Long-Term Outcomes of Hypertrophic Cardiomyopathy Diagnosed During Childhood

Long-Term Outcomes of Hypertrophic Cardiomyopathy Diagnosed During Childhood.

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Item Type: Article
Status: Published
Official URL: https://doi.org/10.1161/CIRCULATIONAHA.117.028895
Journal or Publication Title: Circulation
Volume: 138
Number: 1
Page Range: pp. 29-36
Date: 2018
Divisions: Molecular Cardiology
Depositing User: General Admin
Identification Number: 10.1161/CIRCULATIONAHA.117.028895
ISSN: 0009-7322
Date Deposited: 03 Jan 2021 22:24
Abstract:

Background: Late survival and symptomatic status of children with hypertrophic cardiomyopathy have not been well defined. We examined long-term outcomes for pediatric hypertrophic cardiomyopathy.

Methods: The National Australian Childhood Cardiomyopathy Study is a longitudinal population-based cohort study of children (0-10 years of age) diagnosed with cardiomyopathy between 1987 and 1996. The primary study end point was time to death or cardiac transplantation.

Results: There were 80 patients with hypertrophic cardiomyopathy, with a median age at diagnosis of 0.48 (interquartile range, 0.1, 2.5) years. Freedom from death/transplantation was 86% (95% confidence interval [CI], 77.0-92.0) 1 year after presentation, 80% (95% CI, 69.0-87.0) at 10 years, and 78% (95% CI, 67.0-86.0) at 20 years. From multivariable analyses, risk factors for death/transplantation included symmetrical left ventricular hypertrophy at the time of diagnosis (hazard ratio, 4.20; 95% CI, 1.60-11.05; P=0.004), Noonan syndrome (hazard ratio, 2.88; 95% CI, 1.02-8.08; P=0.045), higher posterior wall thickness z score (hazard ratio, 1.45; 95% CI, 1.22-1.73; P<0.001), and lower fractional shortening z score (hazard ratio, 0.84; 95% CI, 0.74-0.95; P=0.005) during follow-up. Nineteen (23%) subjects underwent left ventricular myectomy. At a median of 15.7 years of follow-up, 27 (42%) of 63 survivors were treated with β-blocker, and 13 (21%) had an implantable cardioverter-defibrillator.

Conclusions: The highest risk of death or transplantation for children with hypertrophic cardiomyopathy is within 1 year after diagnosis, with low attrition rates thereafter. Many subjects receive medical, surgical, or device therapy.

Keywords: cardiovascular surgery; myocardial cardiomyopathy disease; pediatric and congenital heart disease.

© 2018 American Heart Association, Inc.

Creators:
Creators
Email
Alexander, Peta M.A.
UNSPECIFIED
Nugent, Alan W.
UNSPECIFIED
Daubeney, Piers E.F.
UNSPECIFIED
Lee, Katherine J.
UNSPECIFIED
Sleeper, Lynn A.
UNSPECIFIED
Schuster, Tibor
UNSPECIFIED
Turner, Christian
UNSPECIFIED
Davis, Andrew M.
UNSPECIFIED
Semsarian, Chris
UNSPECIFIED
Colan, Steven D.
UNSPECIFIED
Robertson, Terry
UNSPECIFIED
Ramsay, James
UNSPECIFIED
Justo, Robert
UNSPECIFIED
Sholler, Gary F.
UNSPECIFIED
King, Ingrid
UNSPECIFIED
Weintraub, Robert G.
UNSPECIFIED
Last Modified: 03 Jan 2021 22:24
URI: https://eprints.centenary.org.au/id/eprint/561

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