Key result
Quinidine completely suppresses ventricular ectopy and improves LV function in a patient with SCN5A R814W-associated DCM.
Why the study?
Does quinidine improve ventricular ectopy and left ventricular function in a patient with SCN5A R814W-associated multifocal ectopic Purkinje-related premature contractions and dilated cardiomyopathy?
Does quinidine improve ventricular ectopy and left ventricular function in a patient with SCN5A R814W-associated multifocal ectopic Purkinje-related premature contractions and dilated cardiomyopathy?
Quinidine is an effective treatment for multifocal ectopic Purkinje-related premature contractions (MEPPC) and associated dilated cardiomyopathy caused by the SCN5A R814W variant.
Supports quinidine in SCN5A R814W cardiomyopathy; hypothesis-generating and requires prospective validation before practice change.
Introduction Multifocal ectopic Purkinje‐related premature contractions (MEPPC) is a syndrome caused by gain‐of‐function SCN5A variants, characterized by multifocal ventricular ectopy and dilated cardiomyopathy (DCM). Methods and Results We report a case of a 63‐year‐old woman with longstanding DCM and a high burden of multifocal premature ventricular contractions arising from the His–Purkinje system, refractory to multiple antiarrhythmic drugs and associated with progressive heart failure. Genetic testing identified the pathogenic SCN5A R814W variant. Quinidine resulted in complete suppression of ventricular ectopy, marked improvement in left ventricular function, and clinical recovery. Conclusion This case supports the role of a rare SCN5A variant in MEPPC and the efficacy of quinidine.
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Bernardo et al. (2026) studied this question. Quinidine completely suppressed ventricular ectopy and markedly improved left ventricular function in a patient with SCN5A R814W-associated dilated cardiomyopathy.
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