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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.
Abstract:
Background: Apical hypertrophic cardiomyopathy (aHCM) is thought to have a more benign clinical course compared to septal HCM (sHCM), but most data have been derived from Asian cohorts. Comparative data on clinical outcome in Caucasian aHCM cohorts are scarce, and the results are conflicting. The aim of this study was to estimate the prevalence and outcome of aHCM in French-Canadians of Caucasian descent. Methods and results: We conducted a retrospective, single-center cohort study. The primary endpoint was a composite of documented sustained ventricular arrhythmia (VA), appropriate ICD therapy, arrhythmogenic syncope, cardiac arrest, or all-cause mortality. A total of 301 HCM patients (65% males) were enrolled including 80/301 (27%) with aHCM and 221/301 (73%) with sHCM. Maximal wall thickness was similar in both groups. Left ventricular apical aneurysm was significantly more common in aHCM (10 vs. 0.5%; p < 0.001). The proportion of patients with myocardial fibrosis ≥ 15% of the left ventricular mass was similar between aHCM and sHCM (21 vs. 24%; p = 0.68). Secondary prevention ICDs were more often implanted in aHCM patients (16 vs. 7%; p = 0.02). The primary endpoint occurred in 26% of aHCM and 10.4% of sHCM patients (p = 0.001) and was driven by an increased incidence of sustained VA (10 vs. 2.3%; p = 0.01). Multivariate analysis identified apical aneurysm and a phenotype of aHCM as independent predictors of the primary endpoint and the occurrence of sustained ventricular tachycardia. Unexplained syncope and a family history of sudden cardiac death were additional predictors for sustained VA. Apical HCM was associated with an increased risk of ventricular arrhythmia even when excluding patients with apical aneurysm. Conclusions: The phenotype of apical HCM is much more common in French-Canadians (27%) of Caucasian descent compared to other Caucasian HCM populations. Apical HCM in French-Canadians is associated with an increased risk for ventricular arrhythmia.
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