Longitudinal Outcomes of Patients With Single Ventricle After the Fontan Procedure

Pediatric Heart Network Investigators

Research output: Contribution to journalArticle

39 Citations (Scopus)

Abstract

Background Multicenter longitudinal objective data for survival into adulthood of patients who have undergone Fontan procedures are lacking. Objectives This study sought to describe transplant-free survival and explore relationships between laboratory measures of ventricular performance and functional status over time. Methods Exercise testing, echocardiography, B-type natriuretic peptide, functional health assessment, and medical history abstraction were repeated 9.4 ± 0.4 years after the Fontan Cross-Sectional Study (Fontan 1) and compared with previous values. Cox regression analysis explored risk factors for interim death or cardiac transplantation. Results From the original cohort of 546 subjects, 466 were contacted again, and 373 (80%) were enrolled at 21.2 ± 3.5 years of age. Among subjects with paired testing, the percent predicted maximum oxygen uptake decreased (69 ± 14% vs. 61 ± 16%; p < 0.001; n = 95), ejection fraction decreased (58 ± 11% vs. 55 ± 10%; p < 0.001; n = 259), and B-type natriuretic peptide increased (median [interquartile range] 13 [7 to 25] pg/mol vs. 18 [9 to 36] pg/mol; p < 0.001; n = 340). At latest follow-up, a lower Pediatric Quality of Life Inventory physical summary score was associated with poorer exercise performance (R2 adjusted = 0.20; p < 0.001; n = 274). Cumulative complications since the Fontan procedure included additional cardiac surgery (32%), catheter intervention (62%), arrhythmia treatment (32%), thrombosis (12%), and protein-losing enteropathy (8%). Since Fontan 1, 54 subjects (10%) have received a heart transplant (n = 23) or died without transplantation (n = 31). The interval risk of death or/cardiac transplantation was associated with poorer ventricular performance and functional health status assessed at Fontan 1, but it was not associated with ventricular morphology, the subject's age, or the type of Fontan connection. Conclusions Interim transplant-free survival over 12 years in this Fontan cohort was 90% and was independent of ventricular morphology. Exercise performance decreased and was associated with worse functional health status. Future interventions might focus on preserving exercise capacity. (Relationship Between Functional Health Status and Ventricular Performance After Fontan—Pediatric Heart Network; NCT00132782)

Original languageEnglish (US)
Pages (from-to)2735-2744
Number of pages10
JournalJournal of the American College of Cardiology
Volume69
Issue number22
DOIs
StatePublished - Jun 6 2017

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Health Status
Fontan Procedure
Brain Natriuretic Peptide
Heart Transplantation
Transplants
Protein-Losing Enteropathies
Exercise
Survival
Thoracic Surgery
Echocardiography
Cardiac Arrhythmias
Thrombosis
Catheters
Cross-Sectional Studies
Transplantation
Regression Analysis
Quality of Life
Pediatrics
Oxygen
Equipment and Supplies

Keywords

  • adult congenital heart disease
  • exercise
  • Fontan procedure
  • functional health status
  • single ventricle

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

Cite this

Longitudinal Outcomes of Patients With Single Ventricle After the Fontan Procedure. / Pediatric Heart Network Investigators.

In: Journal of the American College of Cardiology, Vol. 69, No. 22, 06.06.2017, p. 2735-2744.

Research output: Contribution to journalArticle

@article{34150550ac004f56b30e96a54a33727b,
title = "Longitudinal Outcomes of Patients With Single Ventricle After the Fontan Procedure",
abstract = "Background Multicenter longitudinal objective data for survival into adulthood of patients who have undergone Fontan procedures are lacking. Objectives This study sought to describe transplant-free survival and explore relationships between laboratory measures of ventricular performance and functional status over time. Methods Exercise testing, echocardiography, B-type natriuretic peptide, functional health assessment, and medical history abstraction were repeated 9.4 ± 0.4 years after the Fontan Cross-Sectional Study (Fontan 1) and compared with previous values. Cox regression analysis explored risk factors for interim death or cardiac transplantation. Results From the original cohort of 546 subjects, 466 were contacted again, and 373 (80{\%}) were enrolled at 21.2 ± 3.5 years of age. Among subjects with paired testing, the percent predicted maximum oxygen uptake decreased (69 ± 14{\%} vs. 61 ± 16{\%}; p < 0.001; n = 95), ejection fraction decreased (58 ± 11{\%} vs. 55 ± 10{\%}; p < 0.001; n = 259), and B-type natriuretic peptide increased (median [interquartile range] 13 [7 to 25] pg/mol vs. 18 [9 to 36] pg/mol; p < 0.001; n = 340). At latest follow-up, a lower Pediatric Quality of Life Inventory physical summary score was associated with poorer exercise performance (R2 adjusted = 0.20; p < 0.001; n = 274). Cumulative complications since the Fontan procedure included additional cardiac surgery (32{\%}), catheter intervention (62{\%}), arrhythmia treatment (32{\%}), thrombosis (12{\%}), and protein-losing enteropathy (8{\%}). Since Fontan 1, 54 subjects (10{\%}) have received a heart transplant (n = 23) or died without transplantation (n = 31). The interval risk of death or/cardiac transplantation was associated with poorer ventricular performance and functional health status assessed at Fontan 1, but it was not associated with ventricular morphology, the subject's age, or the type of Fontan connection. Conclusions Interim transplant-free survival over 12 years in this Fontan cohort was 90{\%} and was independent of ventricular morphology. Exercise performance decreased and was associated with worse functional health status. Future interventions might focus on preserving exercise capacity. (Relationship Between Functional Health Status and Ventricular Performance After Fontan—Pediatric Heart Network; NCT00132782)",
keywords = "adult congenital heart disease, exercise, Fontan procedure, functional health status, single ventricle",
author = "{Pediatric Heart Network Investigators} and Atz, {Andrew M.} and Victor Zak and Lynn Mahony and Karen Uzark and Nicholas D'agincourt and Goldberg, {David J.} and Williams, {Richard V.} and Breitbart, {Roger E.} and Colan, {Steven D.} and Burns, {Kristin M.} and Renee Margossian and Henderson, {Heather T.} and Rosalind Korsin and Marino, {Bradley S.} and Kaitlyn Daniels and McCrindle, {Brian W.}",
year = "2017",
month = "6",
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doi = "10.1016/j.jacc.2017.03.582",
language = "English (US)",
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T1 - Longitudinal Outcomes of Patients With Single Ventricle After the Fontan Procedure

AU - Pediatric Heart Network Investigators

AU - Atz, Andrew M.

