Author + information
- Fernando Wangüemert Pérez,
- Pablo Ruiz Hernández,
- Paola Berne,
- Oscar Campuzano,
- Brugada Ramón and
- Josep Brugada
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an arrhythmogenic familiar disease characterized by high incidence of sudden cardiac death (SCD) in young people with normal basal electrocardiogram and structurally normal heart. Treatment is based mainly in the use of beta-blockers, although there is controversy on its long-term effectiveness, needs for titration and follow-up. We discovered a family with more than 1400 members with high incidence of SCD in Gran Canaria (Spain). After a genetic screening, 179 alive carriers of a RyR2 mutation (G357S) were identified.
A clinical protocol of treatment and follow-up was offered to all family members, based in exercise stress tests (ET) every 3 months, with an objective of maximum heart rate less than theoretical 80% and the absence of ventricular arrhythmias, increasing or changing betablockers to achieve it. If the carrier constantly failed to achieve the goal, an Implantable cardioverter defibrillator (ICD) was proposed.
After more than 4 years of application of the Protocol no SCD or cardiac arrests have occurred into this group. However, 3 events occurred between those that refused to be genotyped -53 potential carriers- (Figure)./
The Protocol based on seriated ET to titrate Betablockers is useful and effective in management of CPVT, and should be recommended to carriers of a CPVT-related mutation and, probably, to all CPVT patients.
Poster Sessions, Expo North
Sunday, March 10, 2013, 9:45 a.m.-10:30 a.m.
Session Title: Arrhythmias: Sudden Cardiac Death – Mechanisms and Treatment Strategies
Abstract Category: 6. Arrhythmias: Other
Presentation Number: 1192-54
- 2013 American College of Cardiology Foundation