Hepatorenal dysfunction assessment with the Model for End-Stage Liver Disease Excluding INR score predicts worse survival after heart transplant in pediatric Fontan patients

Simpson, Kathleen E.; Thrush, Phil; Shih, Renata; Simmonds, Jacob; Knecht, Ken; Mogul, Douglas; Hurley, Kathleen; Koehl, Devin; Cantor, Ryan S.; Naftel, David C.; Kirklin, James K.; Daly, Kevin P.

doi:10.1016/j.jtcvs.2021.02.014

Cited by 27 publications

(15 citation statements)

References 32 publications

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“…This study confirms again the importance of end organ function at the time of heart transplantation in post-transplant outcomes. This is similar to other diseases where hepatic and renal dysfunction is associated with worse outcomes [2].…”

Section: Hepatorenal Dysfunction Assessment With the Model For End-stage Liver Disease Excluding Inr Score Predicts Worse Survival After supporting

confidence: 84%

From Other Journals: A Review of Recent Articles by Our Editorial Team

Alsaied

Ashfaq

2021

Pediatr Cardiol

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In this review, we provide a brief description of recently published articles addressing topics relevant to pediatric cardiologists. Our hope is to provide a summary of the latest articles published recently in other journals in our field. The articles address (1) outcomes for COVID-19 infection in adults with congenital heart disease which showed no increased mortality compared to the general population, (2) hepatorenal dysfunction before transplantation in patients with Fontan is associated with increased mortality, (3) abnormal Von Willebrand factor metabolism and angiopoietin signaling may contribute to pulmonary AVM formation in children with a Glenn circulation, (4) low cardiac output after the Norwood procedure which improves with higher hemoglobin and with milrionone, (5) a comparison of staged versus complete repair in neonatal tetralogy of Fallot reveiling the pros and cons of each strategy, (6) the long-term outcomes of early repair of complete atrioventricular canal show no difference in outcomes in patients who were repaired below 3 months of life.

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Section: Hepatorenal Dysfunction Assessment With the Model For End-stage Liver Disease Excluding Inr Score Predicts Worse Survival After supporting

confidence: 84%

From Other Journals: A Review of Recent Articles by Our Editorial Team

Alsaied

Ashfaq

2021

Pediatr Cardiol

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“…All patients in our institution undergo standard testing for hepatitis, Epstein‐Barr Virus, cytomegalovirus, and human immunodeficiency virus during pre‐HT evaluation. A modified model for end‐stage liver disease excluding INR (MELD‐XI) previously described by Amdani et al for use in pediatric Fontan HT patients was used to calculate MELD‐XI, with a MELD‐XI of ≥11.5 considered high 10 . Survival at the last follow‐up post‐HT was evaluated and compared between Fontan patients with and without available serial post‐HT liver imaging during the same time period.…”

Section: Methodsmentioning

confidence: 99%

Heterogeneous outcomes of liver disease after heart transplantation for a failed Fontan procedure

Ybarra

Khanna

Turmelle

et al. 2021

Pediatric Transplantation

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Background Fontan‐associated liver disease (FALD) uniformly affects patients with long‐term Fontan physiology. The effect of isolated heart transplant (HT) on the course of FALD post‐HT is not well understood. Methods We evaluated serial liver imaging pre‐ and post‐HT to assess liver changes over time in a single‐center retrospective analysis of Fontan HT recipients who had pre‐ and ≥1‐year post‐HT liver imaging. Available patient demographic and clinical data were reviewed, including available liver biopsy results. Results Serial liver imaging was available in 19 patients with a median age at HT of 12 years (range 3–23), the median age from Fontan to HT of 5.7 years (range 0.8–16), and the median time from imaging to follow up of 27 months (range 12–136 months). Pre‐HT liver imaging was classified as follows: normal (n=1), congested (n=9), fibrotic (n=7), and cirrhotic (n=2). The majority of transplanted patients (15/19) had improvement in their post‐HT liver imaging, including 13 patients with initially abnormal imaging pre‐HT having normal liver imaging at follow‐up. One patient had persistent cirrhosis at 26‐month follow‐up, one patient had unchanged fibrosis at 18‐month follow‐up, and one patient progressed from fibrosis pre‐HT to cirrhosis post‐HT at 136 months. No patients had overt isolated liver failure during pre‐ or post‐HT follow‐up. Liver biopsy did not consistently correlate with imaging findings. Conclusions Post‐HT liver imaging evaluation in Fontan patients reveals heterogeneous liver outcomes. These results not only provide evidence for the improvement of FALD post‐HT but also show the need for serial liver imaging follow‐up post‐HT.

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“…[135] Assessment of liver function is an integral component of the cardiac transplant evaluation for patients with Fontan physiology given prior studies which reveal correlation between higher MELD-XI scores and inferior survival after heart transplantation. [87,136] IHT Heart transplantation may be considered in patients with Fontan physiology who have developed FF, intractable arrhythmia with decompensation, protein-losing enteropathy (PLE), thromboembolic events, and progressive cyanosis. PHTN and HCC are considered indications for CHLT and preclude IHT alone.…”

Section: Tr Anspl Antation In Patients W Ith Faldmentioning

confidence: 99%

“…[ 7,86 ] A recent retrospective study analyzed 596 patients with Fontan physiology who underwent IHT through a review of the Pediatric Heart Transplant database. [ 87 ] Multivariate analysis revealed increased posttransplant mortality for every unit increase in the MELD‐XI score. The authors of this study then divided this patient population into two cohorts: those with a high MELD‐XI score (defined as a MELD‐XI score of 11.5 or higher) and those with a low MELD‐XI score (MELD‐XI score of 11.5 or lower).…”

Section: Diagnosis Of Faldmentioning

confidence: 99%

“…Patients in the high MELD‐XI cohort had inferior postheart transplant survival at 1 (84.4% vs. 88.7%) and 5 years (71.7% vs. 80.9%) ( p = 0.02). [ 87 ] Although specific cutoffs to screen for FALD or stratify risk of transplantation remain to be defined in this patient population, these studies suggest that a high MELD‐XI score may suggest suboptimal functioning of the Fontan circulation and increased risk of liver disease and poorer outcomes after IHT.…”

Section: Diagnosis Of Faldmentioning

confidence: 99%

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