Premium
Arrhythmias and fasciculoventricular pathways in patients with Danon disease: A single center experience
Author(s) -
Jhaveri Simone,
Herber Joshua,
Zahka Kenneth,
Boyle Gerard J.,
Saarel Elizabeth V.,
Aziz Peter F.
Publication year - 2019
Publication title -
journal of cardiovascular electrophysiology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.193
H-Index - 138
eISSN - 1540-8167
pISSN - 1045-3873
DOI - 10.1111/jce.14049
Subject(s) - medicine , cardiology , implantable cardioverter defibrillator , single center , population , hypertrophic cardiomyopathy , heart disease , atrial fibrillation , cardiomyopathy , disease , heart failure , environmental health
Background Danon disease is a rare X‐linked storage disorder characterized by hypertrophic cardiomyopathy leading to arrhythmias and heart failure. A preexcitation pattern on electrocardiogram (ECG) has been described in these patients, however, invasive studies to distinguish between Wolff‐Parkinson‐White syndrome syndrome and fasciculoventricular pathways (FVP) are limited. Objectives The purpose of this study was to delineate the electrophysiological cardiac abnormalities in patients with Danon disease and to describe the presence of FVP in this population. Methods We performed a retrospective study of all patients with a confirmed diagnosis of Danon disease presenting to a single center from May 2005 to May 2018. Baseline demographics, clinical characteristics, ECG findings, and electrophysiology study (EPS) results were collected. Results Ten patients with Danon disease (30% male, average age 17.4 years) were identified. Seven patients (70%) had tachyarrhythmias including five with atrial arrhythmias and six with nonsustained ventricular tachycardia. Preexcitation pattern on ECG was found in four (40%) patients. Of these, two underwent an EPS which confirmed the presence of an FVP. One patient underwent an adenosine challenge which supported a FVP. Implantable cardioverter defibrillator was placed in five patients for primary prevention with no patients receiving an appropriate discharge. Over a follow‐up of 5.3 years, five underwent heart transplantation. Conclusions This study reports a high incidence of FVP in patients with Danon disease and preexcitation. It underscores an alternate etiology of preexcitation in this population which can potentially be diagnosed without invasive EPS testing. Future multicenter studies are needed to expand this experience.