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Layer-Specific Global Longitudinal Strain Predicts Arrhythmic Risk in Arrhythmogenic Cardiomyopathy

Diego Segura-Rodríguez1,2, Francisco José Bermúdez-Jiménez2,3,4, Lorena González-Camacho3

  • 1Cardiology Department, Hospital Universitario San Cecilio, Granada, Spain.

Frontiers in Cardiovascular Medicine
|December 6, 2021
PubMed

Insights

Layer-specific global longitudinal strain (GLS) using echocardiography can identify patients with arrhythmogenic cardiomyopathy (AC) at high risk for major ventricular arrhythmias. Epicardial GLS may help detect individuals at risk of sudden cardiac death (SCD).

Area of Science:

  • Cardiology
  • Medical Imaging
  • Genetics

Background:

  • Arrhythmogenic cardiomyopathy (AC) is a serious inherited heart condition leading to malignant arrhythmias and sudden cardiac death (SCD).
  • Current risk stratification for AC involves electrical, genetic, and imaging data.
  • Identifying early indicators of arrhythmic risk is crucial for patient management.

Purpose of the Study:

  • To evaluate if myocardial deformation parameters, specifically layer-specific global longitudinal strain (GLS), can identify patients with AC at risk of major ventricular arrhythmias.
  • To correlate GLS with established risk markers like non-sustained ventricular tachycardia (NSVT), late gadolinium enhancement (LGE), and genetic status.

Main Methods:

  • A cohort of 45 patients with AC underwent advanced transthoracic echocardiography (TTE) and cardiac magnetic resonance (CMR).
  • Layer-specific GLS was assessed using TTE 2D speckle tracking.
  • Patients were grouped based on the presence of NSVT, LGE, and genetic mutations.

Main Results:

  • Next-generation sequencing revealed pathogenic mutations in 86.7% of patients.
  • LGE was present in 73.3% of patients, predominantly in subepicardial-to-mesocardial layers.
  • Worse epicardial and mesocardial GLS values were observed in patients with NSVT and LGE. Epicardial GLS cut-offs of -15.4% and -16.1% effectively identified individuals with NSVT and LGE, respectively, independent of LVEF.

Conclusions:

  • Layer-specific GLS assessment is effective in identifying high-risk arrhythmic features in AC patients, including NSVT and LGE.
  • Epicardial GLS shows promise as a tool for detecting individuals at risk of SCD in AC.
  • Advanced echocardiographic strain analysis offers valuable insights into AC pathophysiology and risk stratification.

Keywords:
arrhythmogenic cardiomyopathy (ACM)global longitudinal strainlate gadolinium enhancednon-sustained ventricular tachycardiasudden cardiac death (SCD)

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