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Arrhythmias and Sudden Death: What is New in Hypertrophic Cardiomyopathy?
Deepthi Rajan1, Christopher Ryan Zörner2, Morten Lock Hansen2,3
1Department of Cardiology, The Heart Centre, Copenhagen University Hospital, Rigshospitalet Copenhagen, Denmark.
Insights
Hypertrophic cardiomyopathy (HCM) management is advancing with precision diagnostics and targeted therapies. This review covers updated arrhythmia treatments, risk stratification, and novel drugs like myosin inhibitors for better patient outcomes.
Area of Science:
- Cardiology
- Genetics
- Precision Medicine
Background:
- Hypertrophic cardiomyopathy (HCM) is a genetic disorder with varied symptoms, including arrhythmias and sudden cardiac death risk.
- Atrial fibrillation (AF) and ventricular arrhythmias are common and serious complications in HCM patients.
Purpose of the Study:
- To review recent advancements in managing arrhythmias, risk stratification, and targeted therapies for HCM.
- To highlight the role of precision medicine in improving clinical outcomes for HCM patients.
Main Methods:
- Review of current literature on HCM diagnostics, including genetic testing and cardiac imaging.
- Analysis of therapeutic strategies for arrhythmias, such as catheter ablation and ICDs.
- Evaluation of risk stratification models and emerging pharmacological agents, including myosin inhibitors.
Main Results:
- Precision diagnostics refine HCM diagnosis and enable personalized treatment.
- Catheter ablation provides symptom relief for AF in HCM but faces high recurrence rates.
- Risk stratification tools like the ESC HCM Risk-SCD model are crucial for identifying high-risk individuals.
Conclusions:
- Integrated management of HCM requires combining advanced diagnostics, tailored arrhythmia treatments, and precise risk assessment.
- Emerging therapies, including myosin inhibitors, offer new avenues for individualized HCM management.
- A precision medicine approach is essential for optimizing clinical outcomes in hypertrophic cardiomyopathy.
Abstract:
Hypertrophic cardiomyopathy (HCM) is a complex genetic disorder that has garnered significant attention because of its diverse manifestations, including arrhythmias and heightened risk of sudden cardiac death. Advances in precision diagnostics, such as genetic testing and cardiac imaging with late gadolinium enhancement, have refined HCM diagnosis, enabling targeted therapeutic and risk stratification approaches. AF, prevalent in HCM, exacerbates symptoms and stroke risk, while ventricular arrhythmias pose a direct threat to survival. Catheter ablation offers symptom relief in AF patients with HCM, yet recurrence remains high because of unique myocardial changes, highlighting the need for refined patient selection and long-term monitoring. The risk of sudden cardiac death in HCM, particularly in younger individuals, underscores the importance of precise risk stratification tools such as the European Society of Cardiology HCM Risk-SCD model. The expanding role of ICDs and emerging pharmacological agents, including myosin inhibitors, marks a shift toward more individualised management of HCM. This review integrates recent developments in arrhythmia management, targeted therapies and risk assessment, offering a comprehensive perspective on HCM tailored to improve clinical outcomes through a precision-medicine lens.
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