Arrhythmogenic right ventricular dysplasia-cardiomyopathy and provocable coved-type ST-segment elevation in right precordial leads: clues from long-term follow-up.
OBJECTIVE
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.


RESULTS
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.


CONCLUSIONS
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.
Peters
Stefan
S
Klinikum Quedlinburg, Department of Cardiology, Academic Teaching Hospital of the University Hospital Magdeburg, Ditfurter Weg 24, 06484 Quedlinburg, Germany. s.peters@klinikum-quedlinburg.de
D000328:Adult; D000367:Age Factors; D000404:Ajmaline / Q000494:pharmacology; D000889:Anti-Arrhythmia Agents / Q000494:pharmacology; D019571:Arrhythmogenic Right Ventricular Dysplasia / Q000378:metabolism / Q000503:physiopathology* / Q000628:therapy*; D004562:Electrocardiography; D022062:Electrophysiologic Techniques, Cardiac; D005260:Female; D005500:Follow-Up Studies; D006352:Heart Ventricles / Q000187:drug effects / Q000503:physiopathology; D006801:Humans; D008137:Longitudinal Studies; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009124:Muscle Proteins / Q000235:genetics / Q000378:metabolism; D009154:Mutation / Q000235:genetics; D062554:NAV1.5 Voltage-Gated Sodium Channel; D010138:Pacemaker, Artificial; D012737:Sex Factors; D015222:Sodium Channels / Q000235:genetics / Q000378:metabolism; D016896:Treatment Outcome
D000889:Anti-Arrhythmia Agents; D009124:Muscle Proteins; D062554:NAV1.5 Voltage-Gated Sodium Channel; C568320:SCN5A protein, human; D015222:Sodium Channels; D000404:Ajmaline