Arrhythmogenic cardiomyopathy, ARVC, carriers of pathogenic PLN and PKP2 mutations
Conditions
Interventions
Ajmaline provocation
Sponsors
UMC Utrecht
Eligibility
Inclusion criteria
Inclusion criteria: Patients or asymptomatic carriers of pathogenic PLN mutation, pathogenic PKP2 mutation or patients without structural heart disease who are referred for ajmaline provocation to exclude Brugada syndrome. New York Heart Association functional class ¡Ü 1.
Exclusion criteria
Exclusion criteria: Severe hepatic impairment (Child-Pugh class C) Severe renal dysfunction (eGFR <30 ml/min/kg) Symptomatic heart failure, NYHA ¡Ý 2 Women who are currently pregnant Known intolerance or contraindication to Ajmaline Sick sinus syndrome, second or third degree AV block without pacemaker implantation Recent myocardial infarction Known strong allergic reaction to ECG electrodes
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Mean changes in Activation Time Duration before and during ajmaline administration between the subtricuspid area and LV/RV/RVOT as calculated by ECG imaging. | — |
Secondary
| Measure | Time frame |
|---|---|
| Ventricular tachycardia, sudden cardiac death, ventricular fibrillation or development of arrhythmogenic cardiomyopathy (fulfilment of the Task Force Criteria) during follow up. Electrocardiographic parameters (PQ, QRS and QTc intervals, the occurrence of the type I Brugada pattern, terminal activation duration) before, during and directly after ajmaline provocation. Adverse events of ajmaline provocation | — |
Contacts
Public ContactR.W. Roudijk
Afdeling: Cardiologie, Huispost E03.511
Outcome results
None listed