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A Frequent Observation of Wolff-Parkinson-White Syndrome and Fasciculoventricular Pathways in Patients With Danon Disease.
- Source :
-
Circulation journal : official journal of the Japanese Circulation Society [Circ J] 2022 Jan 25; Vol. 86 (2), pp. 309-318. Date of Electronic Publication: 2021 Dec 23. - Publication Year :
- 2022
-
Abstract
- Background: Danon disease is typically associated with cardiomyopathy and ventricular pre-excitation. The study aimed to characterize the clinical profile of Danon disease, analyze electrocardiographic (ECG) and electrophysiologic features, and investigate their association with Wolff-Parkinson-White (WPW) syndrome and fasciculoventricular pathways (FVPs).<br />Methods and results: Clinical course, family history, ECG and electrophysiological data were collected from 16 patients with Danon disease. Over 0.4-8 years of follow up, 1 female patient died suddenly, and 5 male patients died of progressive heart failure by age 13-20 years. Family history analysis revealed that 3 mothers experienced hospitalization or death for heart failure at age 28-41 years. There was 100% penetrance for ECG abnormalities in 13 patients with original ECGs. Short PR intervals and delta waves were present in 9 and 8 patients, respectively. There were significant age-associated increases in the QRS complex width (r=0.556, P=0.048) and the number of leads with notched QRS (r=0.575, P=0.04). Four patients who underwent electrophysiological studies all had FVPs, and 2 of them still had left-side atrioventricular pathways.<br />Conclusions: Danon disease causes a malignant clinical course characterized by early death caused by heart failure in both genders and progressive ECG changes as patients age. The pre-excited ECG pattern is related to FVPs and WPW, which is suggestive of extensive cardiac involvement.
Details
- Language :
- English
- ISSN :
- 1347-4820
- Volume :
- 86
- Issue :
- 2
- Database :
- MEDLINE
- Journal :
- Circulation journal : official journal of the Japanese Circulation Society
- Publication Type :
- Academic Journal
- Accession number :
- 34937809
- Full Text :
- https://doi.org/10.1253/circj.CJ-21-0572