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肥厚型心肌病中舒张早期室间隔中部血流与预示动脉瘤形成的弹力之间的关系。

Relation Between Early Diastolic Mid-Ventricular Flow and Elastic Forces Indicating Aneurysm Formation in Hypertrophic Cardiomyopathy.

机构信息

Department of Cardiology, Thoraxcenter, Erasmus MC, University Medical Center Rotterdam, Rotterdam, the Netherlands.

Department of Cardiology, Thoraxcenter, Erasmus MC, University Medical Center Rotterdam, Rotterdam, the Netherlands.

出版信息

J Am Soc Echocardiogr. 2022 Aug;35(8):846-856.e2. doi: 10.1016/j.echo.2022.04.010. Epub 2022 Apr 27.

DOI:10.1016/j.echo.2022.04.010
PMID:35489541
Abstract

BACKGROUND

The early diastolic paradoxical midventricular flow is suggestive of apical aneurysm (AA) formation in hypertrophic cardiomyopathy (HCM). We aimed to determine whether early diastolic paradoxical midventricular flow may be a useful screening tool in patients, following the time progression of HCM to the aneurysmal stage.

METHODS

One hundred twenty-one HCM patients with dominant hypertrophy in the mid and apical segments, based on echocardiography and/or cardiovascular magnetic resonance, were selected from our HCM database, which comprises 1,332 patients. They were further stratified according to the presence of AA. All imaging studies in a period of 16 years (2005-2021) were considered for time progression. Midventricular Doppler (pulsed-wave, continuous-wave, color, and color M mode) were analyzed.

RESULTS

Thirty-five patients (29% of the study group and 2.6% of all HCM patients) had AA. Early diastolic paradoxical midventricular flow had a sensitivity of 92% and specificity of 98.6% for the detection of AA in the study group. In 108 patients, follow-up echocardiography was performed (median, 5 [3-9] studies). Sixteen patients (15%) with 10 [7-12] years of follow-up displayed progressive time changes in left ventricle (LV) apical morphology and/or mid-LV flow. Ten patients (9%) progressed to an AA, during 7 [4-11] years of follow-up. Patients progressing to AA were younger (P = .009), with more severe LV hypertrophy (P = .01) and more often a significant mid-LV systolic gradient (≥30 mm Hg, P < .001). A wall thickness over 20 mm had 70% sensitivity and 69% specificity in detecting evolution toward AA. With significant systolic gradient, sensitivity was 80% and specificity was 62%. Furthermore, patients with AA had a higher incidence of ventricular tachycardia (log-rank P = .03).

CONCLUSIONS

Early diastolic paradoxical midventricular flow reliably detects AA presence and should prompt extra imaging studies. In HCM with mid and apical dominant involvement there is a progressive trend toward aneurysm formation, especially in patients with wall thickness over 20 mm and significant mid-LV systolic gradient (≥30 mm Hg), which can be monitored through serial Doppler studies.

摘要

背景

舒张早期矛盾性中段心室血流提示肥厚型心肌病(HCM)形成心尖瘤(AA)。我们旨在确定舒张早期矛盾性中段心室血流是否可以作为一种有用的筛查工具,用于监测 HCM 向瘤状阶段的时间进展。

方法

从我们的 HCM 数据库中选择了 121 名中段和心尖段优势性肥厚的 HCM 患者(基于超声心动图和/或心血管磁共振),数据库包含 1332 名患者。根据 AA 的存在对患者进一步分层。考虑了 16 年(2005-2021 年)的所有成像研究,以观察时间进展。分析中段心室多普勒(脉冲波、连续波、彩色和彩色 M 模式)。

结果

35 名患者(研究组的 29%和所有 HCM 患者的 2.6%)有心尖瘤。在研究组中,舒张早期矛盾性中段心室血流对 AA 的检测具有 92%的敏感性和 98.6%的特异性。在 108 名患者中进行了后续超声心动图检查(中位数,5 [3-9] 次)。16 名患者(15%)进行了 10 [7-12] 年的随访,左心室(LV)心尖形态和/或中段 LV 血流发生进行性时间变化。10 名患者(9%)在 7 [4-11] 年的随访中进展为 AA。进展为 AA 的患者更年轻(P =.009),LV 肥厚更严重(P =.01),中段 LV 收缩期梯度更显著(≥30mmHg,P<.001)。壁厚度超过 20mm 时,检测向 AA 进展的敏感性为 70%,特异性为 69%。在存在显著收缩期梯度时,敏感性为 80%,特异性为 62%。此外,心尖瘤患者发生室性心动过速的发生率更高(对数秩检验 P=.03)。

结论

舒张早期矛盾性中段心室血流可靠地检测 AA 的存在,并应提示进行额外的影像学检查。在中段和心尖段优势性受累的 HCM 中,向瘤状形成的进展趋势明显,尤其是壁厚度超过 20mm 和中段 LV 收缩期梯度显著(≥30mmHg)的患者,可以通过连续多普勒研究进行监测。

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