Clinical Course and Treatment of Patients With Apical Aneurysms Due to Hypertrophic Cardiomyopathy

Mark V Sherrid1,2, Daniele Massera1,2, Samuel Bernard2

  • 1Hypertrophic Cardiomyopathy Program; Leon Charney Division of Cardiology, Department of Medicine, NYU Grossman School of Medicine, New York City, New York, USA.

JACC. Advances
|September 16, 2024
PubMed

Insights

Hypertrophic cardiomyopathy (HCM) patients with apical aneurysms face risks of malignant arrhythmias and stroke. Aneurysm size and risk factors significantly predict these events, with smaller aneurysms and no risk factors indicating lower risk.

Area of Science:

  • Cardiology
  • Cardiovascular Imaging
  • Electrophysiology

Background:

  • Controversy exists regarding the risk of malignant arrhythmias and stroke in patients with apical aneurysms in hypertrophic cardiomyopathy (HCM).
  • Apical aneurysms are a recognized complication of HCM, potentially influencing clinical outcomes.
  • Understanding these risks is crucial for patient management and risk stratification.

Purpose of the Study:

  • To assess the association between apical aneurysm size and major HCM risk factors with the incidence of lethal and potentially lethal arrhythmias.
  • To determine the incidence of unexplained stroke in patients with HCM and apical aneurysms.
  • To provide data for improved risk stratification and treatment strategies.

Main Methods:

  • Retrospective analysis of 108 patients from three HCM centers.
  • Assessment of American Heart Association/American College of Cardiology guidelines risk factors.
  • Measurement of initial aneurysm size using echocardiography and cardiac magnetic resonance imaging.
  • Follow-up assessment of outcomes including arrhythmias and stroke over a median of 5.9 years.

Main Results:

  • Implantable cardioverter defibrillator discharges or sudden cardiac death (SCD) occurred in 19.4% of patients.
  • Patients with risk factors had a significantly higher incidence of ventricular tachycardia (VT), ventricular fibrillation (VF), or SCD (55% vs. 10%).
  • Aneurysm size was associated with risk; the upper tercile had a 5-year cumulative risk of 35% compared to 6% for the lower tercile (P=0.0046).
  • Patients with aneurysms <2 cm² and no risk factors had a very low risk (2.5%) of VT, VF, or SCD.
  • Clinical atrial fibrillation (AF) was prevalent (45%) and commonly associated with stroke.
  • Unexplained stroke incidence was 0.5%/year.
  • Surgery and VT ablation showed moderate effectiveness in symptom reduction and preventing recurrent VT.

Conclusions:

  • Major HCM risk factors and apical aneurysm size are significant predictors of subsequent VT, VF, or SCD.
  • Patients with smaller aneurysms (<2 cm²) and without other risk factors have a low risk of life-threatening arrhythmias.
  • Clinical AF is common and a driver of stroke in this population.
  • Unexplained stroke is uncommon, with an incidence comparable to severe bleeding risk.
  • Interventional procedures like surgery and VT ablation can be effective in managing symptoms and preventing recurrent arrhythmias.
Abstract

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