左束支传导阻滞心肌病 (LBBB-CMP):从特发性左束支传导阻滞这一并非良性的发现到左束支传导阻滞心肌病的诊断和治疗。

IF 1.4 4区 医学 Q3 CARDIAC & CARDIOVASCULAR SYSTEMS Heart and Vessels Pub Date : 2024-07-22 DOI:10.1007/s00380-024-02441-2
Catarina Amaral Marques, André Cabrita, Ana Isabel Pinho, Luís Santos, Cátia Oliveira, Rui André Rodrigues, Cristina Cruz, Elisabete Martins
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引用次数: 0

摘要

导言 特发性左束支传导阻滞(iLBBB)并不常见。尽管其自然病史仍不甚明了,但其良性性已受到越来越多的质疑。同样,左束支传导阻滞-心肌病(LBBB-CM)也被越来越多的人认为是一种独特的疾病,机电不同步似乎在左心室功能障碍(LVD)的发展中起着核心作用。然而,这仍然是一个鲜有研究的课题。目标:两个主要目标:(1)探索 "无症状 "iLBBB携带者的自然史;(2)描述可能的LBBB-CMP患者(pts)"真实世界 "队列中的结果和治疗方法。方法:三级医疗中心回顾性研究,研究对象为iLBBB和可能的LBBB-CMP患者,筛选自2011年至2017年的大型医院心电图数据库(LBBB = 347)。为了实现第一个目标,研究只纳入了左室射血分数(LVEF)≥50%且有随访(FU)数据的患者(n = 152)。关于第二个目标,我们选择了可能的 LBBB-CMP 患者,并将其定义为有 LVD 的 iLBBB 患者(LVEF 结果:针对第一个目标,我们确定了 152 名 iLBBB 携带者。中位生存时间为 8 年,61% 为女性。在治疗期间,约 25% 的患者出现了 LVD,20% 的患者需要住院治疗心血管疾病(CV)≥ 1 次,15% 的患者需要植入心脏设备。大部分(2/3)在持续治疗期间出现 LVD 的患者(n = 35)没有继发 LVD 的原因,被归类为可能的 LBBB-CMP 患者。LVD发生时间分析表明,已知LVD原因的患者与LBBB-CMP患者之间没有差异(Log-rank = 0.713)。关于第二个目标,53 例可能的 LBBB-CMP 患者被确定。中位治疗时间为10年,51%为女性。在治疗期间,77%的患者出现心力衰竭(HF)症状,42%的患者需要≥1次CV住院治疗,主要原因是HF。半数患者在某个时间点出现了严重的低密度心衰,55%的患者需要使用心脏设备,其中大部分是心脏再同步化治疗(CRT)设备。将CRT与非CRT患者进行比较后发现,两者在药物治疗方面没有差异,但CRT组的疗效更好:LVEF 改善率更高(非 CRT 组 LVEF 改善率中位数为 11%,CRT 组为 27%;P 结论:我们的数据加强了目前有关 iLBBB 自然史的证据,显示了与 iLBBB 存在相关的重大心血管疾病发病率,并加强了对这些携带者进行连续和适当 FU 的必要性。我们关于 "真实世界 "中可能存在的 LBBB-CMP 病例的数据显示,心血管事件(即 HF 相关事件)的发生率很高,这支持了越来越多的证据指出 CRT 是这一亚组病例的治疗基石。总之,我们的工作进一步揭示了这些在很大程度上不为人知的课题,并强调迫切需要进行更大规模的前瞻性研究,以确定 iLBBB 携带者 LVD 发展的预测因素,并建立 LBBB-CMP 的诊断标准和治疗方法。
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Left bundle branch block cardiomyopathy (LBBB-CMP): from the not-so-benign finding of idiopathic LBBB to LBBB-CMP diagnosis and treatment.

Introduction Idiopathic left bundle branch block (iLBBB) is an uncommon finding. Its benignity has been increasingly questioned, though its natural history remains poorly clarified. Similarly, LBBB-cardiomyopathy (LBBB-CM) has been also increasingly recognized as a distinct entity, where electromechanical dyssynchrony seems to play a central role in left ventricular dysfunction (LVD) development. Still, it remains a scarcely studied topic. There is an urgent need for investigation and evidence reinforcement in these areas.

Objectives: two main objectives: (1) to explore the natural history of "asymptomatic" iLBBB carriers; (2) to characterize the outcomes and therapeutic approach used in a "real-world" cohort of possible LBBB-CMP patients (pts).

Methods: tertiary care centre retrospective study of pts with iLBBB and possible LBBB-CMP, screened from a large hospital electrocardiographic database from 2011 to 2017 (LBBB = 347). To assign the 1st objective, only pts with left ventricular ejection fraction (LVEF) ≥ 50% and available follow-up (FU) data were included (n = 152). Regarding the 2nd objective, possible LBBB-CMP pts were selected and defined as iLBBB pts with LVD (LVEF < 50%) and no secondary causes for LVD (n = 53). Data were based on pts' careful review of medical records.

Results: focusing our 1st objective, 152 iLBBB carriers were identified. Median FU time were 8 years, and 61% were female. During FU, approximately 25% developed LVD, 20% needed ≥ 1 cardiovascular (CV) hospitalization, and 15% needed a cardiac device implantation. The majority (2/3) of pts with LVD on FU (n = 35) had no secondary causes for LVD, being classified as possible LBBB-CMP pts. Time-to-LVD analysis showed no differences between pts with a known cause for LVD vs LBBB-CMP pts (Log-rank = 0.713). Concerning the 2nd objective, 53 possible LBBB-CMP pts were identified. Median FU time were 10 years, and 51% were female. During the FU, 77% presented heart failure (HF) symptoms, and 42% needed ≥ 1 CV hospitalization, mainly due to HF. Half presented severe LVD at some point in time, and 55% needed a cardiac device, most of them a cardiac resynchronization therapy (CRT) device. Comparing CRT with non-CRT pts, no differences were found in terms of medical therapy, but better outcomes were observed in CRT group: LVEF improvement was higher (median LVEF improvement of 11% in non-CRT vs 27% in CRT; p < 0.001), and fully recovery from LVD was more frequent (50% of CRT vs 14% non-CRT; p = 0.028).

Conclusion: our data strengthen current evidence on natural history of iLBBB, showing significant CV morbidity associated with the presence of iLBBB, and reinforces the need for a serial and proper FU of these carriers. Our data on "real-world" possible LBBB-CMP pts shows high rates of CV events, namely HF-related events, and supports the growing evidence pointing out CRT as this subgroup of pts' cornerstone of treatment. In conclusion, our work sheds additional light on these largely unknown topics and underlines the urgent need for larger and prospective studies addressing the identification of LVD development predictors in iLBBB carriers, as well as the establishment of diagnostic criteria and therapeutic approach for LBBB-CMP.

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来源期刊
Heart and Vessels
Heart and Vessels 医学-外周血管病
CiteScore
3.10
自引率
13.30%
发文量
211
审稿时长
2 months
期刊介绍: Heart and Vessels is an English-language journal that provides a forum of original ideas, excellent methods, and fascinating techniques on cardiovascular disease fields. All papers submitted for publication are evaluated only with regard to scientific quality and relevance to the heart and vessels. Contributions from those engaged in practical medicine, as well as from those involved in basic research, are welcomed.
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