TY - JOUR
T1 - Long-Term Outcomes of Hypertrophic Cardiomyopathy Diagnosed during Childhood
T2 - Results from a National Population-Based Study
AU - National Australian Childhood Cardiomyopathy Study
AU - Alexander, Peta M.A.
AU - Nugent, Alan
AU - Daubeney, Piers E.F.
AU - Lee, Katherine J.
AU - Sleeper, Lynn A.
AU - Schuster, Tibor
AU - Turner, Christian
AU - Davis, Andrew M.
AU - Semsarian, Chris
AU - Colan, Steven D.
AU - Robertson, Terry
AU - Ramsay, James
AU - Justo, Robert
AU - Sholler, Gary F.
AU - King, Ingrid
AU - Weintraub, Robert G.
N1 - Funding Information:
This study was supported by grant 98001 from The Royal Children’s Hospital Research Foundation, Melbourne, Australia; grants G 98M 0159, G 04M 1586, G 05M 2151, and G 07M 3180 from the National Heart Foundation of Australia, Sydney; an NACCS grant from the Australia and New Zealand Children’s Heart Research Center, Melbourne, Australia; and an NACCS grant from Heart-kids Australia, Sydney. Dr Daubeney is supported by the Biomedical Research Unit at the Royal Brompton Hospital, London, United Kingdom. Dr Semsarian is the recipient of a National Health and Medical Research Committee Practitioner Fellowship (1059156), Canberra, Australia.
Publisher Copyright:
© 2018 American Heart Association, Inc.
PY - 2018
Y1 - 2018
N2 - 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; P0.001), and lower fractional shortening z score (hazard ratio, 0.84; 95% CI, 0.74-0.95; P=0.005) during followup. 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 cardioverterdefibrillator. 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.
AB - 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; P0.001), and lower fractional shortening z score (hazard ratio, 0.84; 95% CI, 0.74-0.95; P=0.005) during followup. 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 cardioverterdefibrillator. 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.
KW - Cardiovascular surgery
KW - Myocardial cardiomyopathy disease
KW - Pediatric and congenital heart disease
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U2 - 10.1161/CIRCULATIONAHA.117.028895
DO - 10.1161/CIRCULATIONAHA.117.028895
M3 - Article
C2 - 29490994
AN - SCOPUS:85055739609
SN - 0009-7322
VL - 138
SP - 29
EP - 36
JO - Circulation
JF - Circulation
IS - 1
ER -