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.

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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
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article

Resolution of severe dilated cardiomyopathy with significant arrhythmia burden using hydroquinidine, in addition to guideline-directed medical therapy, in a patient with a pathogenic SCN5A variant: a case report

R I Griffiths, Elijah R Behr, Catherine Mercer, Peter J Cowburn
European Heart Journal - Case Reports
Cardiac electrophysiology and arrhythmias
article

Resolution of severe dilated cardiomyopathy with significant arrhythmia burden using hydroquinidine, in addition to guideline-directed medical therapy, in a patient with a pathogenic SCN5A variant: a case report

R I Griffiths, Elijah R Behr, Catherine Mercer, Peter J Cowburn
article en

Abstract

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.

European Heart Journal - Case ReportsVol. 10(3)
St George's, University of London (GB), Southampton General Hospital (GB)
Openalex Percentile: Top 67%
Cardiac electrophysiology and arrhythmias
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