Dr Juan Pablo Kaski MD(Res) FRCP FESC Director of the GOSH Centre for Inherited Cardiovascular Diseases Great Ormond Street Hospital, University College London Institute of Cardiovascular Science, London, UK

Predicting Sudden Death in Children with Hypertrophic Cardiomyopathy

MedicalResearch.com Interview with:

Dr Juan Pablo Kaski MD(Res) FRCP FESC Director of the GOSH Centre for Inherited Cardiovascular Diseases Great Ormond Street Hospital, University College London Institute of Cardiovascular Science, London, UK

Dr. Kaski

Dr Juan Pablo Kaski MD(Res) FRCP FESC
Director of the GOSH Centre for Inherited Cardiovascular Diseases
Great Ormond Street Hospital,
University College London Institute of Cardiovascular Science,
London, UK 

MedicalResearch.com: What is the background for this study? Would you briefly explain what is meant by Hypertrophic Cardiomyopathy? 

Response: Hypertrophic cardiomyopathy (HCM) is a genetic condition characterised by abnormal thickening of the muscle of the heart and can affect people of all ages. It is associated with an increased risk of sudden cardiac death (SCD) and, in the last few years, a clinical risk tool that estimates the 5-year risk of SCD in adults with HCM has been developed.

However, there are no similar risk models in children, where risk stratification has traditionally been based on clinical risk factors extrapolated from the adult population. We have recently shown that this approach does not discriminate risk well in children, and so the aim of this study was to develop a new risk tool to provide an individualised risk of SCD in children with HCM. 

MedicalResearch.com: What are the main findings? 

Response: This was an international multicentre study comprising 1024 children diagnosed with hypertrophic cardiomyopathy aged 16 years or younger, and represents the largest published paediatric HCM cohort. Using preselected predictor variables routinely available in the clinic, we developed and internally validated a new model that may provide individualised estimates of SCD risk at 5 years in childhood HCM. The next step is to externally validate this model to demonstrate its accuracy.

MedicalResearch.com: What should readers take away from your report?

Response: This is the first validated approach to risk stratification in paediatric hypertrophic cardiomyopathy, and may be useful to guide implantable cardioverter-defibrillator (ICD) implantation in young patients with HCM, and provides a starting point for further refinement of the model and assessment of its clinical utility. 

MedicalResearch.com: What recommendations do you have for future research as a result of this work? 

Response: The next step will be to validate the model externally, to see how accurately the model is able to predict risk in an independent, diverse population. In addition, future studies to identify additional risk markers to refine the model will be important.

Disclosures: The study was supported by a British Heart Foundation grant to Dr Norrish and Dr Kaski.

 

Citation:

Norrish G, Ding T, Field E, et al. Development of a Novel Risk Prediction Model for Sudden Cardiac Death in Childhood Hypertrophic Cardiomyopathy (HCM Risk-Kids). JAMA Cardiol. Published online August 14, 2019. doi:10.1001/jamacardio.2019.2861 

 

Aug 14, 2019 @ 3:19 pm

 

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