Catecholaminergic Polymorphic VT (CPVT)
Catecholaminergic Polymorphic VT (CPVT) is a rare but highly lethal inherited arrhythmia syndrome characterized by stress- or exercise-induced polymorphic ventricular tachycardia. This session explores the genetic, electrophysiological, and clinical complexities of CPVT, helping clinicians recognize early warning signs, navigate diagnostic uncertainty, and implement life-saving interventions. As specialists increasingly search for a focused cardiology conference, this session provides the essential foundation for timely diagnosis, genetic counselling, risk stratification, and personalized therapeutic strategies.
The description emphasizes that CPVT often affects children and young adults with structurally normal hearts and normal resting ECGs, making early detection challenging. Participants will review the underlying mechanisms involving RyR2 and CASQ2 mutations, adrenergically triggered calcium release abnormalities, and triggered activity that leads to bidirectional or polymorphic VT. The session highlights common presentations—syncope during exercise, emotional stress triggers, seizures mistaken for epilepsy, and sudden cardiac arrest in seemingly healthy individuals. Attendees will learn how to use exercise testing, ambulatory monitoring, and genetic testing to confirm the diagnosis with confidence.
A central theme focuses on implementing advanced CPVT management strategies. Participants will explore beta-blocker selection, flecainide use, ICD decision-making, lifestyle modification, and exercise restrictions. Special attention is given to risk profiles, including the challenge of deciding when ICD implantation may increase rather than decrease risk due to shock-triggered adrenergic surges. Case-based discussion helps clinicians manage partial responders, drug intolerance, and complex scenarios involving athletes, children, and families with multiple affected members.
The session concludes by reviewing emerging therapies such as gene-based approaches, advanced autonomic modulation, and personalized risk prediction models. By the end, attendees will be prepared to diagnose CPVT early, stabilize high-risk patients, and deliver multidisciplinary care that prevents catastrophic arrhythmic events.
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Core CPVT Concepts
Genetic and Molecular Mechanisms
- Understanding how mutations disrupt calcium handling under stress.
- Recognizing inheritance patterns and implications for family screening.
Diagnostic Challenges and Tools
- Identifying characteristic features on stress testing and monitoring.
- Knowing when and how to apply genetic testing for confirmation.
Tailored Therapeutic Decision-Making
- Using beta-blockers, flecainide, and selective ICD implantation.
- Balancing exercise restriction with lifestyle quality and safety.
Long-Term Management and Family Counselling
- Providing lifelong monitoring for high-risk individuals.
- Offering counselling for relatives needing cascade testing.
Clinical and System Impact
Earlier Detection of High-Risk Patients
Timely recognition prevents sudden cardiac death in young individuals.
More Targeted Treatment Plans
Physiology-driven therapy improves long-term stability.
Improved Family Risk Identification
Cascade testing reduces undiagnosed case rates.
Reduced Emergency Interventions
Optimized therapy minimizes arrhythmic episodes.
Better Psychosocial Support
Families receive structured counselling and reassurance.
Enhanced Multidisciplinary Collaboration
Genetics, cardiology, and EP teams work cohesively.
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