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

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.

Device-Related Complications and Inappropriate Therapies Among Subcutaneous vs. Transvenous Implantable Defibrillator Recipients: Insight Monaldi Rhythm Registry

“…The subcutaneous implantable cardioverter-defibrillator (S-ICD) is an established therapy for the prevention of sudden cardiac death (SCD) ( 1 ) and an alternative to a transvenous implantable cardioverter-defibrillator (T-ICD) system in selected patients ( 2 ). S-ICD may be particularly useful in patients with channelopathies ( 3 ) since several studies showed a high complication rate in those implanted with T-ICD ( 4 – 6 ). S-ICD is non-inferior to T-ICD concerning device-related complications or inappropriate shocks in patients with an indication for defibrillator therapy and not in need of pacing ( 7 – 12 ); however, these data are limited to short follow-up observational case-control studies ( 7 – 10 ) or the context of the randomized clinical trial ( 11 , 12 ).…”

Management of Catecholaminergic Polymorphic Ventricular Tachycardia

“…Так, A. Mazzanti и соавт., изучив результаты лечения 216 больных с КПЖТ, показали, что в течение (в среднем) 9 лет жизнеугрожающие события развились у 13% больных, получающих лечение только бета-адреноблокаторами [14]. В другом исследовании на примере 9 пациентов с КПЖТ со средним периодом наблюдения 8,6 года было установлено, что на фоне базовой терапии бета-адреноблокаторами аритмогенные события возникли у трех пациентов [15]. Недостаточная эффективность бета-адреноблокаторов при КПЖТ отмечена также и в работе S.G Priori и соавт., согласно результатам которой имплантация кардиовертера-дефибриллятора (ИКД) потребовалась 30% таких больных [16].…”

Management of hemodynamically stable wide QRS complex tachycardia in patients with implantable cardioverter defibrillators

“…Betablockers, sedation as well as flecainide have been The different steps of the first phase are summarized. implemented for patients with catecholaminergic polymorphic ventricular tachycardia (38)(39)(40).…”