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How to assess sudden cardiac death risk in hypertrophic cardiomyopathy? Current challenges and future directions
Gabrielle Norrish1,2, Małgorzata Niemiec3,4, Juan P Kaski1,2
1Center for Inherited Cardiovascular Diseases, Zayed Center for Research, Great Ormond Street Hospital, London, United Kingdom.
Insights
Sudden cardiac death (SCD) risk in hypertrophic cardiomyopathy (HCM) is better understood, with improved risk models aiding personalized care. Advanced methods, including AI, offer hope for precise SCD risk assessment in HCM patients.
Area of Science:
- Cardiology
- Genetics
- Medical Diagnostics
Background:
- Knowledge of sudden cardiac death (SCD) risk in hypertrophic cardiomyopathy (HCM) has significantly advanced.
- Standard risk stratification models are crucial for shared decision-making in HCM patient care.
Purpose of the Study:
- To review current understanding and approaches to SCD risk stratification in hypertrophic cardiomyopathy.
- To highlight advanced indicators and challenges in risk assessment for specific HCM patient groups.
Main Methods:
- Review of recent advancements in SCD risk stratification for hypertrophic cardiomyopathy.
- Analysis of fundamental risk factors, risk models, and additional indicators like LVEF, LGE, and genetic variants.
- Exploration of challenges in specific HCM populations and emerging methodologies.
Main Results:
- Established risk factors and models guide SCD risk stratification in HCM.
- Additional indicators (e.g., reduced LVEF, late gadolinium enhancement, genetic variants) refine risk assessment for intermediate-risk patients.
- AI-supported multifactorial approaches show promise for individualized SCD risk prediction.
Conclusions:
- Personalized diagnosis and treatment are enhanced by detailed SCD risk stratification in HCM.
- Risk stratification remains complex in certain HCM patient subsets.
- Future directions involve leveraging advanced methodologies like AI for more precise SCD risk assessment in hypertrophic cardiomyopathy.
Abstract:
Over the past decade, knowledge about the risk of sudden cardiac death (SCD) in patients with hypertrophic cardiomyopathy (HCM) has advanced significantly. A standard well-recognized approach to risk stratification is based on the fundamental risk factors and SCD risk models that should be incorporated into the shared decision-making process. More detailed analysis including additional indicators, such as reduced left ventricular systolic function, the presence of late gadolinium enhancement, or in some cases genetic variants, may provide valuable insights for intermediate-risk patients, enabling more personalized diagnosis and treatment. Risk stratification remains challenging in specific groups, such as patients who have undergone septal reduction therapy, those taking mavacamten, or those with phenocopies of HCM. The advancement of modern methodologies, including multifactorial approaches supported by artificial intelligence algorithms, offers hope for more precise and individualized SCD risk assessment in HCM patients.
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