Clinical and hemodynamic characteristics of the pediatric failing Fontan

Pediatric Heart Transplant Society, Rebecca K. Ameduri

Research output: Contribution to journalArticlepeer-review

7 Scopus citations


AIM: To describe the clinical and hemodynamic characteristics of Fontan failure in children listed for heart transplant.

METHODS: In a nested study of the Pediatric Heart Transplant Society, 16 centers contributed information on Fontan patients listed for heart transplant between 2005and 2013. Patients were classified into four mutually exclusive phenotypes: Fontan with abnormal lymphatics (FAL), Fontan with reduced systolic function (FRF), Fontan with preserved systolic function (FPF), and Fontan with "normal" hearts (FNH). Primary outcome was waitlist and post-transplant mortality.

RESULTS: 177 children listed for transplant were followed over a median 13 (IQR 4-31) months, 84 (47%) were FAL, 57 (32%) FRF, 22 (12%) FNH, and 14 (8%) FPF. Hemodynamic characteristics differed between the 4 groups: Fontan pressure (FP) was most elevated with FPF (median 22, IQR 18-23, mmHg) and lowest with FAL (16, 14-20, mmHg); cardiac index (CI) was lowest with FRF (2.8, 2.3-3.4, L/min/m 2). In the entire cohort, 66% had FP >15 mmHg, 21% had FP >20 mmHg, and 10% had CI <2.2 L/min/m 2. FRF had the highest risk of waitlist mortality (21%) and FNH had the highest risk of post-transplant mortality (36%).

CONCLUSIONS: Elevated Fontan pressure is more common than low cardiac output in pediatric failing Fontan patients listed for transplant. Subtle hemodynamic differences exist between the various phenotypes of pediatric Fontan failure. Waitlist and post-transplant mortality risks differ by phenotype.

Original languageEnglish (US)
Pages (from-to)1529-1539
Number of pages11
JournalJournal of Heart and Lung Transplantation
Issue number12
Early online dateJul 29 2021
StatePublished - Dec 2021

Bibliographical note

Funding Information:
The authors would like to thank the data coordinating center at the Pediatric Heart Transplant Society, University of Alabama, and Yulin Zhang at the Clinical and Translational Research Program of the Betty Irene Moore Children's Heart Center, Stanford University, for their assistance in performing this study

Publisher Copyright:
© 2021 International Society for Heart and Lung Transplantation


  • Fontan
  • heart failure
  • heart transplant
  • hemodynamics
  • single ventricle


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