Background

Management of young adults with hypertrophic cardiomyopathy (HCM) is challenging.

Aims

To evaluate the profile of young adults (16–25 years) with HCM included in the French prospective HCM registry.

Methods

Patients were compared according to occurrence of major adverse cardiac events (MACE), comprising sudden cardiac death (SCD) events (implantable cardioverter defibrillator [ICD] discharge, SCD, sustained ventricular tachycardia), atrial fibrillation/embolic stroke, heart failure hospitalisation and unexplained syncope, at a mean follow-up of 4.4 ± 2.2 years.

Results

At baseline, among 61 patients (20.5 ± 3.0 years; 16 women, 26.2%), 13 (21.3%) had a prophylactic ICD, 24.6% a family history of SCD, 29.5% obstruction, 86.0% magnetic resonance imaging myocardial fibrosis, 11.8% abnormal exercise blood pressure and 52.8% a European Society of Cardiology (ESC) 5-year SCD score < 4% (24.5% ≥ 6%). At follow-up, 15 patients (24.6%; seven women; all with fibrosis) presented 17 MACE, comprising: SCD events (n = 7, 41.2%; including three patients with an ICD, five with at least one SCD major classical risk factor and an ESC score ≥ 5% and two with no risk factors and an ESC score < 4%); atrial fibrillation/stroke (n = 6, 35.3%); heart failure (n = 1, 5.9%); syncope (n = 3, 17.6%). An ICD was implanted in 11 patients (four for secondary prevention), but in only 61.5% of patients with a score ≥ 6%. Only obstruction significantly increased MACE risk (odds ratio 3.96; P = 0.035), with a non-significant trend towards a lower risk in men (OR 0.29; P = 0.065).

Conclusions

In young adults with HCM, MACE are common in the short term, especially in obstructive HCM and women, mostly arrhythmic in origin. Prophylactic ICD implantation is frequent and does not strictly follow the guidelines, while the use of European/USA guidelines is helpful but imperfect in identifying SCD risk.

中文翻译：

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年轻成人肥厚型心肌病的管理和结果

背景

对患有肥厚型心肌病 (HCM) 的年轻成人的管理具有挑战性。

宗旨

评估法国前瞻性 HCM 登记册中包含的 HCM 的年轻成人（16-25 岁）的概况。

方法

根据主要不良心脏事件 (MACE) 的发生情况对患者进行比较，包括心源性猝死 (SCD) 事件（植入式心律转复除颤器 [ICD] 放电、SCD、持续性室性心动过速）、房颤/栓塞性卒中、心力衰竭住院和不明原因晕厥，平均随访 4.4  ±  2.2 年。

结果

在基线时，在 61 名患者（20.5  ±  3.0 岁；16 名女性，26.2%）中，13 名（21.3%）患有预防性 ICD，24.6% 有 SCD 家族史，29.5% 阻塞，86.0% 磁共振成像心肌纤维化，11.8 % 异常运动血压和 52.8% 欧洲心脏病学会 (ESC) 5 年 SCD 评分 <  4% (24.5%  ≥  6%)。随访时，15 名患者（24.6%；7 名女性；均患有纤维化）出现 17 次 MACE，包括：SCD 事件（n  =  7，41.2%；包括 3 名 ICD 患者，5 名至少有一种 SCD 主要经典风险）因素和 ESC 评分 ≥  5% 和两个没有危险因素且 ESC 评分 <  4%）；心房颤动/中风 ( n  = 6, 35.3%); 心力衰竭 ( n  =  1, 5.9%); 晕厥 ( n  =  3, 17.6%)。11 名患者（其中 4 名用于二级预防）植入了 ICD，但只有 61.5% 的患者评分 ≥  6%。只有阻塞显着增加了 MACE 风险（比值比 3.96；P  =  0.035），男性风险降低的趋势不显着（OR 0.29；P  =  0.065）。

结论

在患有 HCM 的年轻成人中，MACE 在短期内很常见，尤其是阻塞性 HCM 和女性，主要是心律失常。预防性 ICD 植入很频繁，并没有严格遵循指南，而欧洲/美国指南的使用有助于识别 SCD 风险，但并不完善。