Longitudinal Outcomes of Patients With Single Ventricle After the Fontan Procedure

Multicenter longitudinal objective data for survival into adulthood of patients who have undergone Fontan procedures are lacking. This study sought to describe transplant-free survival and explore relationships between laboratory measures of ventricular performance and functional status over time. E...

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Bibliographic Details
Published in:Journal of the American College of Cardiology Vol. 69; no. 22; p. 2735
Main Authors: Atz, Andrew M, Zak, Victor, Mahony, Lynn, Uzark, Karen, D'agincourt, Nicholas, Goldberg, David J, Williams, Richard V, Breitbart, Roger E, Colan, Steven D, Burns, Kristin M, Margossian, Renee, Henderson, Heather T, Korsin, Rosalind, Marino, Bradley S, Daniels, Kaitlyn, McCrindle, Brian W
Format: Journal Article
Language:English
Published: United States 06.06.2017
Subjects:
Adolescent
Child
Cross-Sectional Studies
Echocardiography
Female
Follow-Up Studies
Fontan Procedure - methods
Forecasting
Health Status
Heart Defects, Congenital - diagnosis
Heart Defects, Congenital - mortality
Heart Defects, Congenital - surgery
Heart Ventricles - abnormalities
Heart Ventricles - diagnostic imaging
Humans
Male
Ontario - epidemiology
Quality of Life
Retrospective Studies
Risk Factors
Survival Rate - trends
Treatment Outcome
United States - epidemiology
Young Adult
functional health status
exercise
Fontan procedure
adult congenital heart disease
single ventricle
ISSN:1558-3597, 1558-3597
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Summary:Multicenter longitudinal objective data for survival into adulthood of patients who have undergone Fontan procedures are lacking. This study sought to describe transplant-free survival and explore relationships between laboratory measures of ventricular performance and functional status over time. 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. 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 (R 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. 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).
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ISSN:1558-3597
1558-3597
DOI:10.1016/j.jacc.2017.03.582