Resolution of severe dilated cardiomyopathy with significant arrhythmia burden using hydroquinidine, in addition to guideline-directed medical therapy, in a patient with a pathogenic <i>SCN5A</i> variant: a case report
This atypical presentation of a cardiomyopathy was driven, at least in part, by the patient's extensive arrhythmia. Previous research has shown variable, short-term effects of sodium channel antagonists in familial p.R814W variants. Contrastingly, in our patient, sustained and long-term improvement was observed with the use of hydroquinidine. Close multidisciplinary team working and early genetic testing facilitated personalized care in our patient's case, resulting in a favourable outcome.
Authors
- R I Griffiths
- Elijah R Behr
- Catherine Mercer
- Peter J Cowburn
Institutions
- St George's, University of London (GB)
- Southampton General Hospital (GB)
Publication Details
- Journal
- European Heart Journal - Case Reports
- Published
- 2026-02-03
- DOI
- https://doi.org/10.1093/ehjcr/ytag100
- Primary Topic
- Cardiac electrophysiology and arrhythmias
- Type
- article
- Field-Weighted Citation Impact
- 0.00