Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2006 Sep;152(3):427-33.
doi: 10.1016/j.ahj.2006.02.009.

Design of a large cross-sectional study to facilitate future clinical trials in children with the Fontan palliation

Lynn A Sleeper  1 , Page Anderson, Daphne T Hsu, Lynn Mahony, Brian W McCrindle, Stephen J Roth, J Phillip Saul, Richard V Williams, Tal Geva, Steven D Colan, Bernard J Clark; Pediatric Heart Network Investigators
Affiliations

Design of a large cross-sectional study to facilitate future clinical trials in children with the Fontan palliation

Lynn A Sleeper et al. Am Heart J. 2006 Sep.

Abstract

Background: Clinical trials in children with congenital heart disease are often limited by the absence of the following: (1) a primary outcome that can be observed in a reasonable period; (2) information regarding health-related quality of life; (3) knowledge of the correlation between health status and ventricular function and exercise performance; (4) a sufficient number of children at a single institution to provide adequate statistical power; and (5) procedural and management differences between and within institutions.

Methods: The NHLBI-funded Pediatric Heart Network designed a cross-sectional study of children aged 6 to 18 years, from 7 pediatric clinical centers, who had undergone a Fontan procedure as treatment for congenital heart disease. Health-related quality of life was measured by the Child Health Questionnaire and the Congenital Heart Adolescent and Teenager Questionnaire. Ventricular function was assessed by maximal exercise testing, echocardiography, cardiac magnetic resonance imaging, and B-type natriuretic peptide. The study was designed to detect a correlation of R > or = 0.30 between health status scores and measures of ventricular function and performance in a subcohort with all study measures completed.

Results: A total of 1078 children were screened by chart review; 644 (60%) were eligible. The consent rate was 85% and 546 children were enrolled. Acquisition of echocardiograms, B-type natriuretic peptide, and health status was > or = 94%; completion rates were lower for maximal exercise testing (76%) and cardiac magnetic resonance imaging (41%).

Conclusions: This large study provides unique information regarding the relationship between health status and clinical measures in post-Fontan patients that will facilitate the design of future randomized trials.

PubMed Disclaimer

Figures

Figure 1
Figure 1. Fontan Cross-Sectional Study Flow and Patient enrollment
Abbreviations in figure: CHQ = Child Health Questionnaire CHAT = Congenital Heart Adolescent and Teenager questionnaire Echo = Echocardiogram BNP = B-Type Natriuretic Peptide MRI = Magnetic Resonance Imaging (cardiac)
See this image and copyright information in PMC

References

    1. Marino BS. Outcomes after the Fontan procedure. Curr Opin Pediatr. 2002;14(5):620–626. - PubMed
    1. van den Bosch AE, Roos-Hesselink JW, Van Domburg R, et al. Long-term outcome and quality of life in adult patients after the Fontan operation. Am J Cardiol. 2004;93(9):1141–1145. - PubMed
    1. Penny DJ, Rigby ML, Redington A. Abnormal patterns of intraventricular flow and diastolic filling after the Fontan operation: evidence of incoordinate ventricular wall motion. Br Heart J. 1991;66:375–378. - PMC - PubMed
    1. Fogel M, Weinber P, Chin A, Fellows K, et al. Late ventricular geometry and performance changes of functional single ventricle throughout staged Fontan reconstruction assessed by magnetic resonance imaging. J Am Coll Cardiol. 1996;28:212–221. - PubMed
    1. Fogel MA. Assessment of cardiac function by MRI. Pediatr Cardiol. 2000;21:59–69. - PubMed

Publication types

MeSH terms