1. Fasciculoventricular and atrioventricular accessory pathways in patients with Danon disease and preexcitation: A multicenter experience
- Author
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Johannes C. von Alvensleben, Wendy S. Tzou, Gregory K. Feld, Michela Brambatti, William H. Sauer, Jonathan C. Hsu, Eric Adler, Kurt S. Hoffmayer, Mary Brooks, and Douglas Darden
- Subjects
Adult ,Male ,medicine.medical_specialty ,Bundle of His ,Pre-Excitation Syndromes ,Time Factors ,Adolescent ,medicine.medical_treatment ,DNA Mutational Analysis ,Accessory pathway ,030204 cardiovascular system & hematology ,Ventricular tachycardia ,Sudden cardiac death ,03 medical and health sciences ,QRS complex ,Electrocardiography ,Young Adult ,0302 clinical medicine ,Interquartile range ,Physiology (medical) ,Internal medicine ,Lysosomal-Associated Membrane Protein 2 ,medicine ,Prevalence ,Humans ,Danon disease ,030212 general & internal medicine ,Registries ,Child ,Retrospective Studies ,Heart transplantation ,business.industry ,Incidence ,Hypertrophic cardiomyopathy ,DNA ,medicine.disease ,Glycogen Storage Disease Type IIb ,United States ,Accessory Atrioventricular Bundle ,Mutation ,Cardiology ,Female ,Cardiology and Cardiovascular Medicine ,business ,Follow-Up Studies - Abstract
Background Studies have suggested that a fasciculoventricular pathway (FVP) may be the cause of preexcitation in patients with Danon disease, a rare X-linked dominant genetic disorder of hypertrophic cardiomyopathy. Objective The purpose of this study was to describe the prevalence of ventricular preexcitation on resting 12-lead electrocardiogram (ECG) in patients with Danon disease and the electrophysiological study (EPS) results of those with preexcitation. Methods Patients with confirmed Danon disease diagnosed with preexcitation (PR ≤120 ms, delta wave, QRS >110 ms) on ECG were included from a multicenter registry. The incidence of arrhythmias, implantable cardioverter-defibrillator (ICD) procedures, ICD shocks, and EPS results were collected. Results Thirteen of 40 patients (32.5%) with Danon disease were found to have preexcitation (mean age 17.3 years; 38% women). EPS performed in 9 of 13 patients (69%) demonstrated FVP only in 2 (22.2%), extranodal pathway without exclusion of FVP in 2 (22.2%), and both FVP and extranodal pathway in 5 (55.6%). Two patients had malignant accessory pathway (AP) properties. Over median follow-up of 842 days (interquartile range 138–1678), 11 patients (85%) had ICD placement, and 6 (46.1%) underwent heart transplantation. No patients required therapy for ventricular tachycardia, and 2 patients (15%) had paroxysmal atrial fibrillation. Conclusion In a large multicenter cohort of patients with Danon disease, there was a high prevalence of FVP and extranodal pathways diagnosed on EPS in those with preexcitation. These findings suggest patients with preexcitation and Danon disease should undergo EPS to assess for FVP and potentially malignant extranodal AP.
- Published
- 2020