Short‐term outcomes of <i>en bloc</i> combined heart and liver transplantation in the failing Fontan

Vaikunth, Sumeet S.; Concepción, Waldo; Daugherty, Tami; Fowler, Michael B.; Lutchman, Glen; Maeda, Keisuke; Rosenthal, David N.; Teuteberg, Jeffrey J.; Woo, Y. Joseph; Lui, George K.

doi:10.1111/ctr.13540

Cited by 55 publications

(30 citation statements)

References 49 publications

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“…Since the first combined heart-liver transplant (CHLT) in 1984, its indications, patient demographics, and outcomes have evolved significantly [1,2]. Once a rare and herculean endeavor, CHLT is now being practiced with increasing regularity and improved outcomes [2][3][4][5][6][7].…”

Section: Introductionmentioning

confidence: 99%

Adult Combined Heart-Liver Transplantation: The United States Experience

Alexopoulos

Ziogas

et al. 2022

Transpl Int

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Background: We aimed to review the indications and outcomes of adults undergoing combined heart-liver transplantation (CHLT) in the US using national registry data.Methods: Adult (≥18 years) CHLT recipients in the United Network for Organ Sharing database were included (09/1987–09/2020; era 1 = 1989–2000, era 2 = 2001–2010, era 3 = 2011–2020). Survival analysis was conducted by means of Kaplan-Meier method, log-rank test, and Cox regression.Results: We identified 369 adults receiving CHLT between 12/1989–08/2020. The number of adult CHLT recipients (R2 = 0.75, p < 0.001) and centers performing CHLT (R2 = 0.80, p < 0.001) have increased over the study period. The most common cardiac diagnosis in the first two eras was restrictive/infiltrative cardiomyopathy, while the most common in era 3 was congenital heart disease (p = 0.03). The 1-, 3-, and 5-years patient survival was 86.8, 80.1, and 77.9%, respectively. In multivariable analysis, recipient diabetes [adjusted hazard ratio (aHR) = 2.35, 95% CI: 1.23–4.48], CHLT between 1989-2000 compared with 2011–2020 (aHR = 5.00, 95% CI: 1.13–22.26), and sequential-liver first CHLT compared with sequential-heart first CHLT (aHR = 2.44, 95% CI: 1.15–5.18) were associated with increased risk of mortality. Higher left ventricular ejection fraction was associated with decreased risk of mortality (aHR = 0.96, 95% CI: 0.92–0.99).Conclusion: CHLT is being increasingly performed with evolving indications. Excellent outcomes can be achieved with multidisciplinary patient and donor selection and surgical planning.

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Section: Introductionmentioning

confidence: 99%

Adult Combined Heart-Liver Transplantation: The United States Experience

Alexopoulos

Ziogas

et al. 2022

Transpl Int

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“…FALD presents a significant challenge in the chronic care of Fontan patients and at time of HT evaluation with many unanswered questions, including when to refer for combined heart and liver versus heart‐only transplantation. While successful combined heart and liver transplantation has been reported in Fontan patients with comparable survival post‐transplant, donor organs remain a limited precious resource 6,7,12‐14 . Recognized as an inevitable consequence of long‐term Fontan physiology, FALD is progressive over time and appears to correlate with time from Fontan 4,15 …”

Section: Discussionmentioning

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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“…Published outcomes in ACHD heart-liver transplantation are quite good, with reported 1-, 5-, and 10-year survivals of 86%, 83%, and 83% [58]. Small series of heart-liver in ACHD patients with 100% 1-year survival have been reported by Stanford (9 patients), University of Pennsylvania (7 patients), University of California at Los Angeles (5 patients), and Cleveland Clinic Foundation (2 patients) [55,[64][65][66]. These favorable short-term outcomes likely reflect the highly specialized nature of the procedure, with small numbers of carefully selected patients undergoing heart-liver transplantation at a small number of centers.…”

Section: Multiorgan Transplantationmentioning

confidence: 99%

Transplant and mechanical circulatory support in patients with adult congenital heart disease

Monaco¹,

Khanna²,

Khazanie³

2020

Heart Fail Rev

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Advances in surgery and pediatric care over the past decades have achieved improved survival for children born with congenital heart disease (CHD) and have produced a large, growing population of patients with adult congenital heart disease (ACHD). Heart failure has emerged as the leading cause of death and a major cause of morbidity among the ACHD population, while as little evidence supports the efficacy of guideline-directed medical therapies in this population. It is increasingly important that clinicians caring for these patients understand how to utilize mechanical circulatory support (MCS) in ACHD. In this review, we summarize the data on transplantation and MCS in the ACHD-heart failure population and provide a framework for how ACHD patients may benefit from advanced heart failure therapies like transplantation and MCS.

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Short‐term outcomes of en bloc combined heart and liver transplantation in the failing Fontan

Cited by 55 publications

References 49 publications

Adult Combined Heart-Liver Transplantation: The United States Experience

Adult Combined Heart-Liver Transplantation: The United States Experience

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

Transplant and mechanical circulatory support in patients with adult congenital heart disease

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