Cardiac arrhythmias in patients with Danon disease.

Aims: Different cardiac arrhythmias have been suggested to be associated with Danon disease, e.g. Wolff-Parkinson-White syndrome. However, a systematic electrophysiological investigation of patients with Danon disease is lacking thus far.

Methods and results: Seven patients with Danon disease (4 males, 35.8 ± 10.8 years; 3 females, 51.3 ± 19.9 years) from 3 different families were studied. In all patients, the presence of Danon disease was confirmed by western blot of biopsy material or genetic testing. The patients were characterized by 12-lead electrocardiogram (ECG), Holter ECG, echocardiography, and serial implantable cardioverter defibrillator (ICD) interrogations (in ICD recipients). All male patients underwent electrophysiological investigation (EP study). Asymptomatic ventricular tachyarrhythmias were documented in six of the seven patients. Moreover, 5 of the 7 patients suffered from atrial fibrillation (AF), with 1 of them experiencing thromboembolic stroke at the age of 30 years. In male patients, the initial QRS complex was characterized by a slurring upstroke and shortened PQ interval mimicking ventricular pre-excitation. One male patient showed initial QRS complex slurring with prolonged PR interval. However, the presence of an accessory pathway was excluded by an EP study in all patients. In female patients, initial QRS complex slurring was significantly less distinct. In four patients, ICD implantation was performed for primary prevention of sudden cardiac death. However, sustained ventricular arrhythmias were not documented in any of the patients.

Conclusions: The present study indicates that the distinct surface ECG pattern in Danon disease is not associated with ventricular pre-excitation. Atrial fibrillation is frequently observed in these patients and may be associated with thromboembolic events in the young, while sustained ventricular arrhythmias occur less frequently than previously reported.