Original Clinical Science
Clinical and hemodynamic characteristics of the pediatric failing Fontan

https://doi.org/10.1016/j.healun.2021.07.017Get rights and content

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/m2). In the entire cohort, 66% had FP >15 mmHg, 21% had FP >20 mmHg, and 10% had CI <2.2 L/min/m2. 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.

Section snippets

Patient cohort

Using the Pediatric Heart Transplant Society (PHTS) database, we identified all children <18 years of age with a functional single ventricle anomaly who had undergone a Fontan procedure and were listed for heart transplantation between January 1, 2005, and December 31, 2013. The PHTS database is an event-driven registry of pediatric patients listed for transplant and details of the registry have been previously published.7 Data for this study were drawn from 16 PHTS centers in an era before the

Clinical characteristics

Sixteen PHTS centers contributed supplemental data on 208 Fontan patients listed for heart transplantation between January 1, 2005, and December 31, 2013. Twenty-eight patients were excluded due to incomplete hemodynamic data, three were excluded due to being on ECMO at time of listing, and no patients were on a ventricular assist device (VAD) at time of listing, resulting in a final study cohort of 177 patients. Categorization of the study cohort into the 4Fontan phenotypes is outlined in

Discussion

This study provides a comprehensive description of the clinical and hemodynamic characteristics of pediatric Fontan patients listed for heart transplant in the Pediatric Heart Transplant Society database. Pediatric Fontan failure is a unique entity – the majority of patients do not present with the classic findings of patients in heart failure. In our study, less than 10% have tachypnea, orthopnea, gallop, or elevated jugular venous distention. Even heart failure symptoms more prevalent in

Conclusions and future directions

Children with Fontan failure do not present with the classic clinical manifestations seen in adult heart failure, and their signs and symptoms also differ from more common presentations of pediatric heart failure. Elevated Fontan pressure is more common than decreased cardiac output, and is more strongly associated with end-organ dysfunction than cardiac output. Overall outcomes vary depending on the phenotype of Fontan failure.

The observations from this study add to the understanding of

Disclosure Statement

This study complies with the Declaration of Helsinki and the research was approved by Stanford University Institutional Review Board. Participating centers contributed supplemental data to the Pediatric Heart Transplant Society under Institutional Review Board approval or waiver of consent where applicable.

Acknowledgments

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

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