Outcomes and complications of combined heart-liver transplantation in patients with failing Fontan physiology: A systematic review

doi:10.5500/wjt.v15.i4.106444

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World Journal of Transplantation

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World J Transplant. Dec 18, 2025; 15(4): 106444
Published online Dec 18, 2025. doi: 10.5500/wjt.v15.i4.106444

Outcomes and complications of combined heart-liver transplantation in patients with failing Fontan physiology: A systematic review

Muhammad Shahzil, Ume Habiba, Mariyah Zainab Irfan, Muhammad Ahmad Qureshi, Muhammad Saad Faisal, Talha Kashif, Ali Akram Qureshi, Hassam Ali, Vinay Jahagirdar, Rakesh Vinayek

Muhammad Shahzil, Department of Internal Medicine, Milton S. Hershey Medical Center, Milton S. Hershey Medical Center, The Pennsylvania State University, Hershey, PA 17033, United States

Ume Habiba, Department of Internal Medicine, Dow University of Health Sciences Department of Medicine, Karachi 74200, Sindh, Pakistan

Mariyah Zainab Irfan, Department of Internal Medicine, Fatima Memorial Hospital College of Medicine and Dentistry, Lahore 54000, Punjab, Pakistan

Muhammad Ahmad Qureshi, Department of Internal Medicine, Mayo Hospital, Lahore 54000, Pakistan

Muhammad Saad Faisal, Department of Internal Medicine, Henry Ford Hospital, Detroit, MI 48202, United States

Talha Kashif, Ali Akram Qureshi, Department of Internal Medicine, King Edward Medical University, Lahore 54000, Punjab, Pakistan

Hassam Ali, Division of Gastroenterology, Hepatology and Nutrition, East Carolina University/Brody School of Medicine, Greenville, North Carolina 27858, United States

Vinay Jahagirdar, Department of Internal Medicine, University of Missouri-Kansas City, Kansas, MI 64110, United States

Rakesh Vinayek, Department of Gastroenterology, Sinai Hospital of Baltimore, Baltimore, MD 21215, United States

Author contributions: Shahzil M, Qureshi MA, Vinayek R contributed to conception and design; Shahzil M, Habiba U, Irfan MZ contributed to data curation and management; Shahzil M, Habiba U, Kashif T, Jahagirdar V contributed to data extraction and analysis; Shahzil M, Qureshi MA contributed to methodology development; Shahzil M, Kashif T software development and statistical analysis; Habiba U, Irfan MZ, Qureshi MA, Kashif T, Qureshi AA, Faisal MS contributed to drafting of the article; Qureshi AA, Ali H, Jahagirdar V, Faisal MS contributed to visualization and data presentation; Vinayek R, Jahagirdar V, Ali H contributed to supervision; Vinayek R, Jahagirdar V, Kashif T contributed to critical revision of the article; Vinayek R, Jahagirdar V, Kashif T critical revision of the article.

Conflict-of-interest statement: The authors declare no conflicts of interest related to the research, authorship, or publication of this article.

PRISMA 2009 Checklist statement: The authors have read the PRISMA 2009 Checklist, and the manuscript was prepared and revised according to the PRISMA 2009 Checklist.

Open Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/

Corresponding author: Hassam Ali, MD, Division of Gastroenterology, Hepatology and Nutrition, East Carolina University/Brody School of Medicine, 2100 statonsburg rd, Greenville, North Carolina 27858, United States. hassamali155@gmail.com

Received: February 28, 2025
Revised: March 30, 2025
Accepted: April 18, 2025
Published online: December 18, 2025
Processing time: 265 Days and 6 Hours

Abstract

BACKGROUND

Single-ventricle congenital heart disease often requires the Fontan procedure, which can lead to Fontan-associated liver disease (FALD) and multi-organ failure. Combined heart-liver transplantation (CHLT) is a potential lifesaving option for these patients.

AIM

To investigate the outcomes and complications of CHLT in patients with failing Fontan physiology.

METHODS

Seven retrospective studies of 121 patients undergoing CHLT were systematically reviewed. Quality was assessed with the Newcastle-Ottawa Scale. A meta-analysis using random-effects models to calculate odds ratios (ORs) or mean differences (MDs) with 95% confidence intervals.

RESULTS

The pooled 30-day, 1-year, 5-year, and 10-year survival rates after CHLT were 92.6%, 86.78%, 81.17%, and 77.8%, respectively. The mean intensive care unit and total hospital lengths of stay were 8.46 and 28.16 days. Mean ischemic time was 267.29 minutes, while cardiopulmonary bypass time was 260.27 minutes. Infections (30%), renal replacement therapy (36.84%), and graft rejection (12.34%) were notable complications. Compared to orthotopic heart transplantation (OHT), CHLT significantly reduced mortality (OR: 0.30, P = 0.009) and ischemic time (MD: –65.93 minutes), with no major differences in perioperative morbidity.

CONCLUSION

CHLT offers a survival advantage over OHT for patients with FALD and failing Fontan physiology. Future prospective studies are warranted to refine eligibility and improve long-term survival.

Keywords: Fontan procedure; Heart defects; Congenital; Heart transplantation; Liver transplantation; Multi-organ failure

Core Tip: This systematic review analyzes outcomes and complications associated with combined heart-liver transplantation (CHLT) in patients with failing Fontan physiology, a condition resulting from single-ventricle congenital heart disease palliated by the Fontan procedure. CHLT demonstrates a survival advantage over isolated heart transplantation, with significantly lower mortality and shorter ischemic times, while maintaining comparable perioperative outcomes. The findings emphasize the importance of multidisciplinary care, careful patient selection, and surgical expertise. Future prospective studies are needed to refine selection criteria and improve long-term survival in this high-risk population.