Imperial College London

DrPiersDaubeney

Faculty of MedicineNational Heart & Lung Institute

Professor of Practice (Paediatric Cardiology)
 
 
 
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Contact

 

+44 (0)20 7351 8430p.daubeney

 
 
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Location

 

Royal BromptonRoyal Brompton Campus

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Summary

 

Publications

Citation

BibTex format

@article{Alexander:2018:10.1161/CIRCULATIONAHA.117.028895,
author = {Alexander, PMA and Nugent, AW and Daubeney, PEF and Lee, KJ and Sleeper, LA and Schuster, T and Turner, C and Davis, AM and Semsarian, C and Colan, SD and Robertson, T and Ramsay, J and Justo, R and Sholler, GF and King, I and Weintraub, RG and National, Australian Childhood Cardiomyopathy Study},
doi = {10.1161/CIRCULATIONAHA.117.028895},
journal = {Circulation},
pages = {29--36},
title = {Long-term outcomes of hypertrophic cardiomyopathy diagnosed during childhood: results from a national population-based study},
url = {http://dx.doi.org/10.1161/CIRCULATIONAHA.117.028895},
volume = {138},
year = {2018}
}

RIS format (EndNote, RefMan)

TY  - JOUR
AB - 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.
AU - Alexander,PMA
AU - Nugent,AW
AU - Daubeney,PEF
AU - Lee,KJ
AU - Sleeper,LA
AU - Schuster,T
AU - Turner,C
AU - Davis,AM
AU - Semsarian,C
AU - Colan,SD
AU - Robertson,T
AU - Ramsay,J
AU - Justo,R
AU - Sholler,GF
AU - King,I
AU - Weintraub,RG
AU - National,Australian Childhood Cardiomyopathy Study
DO - 10.1161/CIRCULATIONAHA.117.028895
EP - 36
PY - 2018///
SN - 0009-7322
SP - 29
TI - Long-term outcomes of hypertrophic cardiomyopathy diagnosed during childhood: results from a national population-based study
T2 - Circulation
UR - http://dx.doi.org/10.1161/CIRCULATIONAHA.117.028895
UR - https://www.ncbi.nlm.nih.gov/pubmed/29490994
UR - http://hdl.handle.net/10044/1/57928
VL - 138
ER -