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.

Trial registration: ClinicalTrials.gov NCT00132782.

Keywords: Congenital; Fontan Procedure; Functional Health Status; Heart Defects; Pediatrics.

Figure 1

Transplant-free survival since Fontan 1 (with 95% confidence intervals) in a cohort of 532 subjects with complete follow-up data. For improved resolution, the scale on the y-axis is limited to 0.9–1.0.

Figure 2

Association of interim (between Fontan 1 and Fontan 2) transplant-free survival with CHQ-PF50 physical summary score (A); serum BNP level (B); and both (C). For improved resolution, the scale on the y-axis is limited to 0.75–1.0. BNP, brain natriuretic peptide; CHQ, Child Health Questionnaire.

Figure 3

The distribution of Child Health Questionnaire-Parent Form (CHQ-PF50) physical (A) and psychosocial (B) summary scores from 257 children enrolled in the Fontan 2 Study. The line shows the normal distribution for the general population. SD, standard deviation.

Figure 4

The distribution of Short Form 36 (SF-36) physical (A) and mental aggregate (B) scores from 153 adults enrolled in the Fontan 2 Study. The line shows the normal distribution for the general population. SD, standard deviation.