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Optimal echocardiographic assessment of myocardial dysfunction for arrhythmic risk stratification in phospholamban

Karim Taha1,2, Tom E Verstraelen3, Remco de Brouwer4

  • 1Department of Cardiology, University Medical Center Utrecht, Utrecht, TheNetherlands.

European Heart Journal. Cardiovascular Imaging
|September 13, 2021
PubMed

Insights

Left ventricular mechanical dispersion (LVMD) improves risk prediction for ventricular arrhythmias (VAs) in phospholamban (PLN) p.Arg14del mutation carriers. The

Area of Science:

  • Cardiology
  • Genetics
  • Echocardiography

Background:

  • Phospholamban (PLN) p.Arg14del mutation carriers face high risks of malignant ventricular arrhythmias (VAs) and heart failure.
  • Left ventricular ejection fraction (LVEF) is currently a key factor in assessing VA risk in these patients.

Purpose of the Study:

  • To evaluate the added prognostic value of left ventricular mechanical dispersion (LVMD) using echocardiographic deformation imaging for predicting sustained VA in PLN p.Arg14del mutation carriers.

Main Methods:

  • 243 PLN p.Arg14del mutation carriers were stratified into three groups based on LVEF and LVMD using the '45/45' rule.
  • Follow-up median of 3.3 years to monitor for sustained VA.
  • Multivariable analysis was employed to identify independent predictors of VA.

Main Results:

  • Sustained VA occurred in 35 individuals.
  • A negative predictive value of 99% for normal LV function (LVEF ≥45% and LVMD ≤45 ms).
  • Mechanical LV dysfunction (LVEF ≥45% and LVMD >45 ms) was an independent predictor of sustained VA (aHR: 20.48).

Conclusions:

  • LVMD provides incremental prognostic information beyond LVEF in PLN p.Arg14del mutation carriers, especially those with preserved LVEF.
  • The '45/45' rule offers a practical echocardiographic risk stratification method for this patient group.
  • This approach may benefit risk assessment in other conditions where LVEF decline is a late indicator of myocardial dysfunction.
Abstract

Keywords:
deformation imaginggenetic cardiomyopathymechanical dispersionphospholambanrisk stratificationventricular arrhythmia

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