AU - Zak, Victor

AU - Mahony, Lynn

AU - Uzark, Karen

AU - D'agincourt, Nicholas

AU - Goldberg, David J.

AU - Williams, Richard V.

AU - Breitbart, Roger E.

AU - Colan, Steven D.

AU - Burns, Kristin M.

AU - Margossian, Renee

AU - Henderson, Heather T.

AU - Korsin, Rosalind

AU - Marino, Bradley S.

AU - Daniels, Kaitlyn

AU - McCrindle, Brian W.

PY - 2017/6/6

Y1 - 2017/6/6

N2 - Background Multicenter longitudinal objective data for survival into adulthood of patients who have undergone Fontan procedures are lacking. Objectives This study sought to describe transplant-free survival and explore relationships between laboratory measures of ventricular performance and functional status over time. Methods Exercise testing, echocardiography, B-type natriuretic peptide, functional health assessment, and medical history abstraction were repeated 9.4 ± 0.4 years after the Fontan Cross-Sectional Study (Fontan 1) and compared with previous values. Cox regression analysis explored risk factors for interim death or cardiac transplantation. Results From the original cohort of 546 subjects, 466 were contacted again, and 373 (80%) were enrolled at 21.2 ± 3.5 years of age. Among subjects with paired testing, the percent predicted maximum oxygen uptake decreased (69 ± 14% vs. 61 ± 16%; p < 0.001; n = 95), ejection fraction decreased (58 ± 11% vs. 55 ± 10%; p < 0.001; n = 259), and B-type natriuretic peptide increased (median [interquartile range] 13 [7 to 25] pg/mol vs. 18 [9 to 36] pg/mol; p < 0.001; n = 340). At latest follow-up, a lower Pediatric Quality of Life Inventory physical summary score was associated with poorer exercise performance (R2 adjusted = 0.20; p < 0.001; n = 274). Cumulative complications since the Fontan procedure included additional cardiac surgery (32%), catheter intervention (62%), arrhythmia treatment (32%), thrombosis (12%), and protein-losing enteropathy (8%). Since Fontan 1, 54 subjects (10%) have received a heart transplant (n = 23) or died without transplantation (n = 31). The interval risk of death or/cardiac transplantation was associated with poorer ventricular performance and functional health status assessed at Fontan 1, but it was not associated with ventricular morphology, the subject's age, or the type of Fontan connection. Conclusions Interim transplant-free survival over 12 years in this Fontan cohort was 90% and was independent of ventricular morphology. Exercise performance decreased and was associated with worse functional health status. Future interventions might focus on preserving exercise capacity. (Relationship Between Functional Health Status and Ventricular Performance After Fontan—Pediatric Heart Network; NCT00132782)

AB - Background Multicenter longitudinal objective data for survival into adulthood of patients who have undergone Fontan procedures are lacking. Objectives This study sought to describe transplant-free survival and explore relationships between laboratory measures of ventricular performance and functional status over time. Methods Exercise testing, echocardiography, B-type natriuretic peptide, functional health assessment, and medical history abstraction were repeated 9.4 ± 0.4 years after the Fontan Cross-Sectional Study (Fontan 1) and compared with previous values. Cox regression analysis explored risk factors for interim death or cardiac transplantation. Results From the original cohort of 546 subjects, 466 were contacted again, and 373 (80%) were enrolled at 21.2 ± 3.5 years of age. Among subjects with paired testing, the percent predicted maximum oxygen uptake decreased (69 ± 14% vs. 61 ± 16%; p < 0.001; n = 95), ejection fraction decreased (58 ± 11% vs. 55 ± 10%; p < 0.001; n = 259), and B-type natriuretic peptide increased (median [interquartile range] 13 [7 to 25] pg/mol vs. 18 [9 to 36] pg/mol; p < 0.001; n = 340). At latest follow-up, a lower Pediatric Quality of Life Inventory physical summary score was associated with poorer exercise performance (R2 adjusted = 0.20; p < 0.001; n = 274). Cumulative complications since the Fontan procedure included additional cardiac surgery (32%), catheter intervention (62%), arrhythmia treatment (32%), thrombosis (12%), and protein-losing enteropathy (8%). Since Fontan 1, 54 subjects (10%) have received a heart transplant (n = 23) or died without transplantation (n = 31). The interval risk of death or/cardiac transplantation was associated with poorer ventricular performance and functional health status assessed at Fontan 1, but it was not associated with ventricular morphology, the subject's age, or the type of Fontan connection. Conclusions Interim transplant-free survival over 12 years in this Fontan cohort was 90% and was independent of ventricular morphology. Exercise performance decreased and was associated with worse functional health status. Future interventions might focus on preserving exercise capacity. (Relationship Between Functional Health Status and Ventricular Performance After Fontan—Pediatric Heart Network; NCT00132782)

KW - adult congenital heart disease

KW - exercise

KW - Fontan procedure

KW - functional health status

KW - single ventricle

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