Short-coupled idiopathic ventricular fibrillation is a rare syndrome and subtype of idiopathic ventricular fibrillation that is characterized by ventricular fibrillation or polymorphic ventricular tachycardia (PVT) initiated by a short-coupled premature ventricular contraction (PVC).
Conditions
Interventions
Trade Name: Quinidine
Pharmaceutical Form: Tablet
INN or Proposed INN: Quinidine
CAS Number: 50-54-4
Other descriptive name: QUINIDINE SULFATE
Trade Name: Verapamil
Pharmaceutical Form: Tablet
INN or
Sponsors
Amsterdam UMC
Eligibility
Sex/Gender
All
Inclusion criteria
Inclusion criteria: 1. At least one of the following 3 principal diagnostic criteria for short-coupled IVF: A. Diagnosis of short-coupled IVF, based on any documentation (i.e., ECG, Holter monitor, device electrogram (EGM), or telemetry) of PVT of =3 consecutive beats or VF initiated by a PVC with a coupling interval =65 years) yes F.1.3.1 Number of subjects for this age range 35
Exclusion criteria
Exclusion criteria: • Pregnancy or lactation • Current treatment with amiodarone • Patients with a history of therapy refractory ventricular arrhythmia on an adequate dose of verapamil or quinidine, as determined by the treating cardiologist. • Contra-indication to quinidine or verapamil (see section 7.6) • Significant structural heart disease (left ventricular ejection fraction 480 ms) heart-rate corrected QT interval on the resting ECG at baseline • Presence of a pathogenic or likely-pathogenic ryanodine receptor 2 (RYR2) mutation • Presence of ischemia-induced short-coupled ventricular arrhythmia in patient with documented coronary spasm • Presence of pause-dependent torsade de pointes [preceding R–R interval prior to the trigger PVC >1500 ms in individuals without pacemaker/ICD or >1300 ms in individuals with pacemaker/ICD] following a stable baseline rhythm. Initiation of ventricular arrhythmia by short-long-short cycles (R–R cycles <1300 ms) with a short-coupled trigger PVC is allowed • Significant coronary artery disease (=50% narrowing of the diameter of the lumen of the left main coronary artery or =70% narrowing of the diameter of the lumen of the left anterior descending coronary artery, left circumflex artery or right coronary artery) • Reversible metabolic or pharmacological/toxicological conditions that may cause electrophysiological findings similar to short-coupled IVF • Patients who are considered electrically unstable, at physician’s discretion, due to active electrical storm or very frequent nonsustained episodes of short-coupled IVF requiring intravenous or invasive therapy • Successful radiofrequency ablation of the PVC initiating short-coupled IVF and absence of documented (non)sustained episodes of short-coupled PVT/VF afterwards. The patient will, however, be eligible to participate in the study if = 1 episode of short-coupled PVT/VF is documented after the ablation procedure • Intention to perform radiofrequency ablation of the PVC initiating short-coupled IVF during the course of the study • Serious known comorbid disease with a life expectancy of less than two years • Ongoing medical condition that is deemed by the principal investigator to interfere with the conduct or assessments of the study or safety of the subjects • Circumstances that prevent follow-up • Inability to take orally administered tablets • Inability to provide informed consent
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Main Objective: The objective of this study is to determine the advisability and feasibility of a definitive RCT that will assess quinidine as compared with verapamil in patients with short-coupled IVF in terms of the safety and efficacy with regard to arrhythmic events. ;Secondary Objective: Not applicable;Primary end point(s): The primary endpoint is: •Sustained ventricular arrhythmia, assessed using the severity scoring system. A subject will be scored in each treatment period according to the scoring system by the ECC. The highest applicable score will be used. Ventricular arrhythmia scoring system: 0= No arrhythmic events 1= A single arrhythmic event 2= Electrical storm (=3 episodes of sustained PVT/VF within 24 hours) 3= =2, but <5 arrhythmic events 4= =5 arrhythmic events ;Timepoint(s) of evaluation of this end point: At each moment of follow-up | — |
Secondary
| Measure | Time frame |
|---|---|
| Secondary end point(s): The secondary endpoints are: • Time to first arrhythmic event • Incidence of quinidine-induced torsade de pointes • Incidence of sustained monomorphic ventricular tachycardia • Inappropriate ICD shocks ;Timepoint(s) of evaluation of this end point: At each moment of follow-up | — |
Countries
Netherlands
Contacts
Public ContactDepartment of cardiology
Amsterdam UMC
Outcome results
None listed