Survival data and predictors of functional outcome an average of 15 years after the Fontan procedure: the pediatric heart network Fontan cohort.

Abstract

OBJECTIVE:

Multicenter longitudinal outcome data for Fontan patients surviving into adulthood are lacking. The aim of this study was to better understand contemporary outcomes in Fontan survivors by collecting follow-up data in a previously well-characterized cohort.

DESIGN:

Baseline data from the Fontan Cross-Sectional Study (Fontan 1) were previously obtained in 546 Fontan survivors aged 11.9 ± 3.4 years. We assessed current transplant-free survival status in all subjects 6.8 ± 0.4 years after the Fontan 1 study. Anatomic, clinical, and surgical data were collected along with socioeconomic status and access to health care.

RESULTS:

Thirty subjects (5%) died or underwent transplantation since Fontan 1. Subjects with both an elevated (>21 pg/mL) brain natriuretic peptide and a low Child Health Questionnaire physical summary score (<44) measured at Fontan 1 were significantly more likely to die or undergo transplant than the remainder, with a hazard ratio of 6.2 (2.9-13.5). Among 516 Fontan survivors, 427 (83%) enrolled in this follow-up study (Fontan 2) at 18.4 ± 3.4 years of age. Although mean scores on functional health status questionnaires were lower than the general population, individual scores were within the normal range in 78% and 88% of subjects for the Child Health Questionnaire physical and psychosocial summary score, and 97% and 91% for the SF-36 physical and mental aggregate score, respectively. Since Fontan surgery, 119 (28%) had additional cardiac surgery; 55% of these (n = 66) in the interim between Fontan 1 and Fontan 2. A catheter intervention occurred in 242 (57%); 32% of these (n = 78) after Fontan 1. Arrhythmia requiring treatment developed in 118 (28%) after Fontan surgery; 58% of these (n = 68) since Fontan 1.

CONCLUSIONS:

We found 95% interim transplant-free survival for Fontan survivors over an average of 7 years of follow-up. Continued longitudinal investigation into adulthood is necessary to better understand the determinants of long-term outcomes and to improve functional health status.

• Authors: Andrew M Atz, Victor Zak, Lynn Mahony, Karen Uzark, Peter Shrader, Dianne Gallagher, Stephen M Paridon, Richard V Williams, Roger E Breitbart, Steven D Colan, Jonathan R Kaltman, Renee Margossian, Sara K Pasquali, Kerstin Allen, Wyman W Lai, Rosalind Korsin, Bradley S Marino, Nicole Mirarchi, Brian W McCrindle, Pediatric Heart Network Investigators
• Journal: Congenital heart disease (Congenit Heart Dis) 2015 Jan-Feb Vol. 10 Issue 1 Pg. E30-42 ISSN: 1747-0803 [Electronic] United States
• PMID: 24934522 (Publication Type: Journal Article, Multicenter Study, Research Support, N.I.H., Extramural)
• Copyright: © 2014 Wiley Periodicals, Inc.

Chemical References

• Biomarkers
• Natriuretic Peptide, Brain

Topics

• Adolescent
• Biomarkers (blood)
• Canada
• Disease-Free Survival
• Female
• Fontan Procedure (adverse effects, mortality)
• Health Services Accessibility
• Health Status
• Heart Defects, Congenital (diagnosis, mortality, physiopathology, surgery)
• Heart Transplantation
• Humans
• Kaplan-Meier Estimate
• Longitudinal Studies
• Male
• Natriuretic Peptide, Brain (blood)
• Proportional Hazards Models
• Prospective Studies
• Risk Assessment
• Risk Factors
• Socioeconomic Factors
• Surveys and Questionnaires
• Time Factors
• Treatment Outcome
• United States
• Young Adult