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Long-Term Follow-Up of Patients with Catecholaminergic Polymorphic Ventricular Arrhythmia
Michael Veith1, Ibrahim El-Battrawy1,2, Gretje Roterberg1
1First Department of Medicine, Faculty of Medicine, University Medical Centre Mannheim (UMM), University of Heidelberg, Mannheim 68167, Germany.
Journal of Clinical Medicine
|March 29, 2020
Summary
Catecholaminergic polymorphic ventricular tachycardia (CPVT) patients face a high risk of cardiac events despite treatment. Early diagnosis, family screening, and tailored therapies are crucial for managing this inherited arrhythmia.
Area of Science:
- Cardiology
- Genetics
- Electrophysiology
Background:
- Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare, inherited channelopathy.
- It causes life-threatening arrhythmias and requires long-term outcome studies.
Purpose of the Study:
- To summarize clinical profiles, treatment approaches, and long-term outcomes in CPVT patients.
- To assess the risk of adverse cardiac events in a CPVT cohort.
Main Methods:
- Retrospective and prospective data collection from nine CPVT patients across seven families.
- Analysis of clinical presentation, genetic mutations (RYR2), treatment strategies (beta-blockers, flecainide), and adverse events.
Main Results:
- Median follow-up of 8.6 years; mean age at diagnosis 26.4 years.
- Five patients had RYR2 mutations; seven received beta-blockers, four with added flecainide.
- Despite treatment, three patients experienced adverse cardiac events; seven received ICDs. Ventricular arrhythmias occurred frequently.
Conclusions:
- The CPVT cohort demonstrated a high incidence of cardiac events.
- Optimal medical therapy, family screening, and individualized treatment are essential for CPVT management in referral centers.
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