Ventricular Arrhythmia in Septal and Apical Hypertrophic Cardiomyopathy: The French-Canadian Experience

Christian Steinberg1, Charles Nadeau-Routhier1, Philippe André1

  • 1Division of Cardiology, Multidisciplinary Cardiovascular Department, Institut Universitaire de Cardiologie et Pneumologie de Québec (IUCPQ-UL), Université Laval, Québec City, QC, Canada.

Insights

Apical hypertrophic cardiomyopathy (aHCM) is more common in French-Canadians and carries a higher risk of ventricular arrhythmias. This contrasts with the generally assumed benign course of aHCM, highlighting the need for closer monitoring in this population.

Area of Science:

  • Cardiology
  • Genetics
  • Internal Medicine

Background:

  • Apical hypertrophic cardiomyopathy (aHCM) is often considered to have a more favorable prognosis than septal hypertrophic cardiomyopathy (sHCM).
  • Existing data predominantly originate from Asian populations, with limited and conflicting information available for Caucasian cohorts.
  • The clinical outcomes and prevalence of aHCM in Caucasian populations, particularly French-Canadians, remain understudied.

Purpose of the Study:

  • To determine the prevalence of apical hypertrophic cardiomyopathy (aHCM) in a French-Canadian cohort of Caucasian descent.
  • To compare the clinical outcomes between patients with aHCM and those with septal hypertrophic cardiomyopathy (sHCM).
  • To identify predictors of adverse events in patients with aHCM.

Main Methods:

  • A retrospective, single-center cohort study was conducted.
  • 301 patients with hypertrophic cardiomyopathy (HCM) were analyzed, categorizing them into aHCM and sHCM groups.
  • The primary endpoint included sustained ventricular arrhythmia (VA), appropriate implantable cardioverter-defibrillator (ICD) therapy, syncope, cardiac arrest, or all-cause mortality.

Main Results:

  • Apical hypertrophic cardiomyopathy (aHCM) was identified in 27% of the cohort, a higher prevalence than typically reported in other Caucasian populations.
  • Patients with aHCM showed a significantly higher incidence of the primary endpoint (26% vs. 10.4%, p=0.001), primarily driven by sustained ventricular arrhythmias (VA).
  • Apical aneurysm and the aHCM phenotype were independent predictors of adverse outcomes, including sustained ventricular tachycardia.

Conclusions:

  • Apical hypertrophic cardiomyopathy (aHCM) is notably more prevalent in French-Canadians of Caucasian descent compared to other Caucasian HCM cohorts.
  • In this population, aHCM is associated with an increased risk of ventricular arrhythmias, challenging the notion of a universally benign course.
  • The presence of apical aneurysm and unexplained syncope are significant risk factors for adverse arrhythmic events in aHCM patients.

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