, associated with cholestasis, and reversible after LT but may impact peritransplant care and posttransplant hospitalization time. This finding is in contrast to the study by Gorgis et al who documented an increased morbidity and mortality in children with CCM …”

Section: Chronic Liver Disease Leading To Cardiac Diseasecontrasting

confidence: 99%

Section: Chd and The Livermentioning

confidence: 99%

“…CCM is defined as impaired cardiac function or electrophysiology in patients with end‐stage liver disease without known primary cardiac disease (Table ). It includes the combination of reduced cardiac contractility with systolic and diastolic dysfunction and conduction defects and has been extensively reported in adults with cirrhosis who have a higher mortality after LT and more recently in some pediatric studies …”

Section: Chronic Liver Disease Leading To Cardiac Diseasementioning

confidence: 99%

“…In addition to congenital cardiac disease, recent studies have suggested that up to 70% of patients with BA awaiting LT may develop cirrhotic cardiomyopathy (CCM) with an increased left ventricular mass index (LVMI), hyperdynamic contractility, and thickened left ventricular free wall and septum. (4,39) The severity of LVMI was predictive of pretransplant, peritransplant, and posttransplant outcomes, with a high incidence of morbidity and mortality. Other authors have demonstrated a prolonged hospital stay but no increase in mortality in children with CCM, and all made a complete recovery after successful LT. (3,40) So, further evaluation of the significance of CCM in children is required.…”

Section: Alagille Syndromementioning

confidence: 99%

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Cardiac and Liver Disease in Children: Implications for Management Before and After Liver Transplantation

et al. 2019

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There is close interaction between the functions of the liver and heart affecting the presentation, diagnosis, and outcome of acute and chronic cardiac and liver disease. Conditions affecting both organ systems should be considered when proposing transplantation because the interaction between cardiac disease and liver disease has implications for diagnosis, management, selection for transplantation, and, ultimately, for longterm outcomes after liver transplantation (LT). The combination of cardiac and liver disease is well recognized in adults but is less appreciated in pediatric patients. The focus of this review is to describe conditions affecting both the liver and heart and how they affect selection and management of LT in the pediatric population.

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Section: Chronic Liver Disease Leading To Cardiac Diseasecontrasting

confidence: 99%

Section: Chd and The Livermentioning

confidence: 99%

Section: Chronic Liver Disease Leading To Cardiac Diseasementioning

confidence: 99%

Section: Alagille Syndromementioning

confidence: 99%

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Cardiac and Liver Disease in Children: Implications for Management Before and After Liver Transplantation

et al. 2019

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“…In the absence of improved organ availability through increased deceased and live donation, new objective parameters for the PELD scoring system for infants are urgently needed. In their paper “Clinical consequences of cardiomyopathy in children with biliary atresia requiring liver transplantation,” Gorgis et al present a likely candidate for risk stratification . Cirrhotic cardiomyopathy (CCM) is characterized in adults by cardiac dysfunction (systolic and/or diastolic), left ventricular hypertrophy, and/or prolonged QT interval corrected for the heart rate .…”

mentioning

confidence: 99%

Cirrhotic Cardiomyopathy in Children With Biliary Atresia: A New Objective Parameter to Predict Morbidity and Mortality on the Wait List—and Beyond!

Rand

2019

Hepatology

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

et al. 2022

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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.

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Clinical Consequences of Cardiomyopathy in Children With Biliary Atresia Requiring Liver Transplantation

Cited by 30 publications

References 40 publications

Cardiac and Liver Disease in Children: Implications for Management Before and After Liver Transplantation

Cardiac and Liver Disease in Children: Implications for Management Before and After Liver Transplantation

Cirrhotic Cardiomyopathy in Children With Biliary Atresia: A New Objective Parameter to Predict Morbidity and Mortality on the Wait List—and Beyond!

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

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