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%;p0.001). 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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机译：背景：与隔膜HCM（SHCM）相比，ApciC肥厚性心肌病（AHCM）被认为具有更加良性的临床课程，但大多数数据来自亚洲群组。关于高加索AHCM队列的临床结果的比较数据稀缺，结果是矛盾的。本研究的目的是估计白种人血统法国加拿大人中AHCM的患病率和结果。方法和结果：我们进行了回顾性，单中心队列研究。主要终点是记录的持续心律失常（VA）的复合物，适当的ICD-治疗，心律源晕厥，心脏骤停或全导致死亡率。共有301例HCM患者（65％的男性），包括80/301（27％），SHCM和221/301（73％）。两个组中最大壁厚相似。左心室顶端动脉瘤在AHCM中更常见（10％Vs 0.5％; P <0.001）。心肌纤维化患者的比例≥15％的左心室质量在AHCM和SHCM之间相似（21％Vs 24％; P = 0.68）。二次预防ICD常常植入AHCM患者（16％vs 7％; P = 0.02）。主要终点以26％的AHCM和10.4％的SHCM患者（P = 0.001）发生，并且由持续的VA的发生率增加（10％Vs 2.3％; P = 0.01）。多变量分析鉴定为AHCM作为初级终点的独立预测因子的AHCM和持续心室性心动过缓的表型。不明原因的晕厥和突然心脏死亡的家族史是持续VA的额外预测因子。即使在排除患有顶端动脉瘤的患者时，Apick HCM也与心间心律失常的风险增加有关。结论：与其他高加索人HCM人口相比，Apick HCM的表型在法国 - 加拿大人（27％）中的白种人血统中更常见。法国加拿大人的顶端HCM与心间心律失常的风险增加有关。
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