The International CPVT Registry
Research type
Research Study
Full title
The International CPVT Registry
IRAS ID
277642
Contact name
Luke Starling
Contact email
Sponsor organisation
Amsterdam University Medical Centre
Duration of Study in the UK
10 years, 0 months, 1 days
Research summary
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare genetic condition characterised by abnormal, potentially life-threatening heart rhythms. The condition is caused by genetic mutations which affect the way that calcium is used within the heart muscle cells. In CPVT, abnormal heart rhythms are triggered by physical exertion or by emotional stressors, such as excitement or fear. Affected individuals can experience symptoms of dizziness, palpitations or fainting episodes. Where it remains undetected and untreated, CPVT can cause sudden death. The condition is thought to affect around 1 in 10,000 people, with affected patients typically presenting in childhood with exercise- or emotion-induced fainting episodes or cardiac arrest. Diagnosis can be confirmed by exercise testing with ECG monitoring or by genetic testing.
Because CPVT is a very rare condition, clinical data have been historically limited and risk stratification remains ill-defined. The current consensus is to offer treatment with beta-blocker medication to all clinically or genetically diagnosed patients. In recent years, significant advances have been made in developing new treatment alternatives for CPVT, including left cardiac sympathetic denervation and flecainide drug therapy.
This international registry of CPVT patients has been established to enable detailed investigation of clinical and genetic aspects of CPVT over the long term.
The first specific aims of the registry are:
- to evaluate the efficacy of different types of beta blockers
- to understand the risk of cardiac events in concealed mutation carriers
- to establish the age of onset of arrhythmias and symptoms in the pediatric population
The data collected by this registry will enable better understanding of disease mechanisms and outcomes in CPVT, and will help to establish the most effective ways of treating the condition in order to reduce risk and symptoms in affected patients.REC name
London - West London & GTAC Research Ethics Committee
REC reference
21/PR/0737
Date of REC Opinion
7 Jun 2021
REC opinion
Favourable Opinion