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

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 ai...

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Main Authors: Christian Steinberg, Charles Nadeau-Routhier, Philippe André, François Philippon, Jean-François Sarrazin, Isabelle Nault, Gilles O'Hara, Louis Blier, Franck Molin, Benoit Plourde, Karine Roy, Eric Larose, Marie Arsenault, Jean Champagne
Format: Article
Language:English
Published: Frontiers Media S.A. 2020-10-01
Series:Frontiers in Cardiovascular Medicine
Subjects:
Online Access:https://www.frontiersin.org/articles/10.3389/fcvm.2020.548564/full
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author Christian Steinberg
Charles Nadeau-Routhier
Philippe André
François Philippon
Jean-François Sarrazin
Isabelle Nault
Gilles O'Hara
Louis Blier
Franck Molin
Benoit Plourde
Karine Roy
Eric Larose
Marie Arsenault
Jean Champagne
author_facet Christian Steinberg
Charles Nadeau-Routhier
Philippe André
François Philippon
Jean-François Sarrazin
Isabelle Nault
Gilles O'Hara
Louis Blier
Franck Molin
Benoit Plourde
Karine Roy
Eric Larose
Marie Arsenault
Jean Champagne
author_sort Christian Steinberg
collection DOAJ
description 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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spelling doaj.art-dd017c4ee982407f9950061f8814bb582022-12-21T23:07:31ZengFrontiers Media S.A.Frontiers in Cardiovascular Medicine2297-055X2020-10-01710.3389/fcvm.2020.548564548564Ventricular Arrhythmia in Septal and Apical Hypertrophic Cardiomyopathy: The French-Canadian ExperienceChristian SteinbergCharles Nadeau-RouthierPhilippe AndréFrançois PhilipponJean-François SarrazinIsabelle NaultGilles O'HaraLouis BlierFranck MolinBenoit PlourdeKarine RoyEric LaroseMarie ArsenaultJean ChampagneBackground: 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.https://www.frontiersin.org/articles/10.3389/fcvm.2020.548564/fullhypertrophic cardiomyopathyapical hypertrophic cardiomyopathyventricular arrhythmiaseptal hypertrophic cardiomyopathyFrench-Canadian
spellingShingle Christian Steinberg
Charles Nadeau-Routhier
Philippe André
François Philippon
Jean-François Sarrazin
Isabelle Nault
Gilles O'Hara
Louis Blier
Franck Molin
Benoit Plourde
Karine Roy
Eric Larose
Marie Arsenault
Jean Champagne
Ventricular Arrhythmia in Septal and Apical Hypertrophic Cardiomyopathy: The French-Canadian Experience
Frontiers in Cardiovascular Medicine
hypertrophic cardiomyopathy
apical hypertrophic cardiomyopathy
ventricular arrhythmia
septal hypertrophic cardiomyopathy
French-Canadian
title Ventricular Arrhythmia in Septal and Apical Hypertrophic Cardiomyopathy: The French-Canadian Experience
title_full Ventricular Arrhythmia in Septal and Apical Hypertrophic Cardiomyopathy: The French-Canadian Experience
title_fullStr Ventricular Arrhythmia in Septal and Apical Hypertrophic Cardiomyopathy: The French-Canadian Experience
title_full_unstemmed Ventricular Arrhythmia in Septal and Apical Hypertrophic Cardiomyopathy: The French-Canadian Experience
title_short Ventricular Arrhythmia in Septal and Apical Hypertrophic Cardiomyopathy: The French-Canadian Experience
title_sort ventricular arrhythmia in septal and apical hypertrophic cardiomyopathy the french canadian experience
topic hypertrophic cardiomyopathy
apical hypertrophic cardiomyopathy
ventricular arrhythmia
septal hypertrophic cardiomyopathy
French-Canadian
url https://www.frontiersin.org/articles/10.3389/fcvm.2020.548564/full
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