Risk prediction of ventricular arrhythmias and myocardial function in Lamin A/C mutation positive subjects

Nina E Hasselberg, Thor Edvardsen, Helle Petri, Knut E Berge, Trond P Leren, Henning Bundgaard, Kristina H Haugaa

    87 Citationer (Scopus)

    Abstract

    Mutations in the Lamin A/C gene may cause atrioventricular block, supraventricular arrhythmias, ventricular arrhythmias (VA), and dilated cardiomyopathy. We aimed to explore the predictors and the mechanisms of VA in Lamin A/C mutation-positive subjects.METHODS AND RESULTS: We included 41 Lamin A/C mutation-positive subjects. PR-interval and occurrence of VA were recorded. Left ventricular (LV) myocardial function was assessed as ejection fraction and speckle tracking longitudinal strain by echocardiography. Magnetic resonance imaging was performed to assess fibrosis in a selection of subjects. Ventricular arrhythmias were documented in 21 patients (51%). Prolonged PR-interval was the best predictor of VA (P

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