ajmaline and Arrhythmogenic-Right-Ventricular-Dysplasia

Topics: Ajmaline; Arrhythmogenic Right Ventricular Dysplasia; Brugada Syndrome; Diagnostic Imaging; Electrocardiography; Female; Humans; Male

Provocable coved-type ST-segment elevation in right precordial leads is an observation in approximately 16% of patients with typical arrhythmogenic right ventricular cardiomyopathy (ARVC). The value of this observation should be analysed in a long-term follow-up of 17 patients identified by systematic ajmaline challenge.. At first evaluation, one female had an aborted sudden cardiac death and eight patients suffered from recurrent syncopes. Intrathoracic cardioverter defibrillator (ICD) implantation was done in the patient with aborted sudden cardiac death and in six patients with recurrent syncopes. One of these six patients had intermittant 2-3 degrees AV block. Another patient had inducible ventricular tachycardia (VT) at electrophysiological study. Follow-up over more than 3 years in all but one patient was characterized by documented monomorphic VT in the patient with inducible VT and ICD implantation (6%). The patient with aborted sudden cardiac death had only non-sustained VT's shortly after ICD implantation. From the eight patients without syncopes two more patients developed AV block and SA block 3 degrees (18%). Lead-associated complications appeared in three of eight patients with ICDs (38%). Repeated ajmaline challenge was positive in four of eight cases (50%). One patient had a new mutation encoding for SCN5A gene.. Ajmaline challenge in typical ARVC characterizes a subgroup of elderly, predominantly female patients with the risk of developing conduction disease. Tachycardia-related events are rare. The indication of ICD implantation in recurrent syncopes is critical as the rate of lead-associated complications in a more than 3 years follow-up is high.

Topics: Adult; Age Factors; Ajmaline; Anti-Arrhythmia Agents; Arrhythmogenic Right Ventricular Dysplasia; Electrocardiography; Electrophysiologic Techniques, Cardiac; Female; Follow-Up Studies; Heart Ventricles; Humans; Longitudinal Studies; Magnetic Resonance Imaging; Male; Middle Aged; Muscle Proteins; Mutation; NAV1.5 Voltage-Gated Sodium Channel; Pacemaker, Artificial; Sex Factors; Sodium Channels; Treatment Outcome

An association between arrhythmogenic right ventricular dysplasia-cardiomyopathy (ARVD/C) and Brugada syndrome can be supposed according to several case reports. In order to examine a possible link between ARVD/C and Brugada syndrome, systematic ajmaline testing with 1 mg/kg body weight intravenously, was done in 55 patients (32 males, mean age 46.7+/-12.3 years) with ISFC/ESC criteria of ARVD/C. In nine patients ajmaline testing could demonstrate coved ST segment elevation of at least 2 mm in at least two right precordial leads. Three of these patients had recurrent syncopes. Electrophysiological study revealed non-sustained ventricular tachycardia with left bundle branch block configuration and inferior axis in only one case. Systematic ajmaline testing could demonstrate a definite link between ARVD/C and Brugada syndrome.

Topics: Adult; Aged; Ajmaline; Anti-Arrhythmia Agents; Arrhythmogenic Right Ventricular Dysplasia; Bundle-Branch Block; Electrocardiography; Female; Germany; Humans; Male; Middle Aged; Prevalence; Syncope; Syndrome; Tachycardia, Ventricular