Long-term outcomes of hypertrophic cardiomyopathy diagnosed during childhood: results from a national population-based study

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Title: Long-term outcomes of hypertrophic cardiomyopathy diagnosed during childhood: results from a national population-based study
Author(s): Alexander, PMA
Nugent, AW
Daubeney, PEF
Lee, KJ
Sleeper, LA
Schuster, T
Turner, C
Davis, AM
Semsarian, C
Colan, SD
Robertson, T
Ramsay, J
Justo, R
Sholler, GF
King, I
Weintraub, RG
National Australian Childhood Cardiomyopathy Study
Item Type: Journal Article
Abstract: Background -Late survival and symptomatic status of children with hypertrophic cardiomyopathy (HCM) have not been well defined. We examined long-term outcomes for pediatric HCM. Methods -The National Australian Childhood Cardiomyopathy Study is a longitudinal population-based cohort study of children (0-10 years) 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 HCM with median age at diagnosis of 0.48 (Inter-quartile range [IQR] 0.1, 2.5) years. Freedom from death/transplantation (95% confidence interval [CI]) was 86 (77-92)% one year after presentation, 80 (69-87)% at 10 years and 78 (67-86)% at 20 years. From multivariable analyses, risk factors for death/transplantation included symmetric left ventricular hypertrophy at the time of diagnosis (hazard ratio [HR] 4.20 95%CI 1.60, 11.05 p=0.004), Noonan syndrome (HR 2.88, 95%CI 1.02, 8.08, p=0.045), higher posterior wall thickness z-score (HR 1.45, 95%CI 1.22, 1.73, p<0.001) and lower fractional shortening z-score (HR 0.84, 95%CI 0.74, 0.95, p=0.005) during follow-up. Nineteen (23%) subjects underwent left ventricular myectomy. At median 15.7 years' follow-up, 27 (42%) of 63 survivors were treated with beta-blocker and 13 (21%) had an implantable cardioverter-defibrillator. Conclusions -The highest risk of death or transplantation for children with HCM is within one year post-diagnosis, with low attrition rates thereafter. Many subjects receive medical, surgical or device therapy.
Publication Date: 3-Jul-2018
Date of Acceptance: 15-Feb-2018
URI: http://hdl.handle.net/10044/1/57928
DOI: https://dx.doi.org/10.1161/CIRCULATIONAHA.117.028895
ISSN: 0009-7322
Publisher: American Heart Association
Start Page: 29
End Page: 36
Journal / Book Title: Circulation
Volume: 138
Issue: 1
Copyright Statement: ©2018 American Heart Association, Inc. All rights reserved.
Keywords: Myocardial cardiomyopathy disease
Pediatric and congenital heart disease, including cardiovascular surgery
National Australian Childhood Cardiomyopathy Study
Myocardial cardiomyopathy disease
Pediatric and congenital heart disease, including cardiovascular surgery
1103 Clinical Sciences
1102 Cardiovascular Medicine And Haematology
1117 Public Health And Health Services
Cardiovascular System & Hematology
Publication Status: Published
Conference Place: United States
Online Publication Date: 2018-02-28
Appears in Collections:National Heart and Lung Institute
Faculty of Medicine



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