Format

Send to

Choose Destination
J Am Coll Cardiol. 2017 Jun 6;69(22):2735-2744. doi: 10.1016/j.jacc.2017.03.582.

Longitudinal Outcomes of Patients With Single Ventricle After the Fontan Procedure.

Author information

1
Medical University of South Carolina, Charleston, South Carolina. Electronic address: atzam@musc.edu.
2
New England Research Institutes, Watertown, Massachusetts.
3
University of Texas Southwestern Medical Center, Dallas, Texas.
4
University of Michigan, Ann Arbor, Michigan.
5
Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
6
University of Utah, Salt Lake City, Utah.
7
Boston Children's Hospital, Boston, Massachusetts.
8
National Heart, Lung, and Blood Institute, Bethesda, Maryland.
9
Duke University Medical Center, Durham, North Carolina.
10
Columbia University Medical Center, New York, New York.
11
Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.
12
University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada.

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:

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

PMID:
28571639
PMCID:
PMC5604334
DOI:
10.1016/j.jacc.2017.03.582
[Indexed for MEDLINE]
Free PMC Article

Supplemental Content

Full text links

Icon for Elsevier Science Icon for PubMed Central
Loading ...
Support Center