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

Pediatric Heart Network Investigators

Research output: Contribution to journalArticle

34 Citations (Scopus)

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 ± 3.4years. We assessed current transplant-free survival status in all subjects 6.8 ± 0.4years 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 (>21pg/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 ± 3.4years 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 7years 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.

Original languageEnglish (US)
Pages (from-to)E30-E42
JournalCongenital Heart Disease
Volume10
Issue number1
DOIs
StatePublished - Jan 1 2015

Fingerprint

Fontan Procedure
Survivors
Pediatrics
Survival
Transplants
Health Status
Health Services Accessibility
Brain Natriuretic Peptide
Social Class
Thoracic Surgery
Cardiac Arrhythmias
Reference Values
Catheters
Cross-Sectional Studies
Transplantation
Population
Surveys and Questionnaires
Child Health

Keywords

  • Congenital
  • Fontan procedure
  • Functional health status
  • Heart defects
  • Pediatrics

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Pediatrics, Perinatology, and Child Health
  • Surgery
  • Radiology Nuclear Medicine and imaging

Cite this

Survival data and predictors of functional outcome an average of 15years after the fontan procedure : The pediatric heart network fontan cohort. / Pediatric Heart Network Investigators.

In: Congenital Heart Disease, Vol. 10, No. 1, 01.01.2015, p. E30-E42.

Research output: Contribution to journalArticle

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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 ± 3.4years. We assessed current transplant-free survival status in all subjects 6.8 ± 0.4years 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 (>21pg/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 ± 3.4years 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 7years 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.",
keywords = "Congenital, Fontan procedure, Functional health status, Heart defects, Pediatrics",
author = "{Pediatric Heart Network Investigators} and Atz, {Andrew M.} and Victor Zak and Lynn Mahony and Karen Uzark and Peter Shrader and Dianne Gallagher and Paridon, {Stephen M.} and Williams, {Richard V.} and Breitbart, {Roger E.} and Colan, {Steven D.} and Kaltman, {Jonathan R.} and Renee Margossian and Pasquali, {Sara K.} and Kerstin Allen and Lai, {Wyman W.} and Rosalind Korsin and Marino, {Bradley S.} and Nicole Mirarchi and Mccrindle, {Brian W.}",
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T2 - The pediatric heart network fontan cohort

AU - Pediatric Heart Network Investigators

AU - Atz, Andrew M.

AU - Zak, Victor

AU - Mahony, Lynn

AU - Uzark, Karen

AU - Shrader, Peter

AU - Gallagher, Dianne

AU - Paridon, Stephen M.

AU - Williams, Richard V.

AU - Breitbart, Roger E.

AU - Colan, Steven D.

AU - Kaltman, Jonathan R.

AU - Margossian, Renee

AU - Pasquali, Sara K.

AU - Allen, Kerstin

AU - Lai, Wyman W.

AU - Korsin, Rosalind

AU - Marino, Bradley S.

AU - Mirarchi, Nicole

AU - Mccrindle, Brian W.

PY - 2015/1/1

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N2 - 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 ± 3.4years. We assessed current transplant-free survival status in all subjects 6.8 ± 0.4years 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 (>21pg/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 ± 3.4years 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 7years 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.

AB - 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 ± 3.4years. We assessed current transplant-free survival status in all subjects 6.8 ± 0.4years 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 (>21pg/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 ± 3.4years 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 7years 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.

KW - Congenital

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KW - Functional health status

KW - Heart defects

KW - Pediatrics

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