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Improving outcomes for transplantation in failing Fontan-what is the next target?

Barbara Cardoso1, Andras Kelecsenyi2, Jonathan Smith2,3

  • 1Adult Congenital and Paediatric Heart Unit, Freeman Hospital, Newcastle upon Tyne NHS Foundation Trust, Newcastle upon Tyne, United Kingdom.

JTCVS Open
|August 25, 2022
PubMed

Insights

Major intraoperative bleeding significantly increases mortality risk in adult congenital heart disease patients undergoing orthotopic heart transplantation for Fontan failure. Preventing and managing bleeding is crucial for improving survival in this complex patient group.

Area of Science:

  • Cardiology
  • Transplantation Medicine
  • Congenital Heart Disease

Background:

  • Late Fontan failure presents a significant challenge in adult congenital heart disease management.
  • Orthotopic heart transplantation (OHT) is a viable option for select patients with end-stage single ventricle physiology.
  • Identifying mortality predictors post-OHT in this population is critical for improving outcomes.

Purpose of the Study:

  • To determine the primary factors contributing to postoperative mortality in patients undergoing OHT for late Fontan circulation failure.
  • To analyze the impact of intraoperative bleeding and primary graft dysfunction on survival after OHT in this specific cohort.

Main Methods:

  • Retrospective review of 31 adult patients with congenital heart disease and single ventricle physiology who underwent OHT for Fontan failure between 2007 and 2019.
  • Exclusion of patients receiving combined heart-liver transplantation.
  • Statistical analysis to identify associations between perioperative factors and mortality.

Main Results:

  • Overall 30-day, 1-year, and 5-year survival rates after OHT were 81%, 71%, and 67%, respectively.
  • Major intraoperative bleeding was strongly associated with increased postoperative mortality (OR 30, P=.002).
  • Preoperative ventricular function and primary graft dysfunction did not significantly impact mortality but contributed to morbidity.

Conclusions:

  • Major intraoperative bleeding is the principal risk factor for mortality following OHT in patients with late Fontan failure.
  • Developing advanced strategies for bleeding prevention and management is essential to enhance survival rates in this high-risk group.
Abstract

Keywords:
CHD, congenital heart diseaseCPB, cardiopulmonary bypassECMO, extracorporeal membrane oxygenationEF, ejection fractionFontan circulationICU, intensive care unitIVF, impaired ventricular functionLV, left ventricularMELD-XI, model for end-stage liver disease score excluding the international normalized ratioOHT, orthotopic heart transplantationPGD, primary graft dysfunctionPVF, Preserved ventricular functionRV, right ventricularadult congenital heart diseaseheart transplantation

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