Sherrid Mark V, Massera Daniele, Bernard Samuel, Tripathi Nidhi, Patel Yash, Modi Vivek, Axel Leon, Talebi Soheila, Saric Muhamed, Adlestein Elizabeth, Alvarez Isabel Castro, Reuter Maria C, Wu Woon Y, Xia Yuhe, Ghoshhajra Brian B, Sanborn Danita Y, Fifer Michael A, Swistel Daniel G, Kim Bette
Hypertrophic Cardiomyopathy Program; Leon Charney Division of Cardiology, Department of Medicine, NYU Grossman School of Medicine, New York City, New York, USA.
Echocardiography Laboratory, Leon Charney Division of Cardiology; Department of Medicine, NYU Grossman School of Medicine, New York City, New York, USA.
JACC Adv. 2024 Aug 27;3(10):101195. doi: 10.1016/j.jacadv.2024.101195. eCollection 2024 Oct.
There is controversy about risk of malignant arrhythmias and stroke in patients with apical aneurysms in hypertrophic cardiomyopathy (HCM).
The aim of this study was to estimate the associations of aneurysm size and major HCM risk factors with the incidence of lethal and potentially lethal arrhythmias and to estimate incidence of unexplained stroke.
In 108 patients (age 57.4 ± 13.5 years, 37% female) from 3 HCM centers, we assessed American Heart Association/American College of Cardiology guidelines risk factors and initial aneurysm size by echocardiography and cardiac magnetic resonance imaging and assessed outcomes after median 5.9 (IQR: 3.7-10.0) years.
Implantable cardioverter defibrillator discharges or sudden cardiac death (SCD) occurred in 21 (19.4%) patients. Of patients with a risk factor, 55% subsequently had ventricular tachycardia (VT), ventricular fibrillation (VF), or SCD at follow-up, compared with 10% in those who did not ( < 0.001). The upper tercile of size had a 5-year cumulative risk of 35%, while the lower tercile had 5-year risk of 6% ( = 0.0046). In those with the smallest aneurysms <2 cm and also without risk factors VT, VF, or SCD occurred in only 2.5%. Clinical atrial fibrillation (AF) was prevalent, occurring in 49 (45%). Stroke was commonly associated with AF. Stroke without conventional cause had an incidence of 0.5%/year. Surgery in 19% was effective in reducing symptoms. VT ablation and surgery were moderately effective in preventing recurrent VT.
Risk factors and aneurysm size were associated with subsequent VT, VF, or SCD. Patients with aneurysms in the lowest tercile of size have a low cumulative 5-year risk. Clinical AF occurred frequently. Stroke prevalence in absence of known stroke etiologies is uncommon and comparable to risk of severe bleeding.
肥厚型心肌病（HCM）患者心尖部动脉瘤发生恶性心律失常和中风的风险存在争议。
本研究旨在评估动脉瘤大小和主要HCM危险因素与致死性和潜在致死性心律失常发生率的相关性，并评估不明原因中风的发生率。
在来自3个HCM中心的108例患者（年龄57.4±13.5岁，37%为女性）中，我们通过超声心动图和心脏磁共振成像评估了美国心脏协会/美国心脏病学会指南中的危险因素和初始动脉瘤大小，并在中位随访5.9年（四分位间距：3.7 - 10.0年）后评估了结局。
21例（19.4%）患者发生了植入式心律转复除颤器放电或心源性猝死（SCD）。有危险因素的患者中，55%在随访时随后发生室性心动过速（VT）、心室颤动（VF）或SCD，而无危险因素的患者中这一比例为10%（P<0.001）。大小处于上三分位数的患者5年累积风险为35%，而下三分位数的患者5年风险为6%（P = 0.0046）。在动脉瘤最小且<2 cm且无危险因素的患者中，VT、VF或SCD仅发生在2.5%。临床房颤（AF）很常见，49例（45%）患者发生。中风通常与房颤相关。无传统病因的中风发生率为每年0.5%。19%的手术在减轻症状方面有效。VT消融和手术在预防VT复发方面有一定效果。
危险因素和动脉瘤大小与随后的VT、VF或SCD相关。动脉瘤大小处于最低三分位数的患者5年累积风险较低。临床房颤频繁发生。无已知中风病因的中风患病率不常见，与严重出血风险相当。
