Risk factors for sudden cardiac death in childhood hypertrophic cardiomyopathy: A systematic review and meta-analysis

@article{Norrish2017RiskFF,
  title={Risk factors for sudden cardiac death in childhood hypertrophic cardiomyopathy: A systematic review and meta-analysis},
  author={Gabrielle Norrish and Nicoletta Cantarutti and Eleni Pissaridou and Deborah Ridout and Giuseppe Limongelli and Perry M. Elliott and Juan Pablo Kaski},
  journal={European Journal of Preventive Cardiology},
  year={2017},
  volume={24},
  pages={1220 - 1230}
}
Aims To perform a systematic literature review and meta-analysis of clinical risk factors for sudden cardiac death (SCD) in childhood hypertrophic cardiomyopathy. Methods Medline and PubMed databases were searched for original articles published in English from 1963 through to December 2015 that included patients under 18 years of age with a primary or secondary end-point of either SCD or SCD-equivalent events (aborted cardiac arrest or appropriate implantable cardioverter–defibrillator… 

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TLDR
ST and inducible VT were the most significant univariate predictors of risk for an AE in children with hypertrophic cardiomyopathy without a previous AE in patients age under 21 years.
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Early mortality is high in childhood dilated cardiomyopathy, but the clinical status of long-term survivors is good, and this population-based study identifies children at risk of adverse events.
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TLDR
In families with HCM, children should be screened at an early age, and national cause of death statistics confirm that the mortality rate from HCM is significantly higher in the 8-16 year olds than in the 17-30 year olds.
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TLDR
This is the first validated SCD risk prediction model for patients with HCM and provides accurate individualized estimates for the probability of SCD using readily collected clinical parameters.
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TLDR
Syndromal, genetic, and metabolic causes predominate in children with hypertrophic cardiomyopathy; Ventricular outflow tract obstruction is common and the clinical status of long-term survivors is good.
Predictors of Long-Term Outcome in Children with Hypertrophic Cardiomyopathy
TLDR
Only prior cardiac arrest, QTc dispersion, and NSVT predicted arrhythmic outcome in patients aged <18 years, and LA size, LV posterior wall thickness, and decreased early transmitral flow velocity were strong independent predictors of HF-related events.
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