Quinidine completely suppressed ventricular ectopy and markedly improved left ventricular function in a patient with SCN5A R814W-associated 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.
Absolute Event Rate: 0% vs 0%
ABSTRACT 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.
Bernardo et al. (Tue,) reported a other. Quinidine completely suppressed ventricular ectopy and markedly improved left ventricular function in a patient with SCN5A R814W-associated dilated cardiomyopathy.