Inflammation on Endomyocardial Biopsy Predicts Risk of MACE in Undefined Left Ventricular Arrhythmogenic

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Isolation and Characterization of Cardiac Mesenchymal Stromal Cells from Endomyocardial Bioptic Samples of Arrhythmogenic Cardiomyopathy Patients
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Isolation and Characterization of Cardiac Mesenchymal Stromal Cells from Endomyocardial Bioptic Samples of Arrhythmogenic Cardiomyopathy Patients

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Giovanni Peretto1, Michela Casella2, Marco Merlo3

  • 1Department of Cardiac Electrophysiology and Arrhythmology, IRCCS San Raffaele Scientific Institute, Milan, Italy; School of Medicine, Vita-Salute San Raffaele University, Milan, Italy.

JACC. Clinical Electrophysiology
|February 8, 2023
PubMed

Insights

Major adverse cardiovascular events (MACE) in undefined left ventricular arrhythmogenic cardiomyopathy (ULVACM) are predicted by malignant ventricular arrhythmia (VA) and active myocardial inflammation (AM). Immunosuppressive therapy may reduce MACE risk in these patients.

Area of Science:

  • Cardiology
  • Genetics
  • Pathology

Background:

  • Predictors of major adverse cardiovascular events (MACE) in patients with undefined left ventricular arrhythmogenic cardiomyopathy (ULVACM) remain undescribed.
  • ULVACM is characterized by ventricular arrhythmia, specific late gadolinium enhancement patterns, and preserved ejection fraction.

Purpose of the Study:

  • To investigate the prognostic value of genetic testing and histology for MACE in ULVACM patients.
  • To identify clinical predictors of adverse cardiovascular outcomes in ULVACM.

Main Methods:

  • Retrospective analysis of 313 ULVACM patients from a multicenter registry.
  • Comparison of outcomes for patients undergoing next-generation sequencing (NGS) and endomyocardial biopsy (EMB) versus those who did not.
  • Primary endpoint: MACE (cardiac death, heart transplantation, malignant VA) at 60 months.

Main Results:

  • Genetic variants identified in 16% of patients undergoing NGS.
  • Active myocardial inflammation (AM) found in 52% of patients undergoing EMB.
  • Malignant VA at presentation and EMB-proven AM were associated with increased MACE risk (HR 2.8 and 3.9, respectively).
  • Immunosuppressive therapy was associated with a reduced risk of MACE (HR 0.10).

Conclusions:

  • Presentation with malignant ventricular arrhythmia (VA) or active myocardial inflammation (AM) are significant predictors of MACE in ULVACM.
  • Immunosuppressive therapy may offer a protective effect against MACE in ULVACM patients with active inflammation.
Abstract

Keywords:
arrhythmogenic cardiomyopathyendomyocardial biopsyinflammationleft ventricular nonischemic scarundefinedventricular arrhythmias

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