Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) Arrhythmogenic Right Ventricular Dysplasia (ARVD)
Conditions
Interventions
Ajmaline (class 1c sodium channel blocker, with a short half-life) infusion in
fractions of 10mg every minute up to a target dose of 1mg/kg.
Sponsors
Universitair Medisch Centrum Utrecht
Eligibility
Age
18 Years to 99 Years
Inclusion criteria
Inclusion criteria: Age >=18 years Pathogenic PLN mutation, pathogenic PKP2 mutation or patients without structural heart disease who are referred for ajmaline provocation to exclude Brugada syndrome. Referral for cardiac MRI during routine clinical practice New York Heart Association functional class
Exclusion criteria
Exclusion criteria: Severe hepatic impairment (Child-Pugh class C) Severe renal dysfunction (eGFR <30 ml/min/kg) Symptomatic heart failure, NYHA >= 2 Women who are currently pregnant Known intolerance or contraindication to Ajmaline Sick sinus syndrome, second or third degree AV block Recent myocardial infarction Known strong allergic reaction to ECG electrodes Current use of anti-arrhythmic drugs (Bruagdadrugs.org list class I, IIa, IIb) Current or recent use of amiodarone (within 6 months)
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| The mean difference in local Activation Time Duration before ajmaline administration and at the maximal amount of ajmaline administration in the subtricuspid area, the mid right ventricle area, right ventricular outflow tract, the left ventricle anterior area and left ventricle posterolateral area as calculated by ECG imaging. | — |
Secondary
| Measure | Time frame |
|---|---|
| Mean differences in epicardial and endocardial Activation Time Duration before and at the maximal amount of ajmaline administration in these specified areas (subtricuspid area, the mid RV area, RVOT, the LV anterior and LV posterolateral) as calculated by ECG imaging. 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. Other study parameters: Patient demographics: Sex, Age, Past medical history (including previous episodes of ventricular tachycardia and the results of cardiovascular diagnostic test, for example cardiac holters, cardiac MRI and echocardiography), Allergies, Family history of sudden cardiac death, the amount of 2010 Task Force Criteria points for arrhythmogenic cardiomyopathy (ACM) currently used medication, Weight (kg), Length (cm), Body mass index. Laboratory parameters: Before ajmaline provocation: Creatinine and GFR, Sodium, Potassium, ALAT, ASAT, GGT, AF, bilirubin. Two weeks after ajmaline provocation: ALAT, ASAT, GGT, AF, bilirubin. | — |
Countries
Netherlands
Outcome results
None listed