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Pediatric Marfan Syndrome and Heart Transplantation: Insights From the PHIS Database

Stephanie S Philip1,2, Hugo R Martinez1,2, Matthew F Mikulski3

  • 1Texas Center for Pediatric and Congenital Heart Disease, Dell Medical School at the University of Texas at Austin, Austin, Texas, USA.

Pediatric Transplantation
|November 17, 2025
PubMed

Insights

Pediatric Marfan syndrome (MFS) patients undergoing heart transplants show promising short-term results. This study found no significant MFS-related surgical complications, challenging current practices for these heart failure patients.

Area of Science:

  • Cardiology
  • Pediatric Surgery
  • Genetics

Background:

  • Marfan syndrome (MFS) is a genetic connective tissue disorder.
  • MFS frequently leads to severe cardiovascular complications, including heart failure.
  • Orthotopic heart transplantation (OHT) in pediatric MFS patients is controversial due to potential aortic complications.

Purpose of the Study:

  • To evaluate the outcomes of pediatric Marfan syndrome patients undergoing orthotopic heart transplantation (OHT).
  • To compare the safety and efficacy of OHT in MFS patients versus non-MFS recipients.
  • To assess the impact of MFS on post-transplant complications and survival.

Main Methods:

  • Retrospective review of the Pediatric Health Information System (PHIS) database (2004-2024).
  • Identification of pediatric patients (<18 years) with MFS undergoing OHT.
  • Propensity score matching (3:1) of MFS patients to non-MFS OHT recipients.
  • Analysis of outcomes including graft failure, aortic events, and re-transplant-free survival.

Main Results:

  • Ten pediatric MFS patients were identified among 5493 OHT recipients.
  • Median follow-up was 3.73 years; no in-hospital mortality or re-transplants occurred.
  • Two MFS patients experienced rejection; one developed aortic root dilation without intervention.
  • Propensity-matched analysis revealed no significant differences in rejection, aortic events, or survival between MFS and non-MFS cohorts.

Conclusions:

  • Pediatric Marfan syndrome patients demonstrate excellent short-term outcomes after OHT.
  • No MFS-related surgical complications were observed in this cohort.
  • Findings suggest OHT may be a viable option for select pediatric MFS patients, warranting further long-term investigation.
Abstract

Keywords:
Marfan syndromeheart transplant evaluationpediatric heart failurepediatric heart transplantation

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