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

Nina E Hasselberg1, Thor Edvardsen, Helle Petri

  • 1Dept of Cardiology and Center for Cardiological Innovation, Oslo University Hospital, Rikshospitalet, Oslo, Norway.

Europace : European Pacing, Arrhythmias, and Cardiac Electrophysiology : Journal of the Working Groups on Cardiac Pacing, Arrhythmias, and Cardiac Cellular Electrophysiology of the European Society of Cardiology
|September 24, 2013
PubMed

Insights

In Lamin A/C mutation carriers, a prolonged PR-interval predicts ventricular arrhythmias (VA). Septal fibrosis is linked to impaired heart function and VA, suggesting a mechanism for cardiac issues.

Area of Science:

  • Cardiology
  • Genetics
  • Cardiac Electrophysiology

Background:

  • Lamin A/C gene mutations are associated with various cardiac abnormalities, including arrhythmias and cardiomyopathy.
  • Understanding the predictors and mechanisms of ventricular arrhythmias (VA) in these patients is crucial for risk stratification and management.

Purpose of the Study:

  • To identify predictors of ventricular arrhythmias (VA) in individuals with Lamin A/C gene mutations.
  • To elucidate the underlying mechanisms of VA in this patient cohort.

Main Methods:

  • Retrospective analysis of 41 subjects with Lamin A/C mutations.
  • Assessment of PR-interval, occurrence of VA, left ventricular (LV) function (ejection fraction, strain) via echocardiography.
  • Cardiac magnetic resonance imaging (MRI) to evaluate myocardial fibrosis in a subset of patients.

Main Results:

  • Ventricular arrhythmias (VA) were present in 51% of subjects.
  • A prolonged PR-interval was the strongest predictor of VA (P < 0.001).
  • Reduced myocardial function in the interventricular septum correlated with prolonged PR-interval; septal fibrosis was exclusively found in patients with VA and associated with longer PR-intervals.

Conclusions:

  • Prolonged PR-interval is a key predictor of VA in Lamin A/C mutation-positive subjects.
  • Electrical, mechanical, and structural cardiac properties are interconnected in these patients.
  • Localized septal fibrosis appears to be a significant mechanism contributing to impaired septal function, atrioventricular block, and VA.
Abstract

Keywords:
Atrioventricular blockEchocardiographyGenetic heart diseaseLamin A/CMyocardial mechanicsVentricular arrhythmias

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