What question did this study set out to answer?

To evaluate the clinical presentation, therapeutic strategies, and long-term outcomes in patients diagnosed with catecholaminergic polymorphic ventricular tachycardia.

March 25, 2020Journal of Clinical MedicineOpen Access

Long-Term Follow-Up of Patients with Catecholaminergic Polymorphic Ventricular Arrhythmia

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Why the study?

CPVT is a rare inherited disorder causing life-threatening arrhythmias, but long-term outcome studies of the channelopathy remain limited.

Key Points

To evaluate the clinical presentation, therapeutic strategies, and long-term outcomes in patients diagnosed with catecholaminergic polymorphic ventricular tachycardia.
Conducted a single-center study combining retrospective and prospective data from 9 patients across 7 families (22% male) with a median follow-up of 8.6 years.
Assessed genetic profiles, initial clinical presentations, pharmacological interventions (beta-blockers and flecainide), and implantable cardioverter defibrillator utilization.
Mean age at diagnosis was 26.4 ± 12 years, with initial presentations of syncope in 4 patients and aborted cardiac arrest in 4 patients; RYR2 mutations occurred in 5 patients.
Treatment included beta-blockers in 7 patients and adjunct flecainide in 4 patients, yet 3 patients suffered 7 adverse cardiac events while receiving beta-blocker therapy.
Implantable cardioverter defibrillators were placed in 7 patients; over follow-up, 3 patients experienced 10 ventricular tachycardia events, 5 experienced 9 ventricular fibrillation events, and 0 deaths occurred.

Structured PICO

Population

9 patients with Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT) from 7 families, 22% male, mean age at diagnosis 26.4 ± 12 years. Symptoms at admission included syncope (n=4) and aborted cardiac arrest (n=4).

Intervention

Beta-blockers (n=7), with flecainide added if symptoms persisted (n=4), and implantable cardioverter defibrillator (ICD) implantation (n=7; 1 primary, 6 secondary prevention).

Outcome

Adverse cardiac events, ventricular tachycardia, ventricular fibrillation, and death over long-term follow-up.hard clinical

Patients with CPVT remain at high risk for recurrent life-threatening ventricular arrhythmias despite medical therapy and ICD implantation, though mortality can be prevented.

Implication

Small single-center CPVT cohort adds descriptive data; leaves open optimal long-term management pending larger prospective studies.

Abstract

Background: Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare inherited disorder causing life-threatening arrhythmias. Long-term outcome studies of the channelopathy are limited. Objective: The aim of the present study was to summarize our knowledge on CPVT patients, including the clinical profile treatment approach and long-term outcome. Methods: In this single center study, we retrospectively and prospectively collected data from nine CPVT patients and analyzed them. Results: We reviewed nine patients with CPVT in seven families (22% male), with a median follow-up time of 8.6 years. Mean age at diagnosis was 26.4 ± 12 years. Symptoms at admission were syncope (four patients) and aborted cardiac arrest (four patients). Family history of sudden cardiac death was screened in five patients. In genetic analyses, we found five patients with ryanodine type 2 receptor (RYR2) mutations. Seven patients were treated with beta-blockers, and if symptoms persisted flecainide was added (four patients). Despite beta-blocker treatment, three patients suffered from seven adverse cardiac events. An implantable cardioverter defibrillator was implanted in seven patients (one primary, six secondary prevention). Over the follow-up period, three patients suffered from ventricular tachycardia (ten times) and five patients from ventricular fibrillation (nine times). No one died during follow-up. Conclusion: Our CPVT cohort showed a high risk of cardiac events. Family screening, optimal medical therapy and individualized treatment are necessary in affected patients in referral centers.