■在阻塞性肥厚型心肌病(HOCM)中,尽管使用β-受体阻滞剂或维拉帕米,但仍有症状的患者使用丙吡胺。然而,由于应答者的定义不一致以及文献报道的随访时间不足,因此尚未明确确定丙吡胺治疗的有效性.为了解决这些缺点,我们从详细的数据库中进行了回顾性分析,并进行了长期随访,来自两个HCM转诊中心。
■62例有症状的HOCM患者(43%的女性,年龄52±14岁),静息或激发时左心室(LV)流出道梯度(LVOTG)≥50mmHg,是从两个意大利中心招募的。尽管进行了经典的药物治疗,但症状持续存在的患者仍加入了异吡胺作为二线治疗。基线时达到NYHAII级或I级的患者,基线时达到NYHAI级或症状稳定的NYHAII级患者被定义为应答者.
■在随访中,(平均4.4年,IQR1.1-6.6年),47名患者(76%)是应答者,而15人(24%)是无应答者。与无反应者相比,反应者在基线时显示出更大的LV舒张体积指数(LVEDVi)(61±14vs.49±16ml,分别,p=0.018),and,在后续行动中,LVOTG低于无反应者(43±32vs.66±28mmHg,分别,p=0.013),LVOTG<50mmHg在应答者中的代表性高于无应答者(75%与25%,分别为;p=0.004)。没有记录到需要停止治疗的副作用。
■HOCM患者在相当长的随访中接受了二吡胺作为二线治疗,症状得到了显着改善,这避免了高达70%的SRT。此外,我们的数据表明,基线时更大的LVEDVi可确定在治疗期间症状和LVOTG降至50mmHg以下方面获益最大的患者亚组.我们将讨论特定的情况,其中异吡胺可能优于肌球蛋白抑制,以确保充分考虑有效的治疗选择,而不是过早地驳回。
UNASSIGNED: In obstructive hypertrophic cardiomyopathy (HOCM), disopyramide is used in patients who remain symptomatic despite β-blockers or verapamil. However, effectiveness of disopyramide therapy has not been clearly established due to inconsistent definition of responders and the insufficient length of follow-ups reported in literature. To address these shortcomings, we have conducted a retrospective analysis from detailed databases with long follow-up, from two HCM Referral Centers.
UNASSIGNED: 62 symptomatic HOCM patients (43% women, age 52 ± 14 years) with left ventricular (LV) outflow tract gradient (LVOTG) ≥ 50 mmHg at rest or during provocation, were recruited from two Italian Centers. Disopyramide was added as second-line therapy in the patients in whom
symptoms persisted despite classic pharmacologic treatment. Patients in NYHA class > II at baseline who reached NYHA class II or I, and patients in NYHA class II at baseline who reached NYHA class I or
symptoms stabilization were defined as responders.
UNASSIGNED: At follow-up, (mean 4.4 years, IQR 1.1-6.6 years), 47 patients (76%) were responders, whereas 15 (24%) were no-responders. Responders showed larger LV diastolic volume index (LVEDVi) at baseline as compared to no-responders (61 ± 14 vs. 49 ± 16 ml, respectively, p = 0.018), and, at follow-up, reached lower LVOTG than no-responders (43 ± 32 vs. 66 ± 28 mmHg, respectively, p = 0.013), with a LVOTG <50 mmHg more represented in responders than in no-responders (75% vs. 25%, respectively; p = 0.004). No side effects requiring discontinuation of the therapy were recorded.
UNASSIGNED: HOCM patients treated with disopyramide as second-line therapy in a quite long-follow-up showed a significant improvement of
symptoms, which avoided SRT in up to 70% of them. Moreover, our data suggest that a larger LVEDVi at baseline identify the subgroup of patients who benefit the most from the therapy in terms of
symptoms and reduction of LVOTG below 50 mmHg during treatment. We will discuss specific situations where disopyramide may be preferred over myosin inhibition to ensure that effective therapeutic options are fully considered and not prematurely dismissed.