Abstract 15259: The Prognosis of Catecholaminergic Polymorphic Ventricular Tachycardia

Introduction: Catecholaminergic polymorphic ventricular tachycardia (CPVT) is reported to have one of the worst prognoses among inherited arrhythmias. Methods: This study aimed to investigate the contributing factors affecting the prognosis of CPVT by sending questionnaires to Japanese cardiovascular centers. Results: This study enrolled 88 patients with CPVT, 31 male (35%), and 57 female (65%), the age of onset was 11.3±7.9 years old, and followed up in 20.1±11.1 years. Nine of the patients died suddenly during follow up. Six of 31 (19%) male patients, and 3 of 57 (5%) female patients died suddenly during the follow up period. Sudden death was statistically more common in male patients than females (P=0.0424). Genetic analysis was performed in 41 patients. Ryanodine receptor (RyR2) gene anomaly was detected in 34 patients, and 1 patient died suddenly. RyR2 gene anomaly was not found in 5 patients, and calsequestrin gene was found in 2 patients but no patients were died suddenly in this group. Genetic ana...