Risk prediction of ventricular arrhythmias and myocardial function in Lamin A/C mutation positive subjects
Copyright policy )Risk prediction of ventricular arrhythmias and myocardial function in Lamin A/C mutation positive subjects
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.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 < 0.001). Myocardial function by strain was reduced in the interventricular septum compared with the rest of the LV segments (-16.7% vs. -18.7%, P = 0.001) and correlated to PR-interval (R = 0.41, P = 0.03). Myocardial fibrosis was found exclusively in the interventricular septum and only in patients with VA (P = 0.007). PR-interval was longer in patients with septal fibrosis compared with those without (320 ± 66 vs. 177 ± 40 ms, P < 0.001).Prolonged PR-interval was the best predictor of VA in Lamin A/C mutation-positive subjects. Electrical, mechanical, and structural cardiac properties were related in these subjects. Myocardial function was most reduced in the interventricular septum and correlated to prolonged PR-interval. Myocardial septal fibrosis was associated with prolonged PR-interval and VA. Localized fibrosis in the interventricular septum may be the mechanism behind reduced septal function, atrioventricular block and VA in Lamin A/C mutation-positive subjects.
- University of Oslo Norway
- Oslo University Hospital Norway
- Rigshospitalet Denmark
Adult, Cardiomyopathy, Dilated, Male, Adolescent, Denmark, DNA Mutational Analysis, Arrhythmias, Cardiac, Middle Aged, Lamin Type A, Fibrosis, Magnetic Resonance Imaging, Echocardiography, Doppler, Electrocardiography, Mutation, Humans, Female, Genetic Predisposition to Disease, Atrioventricular Block, Child, Aged
Adult, Cardiomyopathy, Dilated, Male, Adolescent, Denmark, DNA Mutational Analysis, Arrhythmias, Cardiac, Middle Aged, Lamin Type A, Fibrosis, Magnetic Resonance Imaging, Echocardiography, Doppler, Electrocardiography, Mutation, Humans, Female, Genetic Predisposition to Disease, Atrioventricular Block, Child, Aged
selected citations These citations are derived from selected sources.This is an alternative to the "Influence" indicator, which also reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).89 popularity This indicator reflects the "current" impact/attention (the "hype") of an article in the research community at large, based on the underlying citation network.Top 1% influence This indicator reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).Top 10% impulse This indicator reflects the initial momentum of an article directly after its publication, based on the underlying citation network.Top 10%
Citations provided by BIP!Popularity provided by BIP!