Cardiomyopathy Unit, Careggi University Hospital, Florence, Italy (N.M., C.F., I.O.).
Service of Cardiology, University Hospital of Lausanne, Switzerland (N.M., P.A., I.S.).
Circulation. 2024 Oct 22;150(17):1377-1390. doi: 10.1161/CIRCULATIONAHA.124.069378. Epub 2024 Oct 2.
Septal reduction therapy (SRT) provides substantial symptomatic improvement in patients with obstructive hypertrophic cardiomyopathy (HCM). However, long-term disease course after SRT and predictors of adverse outcomes have not been systematically examined.
Data from 13 high clinical volume HCM centers from the international SHARE (Sarcomeric Human Cardiomyopathy Registry) were analyzed. Patients were followed from the time of SRT until last follow-up or occurrence of heart failure (HF) composite outcome (cardiac transplantation, implantation of a left ventricular assist device, left ventricular ejection fraction <35%, development of New York Heart Association class III or IV symptoms), ventricular arrhythmias composite outcome (sudden cardiac death, resuscitated cardiac arrest, or appropriate implantable cardioverter defibrillator therapy), or HCM-related death. Cox proportional hazards models were used to identify predictors of outcome.
Of the 10 225 patients in SHARE, 1832 (18%; 968 [53%] male) underwent SRT, including 455 (25%) with alcohol septal ablation and 1377 (75%) with septal myectomy. The periprocedural 30-day mortality rate was 0.4% (8 of 1832) and 1499 of 1565 (92%) had a maximal left ventricular outflow tract gradient <50 mm Hg at 1 year. After 6.8 years (range, 3.4-9.8 years; 12 565 person-years) from SRT, 77 (4%) experienced HCM-related death (0.6% per year), 236 (13%) a composite HF outcome (1.9% per year), and 87 (5%) a composite ventricular arrhythmia outcome (0.7% per year). Among adults, older age at SRT was associated with a higher incidence of HCM death (hazard ratio, 1.22 [95 CI, 1.1-1.3]; <0.01) and the HF composite (hazard ratio, 1.14 [95 CI, 1.1-1.2] per 5-year increase; <0.01) in a multivariable model. Female patients also had a higher risk of the HF composite after SRT (hazard ratio, 1.4 [95 CI, 1.1-1.8]; <0.01). De novo atrial fibrillation occurred after SRT in 387 patients (21%). Among pediatric patients followed for a median of 13 years after SRT, 26 of 343 (16%) developed the HF composite outcome, despite 96% being free of recurrent left ventricular outflow tract obstruction.
Successful short- and long-term relief of outflow tract obstruction was observed in experienced multidisciplinary HCM centers. A subset of patients progressed to develop HF, but event-free survival at 10 years was 83% and ventricular arrhythmias were rare. Older age, female sex, and SRT during childhood were associated with a greater risk of developing HF.
间隔缩减治疗（SRT）可显著改善梗阻性肥厚型心肌病（HCM）患者的症状。然而，SRT 后的长期疾病过程和不良结局的预测因素尚未得到系统研究。
分析了国际 SHARE（肌节性人类心肌病注册中心）13 个高临床量 HCM 中心的数据。患者从 SRT 时开始随访，直至最后一次随访或出现心力衰竭（HF）复合结局（心脏移植、左心室辅助装置植入、左心室射血分数<35%、纽约心脏协会 III 或 IV 级症状发展）、室性心律失常复合结局（心源性猝死、复苏性心脏骤停或适当的植入式心脏复律除颤器治疗）或 HCM 相关死亡。使用 Cox 比例风险模型来确定结局的预测因素。
在 SHARE 的 10225 例患者中，1832 例（18%；968 例[53%]为男性）接受了 SRT，包括 455 例（25%）接受酒精间隔消融术和 1377 例（75%）接受间隔心肌切除术。围手术期 30 天死亡率为 0.4%（1832 例中的 8 例），1565 例中有 1499 例（92%）在 1 年内左心室流出道最大梯度<50mmHg。在 SRT 后 6.8 年（范围 3.4-9.8 年；12565 人年），77 例（4%）发生与 HCM 相关的死亡（0.6%/年），236 例（13%）发生 HF 复合结局（1.9%/年），87 例（5%）发生室性心律失常复合结局（0.7%/年）。在成年人中，SRT 时年龄较大与 HCM 死亡（风险比，1.22[95%置信区间，1.1-1.3]；<0.01）和 HF 复合结局（风险比，每 5 年增加 1.14[95%置信区间，1.1-1.2]）的发生率较高相关（<0.01）。在多变量模型中，女性患者在 SRT 后也有较高的 HF 复合结局风险（风险比，1.4[95%置信区间，1.1-1.8]；<0.01）。SRT 后 387 例（21%）患者新发心房颤动。在 SRT 后中位随访 13 年的儿科患者中，尽管 96%的患者无复发性左心室流出道梗阻，但 343 例中有 26 例（16%）发生 HF 复合结局。
在经验丰富的多学科 HCM 中心，观察到短期和长期流出道梗阻缓解成功。有一部分患者进展为 HF，但 10 年无事件生存率为 83%，室性心律失常罕见。年龄较大、女性和儿童时期的 SRT 与 HF 发生风险增加相关。
