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肥厚型心肌病中的心尖动脉瘤和左心室中部梗阻。

Apical Aneurysms and Mid-Left Ventricular Obstruction in Hypertrophic Cardiomyopathy.

机构信息

Hypertrophic Cardiomyopathy Program, Leon Charney Division of Cardiology, New York University Langone Health, New York, New York, USA; Echocardiography Laboratory, New York University Langone Health, New York, New York, USA.

Echocardiography Laboratory, New York University Langone Health, New York, New York, USA.

出版信息

JACC Cardiovasc Imaging. 2023 May;16(5):591-605. doi: 10.1016/j.jcmg.2022.11.013. Epub 2023 Jan 11.

Abstract

BACKGROUND

Apical left ventricular (LV) aneurysms in hypertrophic cardiomyopathy (HCM) are associated with adverse outcomes. The reported frequency of mid-LV obstruction has varied from 36% to 90%.

OBJECTIVES

The authors sought to ascertain the frequency of mid-LV obstruction in HCM apical aneurysms.

METHODS

The authors analyzed echocardiographic and cardiac magnetic resonance examinations of patients with aneurysms from 3 dedicated programs and compared them with 63 normal controls and 47 controls with apical-mid HCM who did not have aneurysms (22 with increased LV systolic velocities).

RESULTS

There were 108 patients with a mean age of 57.4 ± 13.5 years; 40 (37%) were women. A total of 103 aneurysm patients (95%) had mid-LV obstruction with mid-LV complete systolic emptying. Of the patients with obstruction, 84% had a midsystolic Doppler signal void, a marker of complete flow cessation, but only 19% had Doppler systolic gradients ≥30 mm Hg. Five patients (5%) had relative hypokinesia in mid-LV without obstruction. Aneurysm size is not bimodal but appears distributed by power law, with large aneurysms decidedly less common. Comparing mid-LV obstruction aneurysm patients with all control groups, the short-axis (SAX) systolic areas were smaller (P < 0.007), the percent SAX area change was greater (P < 0.005), the papillary muscle (PM) areas were larger (P < 0.003), and the diastolic PM areas/SAX diastolic areas were greater (P < 0.005). Patients with aneurysms had 22% greater SAX PM areas compared with those with elevated LV velocities but no aneurysms (median: 3.00 cm [IQR: 2.38-3.70 cm] vs 2.45 [IQR: 1.81-2.95 cm]; P = 0.004). Complete emptying occurs circumferentially around central PMs that contribute to obstruction. Late gadolinium enhancement was always brightest and the most transmural apical of, or at the level of, complete emptying.

CONCLUSIONS

The great majority (95%) of patients in the continuum of apical aneurysms have associated mid-LV obstruction. Further research to investigate obstruction as a contributing cause to apical aneurysms is warranted.

摘要

背景

肥厚型心肌病(HCM)中的心尖左心室(LV)瘤与不良结局相关。报道的中 LV 梗阻的频率从 36%到 90%不等。

目的

作者旨在确定 HCM 心尖瘤中中 LV 梗阻的频率。

方法

作者分析了 3 个专用程序中患有瘤的患者的超声心动图和心脏磁共振检查,并将其与 63 名正常对照者和 47 名无瘤的中尖部 HCM 对照者(22 名左心室收缩速度增加)进行了比较。

结果

共有 108 名患者,平均年龄 57.4±13.5 岁;40 名(37%)为女性。共有 103 名瘤患者(95%)存在中 LV 梗阻,且中 LV 完全收缩排空。梗阻患者中有 84%存在中收缩期多普勒信号缺失,这是完全血流停止的标志物,但仅有 19%的患者存在多普勒收缩期梯度≥30mmHg。5 名(5%)患者存在中 LV 无梗阻的相对运动障碍。瘤大小不是双峰分布,而是呈幂律分布,大瘤明显较少见。将中 LV 梗阻瘤患者与所有对照组比较,短轴(SAX)收缩面积较小(P<0.007),SAX 面积变化百分比较大(P<0.005),乳头肌(PM)面积较大(P<0.003),舒张期 PM 面积/SAX 舒张面积较大(P<0.005)。与左心室收缩速度升高但无瘤的患者相比,瘤患者的 SAX PM 面积增加了 22%(中位数:3.00cm[IQR:2.38-3.70cm]比 2.45cm[IQR:1.81-2.95cm];P=0.004)。完全排空发生在中央 PM 周围,中央 PM 有助于梗阻。晚期钆增强始终最亮,最靠近完全排空的心肌壁最厚。

结论

心尖瘤连续体中的绝大多数(95%)患者存在相关的中 LV 梗阻。进一步研究梗阻作为心尖瘤的致病原因是合理的。

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