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Successful Use of Ranolazine in SCN5A p.R222Q-Related Multifocal Ectopic Purkinje-Related Premature Contractions
Isabella Moreira Gonzalez Fonseca1, Anna Terra França1, André Assis Lopes do Carmo1
1Hospital das Clínicas, Universidade Federal de Minas Gerais, Belo Horizonte, Brazil.
Introduction:
Gain-of-function variants in SCN5A may cause multifocal ectopic Purkinje-related premature contractions (MEPPC), with a high premature ventricular contraction burden that may lead to PVC-induced cardiomyopathy. Pharmacological therapy is usually required, as catheter ablation is ineffective because of the multifocal origin of the ectopy.
Methods And Results:
We report two siblings with the pathogenic SCN5A p.Arg222Gln variant. In both, ranolazine markedly suppressed ventricular ectopy, improved or preserved left ventricular function, and enabled amiodarone withdrawal.
Conclusion:
Ranolazine may represent a useful mechanism-guided option for SCN5A-related MEPPC.
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