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肥厚型梗阻性心肌病-左心室流出道形态及其血流动力学影响的 CMR 应用。

Hypertrophic obstructive cardiomyopathy-left ventricular outflow tract shapes and their hemodynamic influences applying CMR.

机构信息

ECRC Experimental and Clinical Research Center, Charité - Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Lindenberger Weg 80, 13125, Berlin, Germany.

Working Group on Cardiovascular Magnetic Resonance, Experimental and Clinical Research Center, Charité Medical Faculty and the Max-Delbrück Center for Molecular Medicine, Charité - Universitätsmedizin Berlin, Lindenberger Weg 80, 13125, Berlin, Germany.

出版信息

Int J Cardiovasc Imaging. 2024 Nov;40(11):2371-2381. doi: 10.1007/s10554-024-03242-4. Epub 2024 Sep 20.

Abstract

Hypertrophic cardiomyopathy (HCM) is one of the most common genetic cardiac disorders and is characterized by different phenotypes of left ventricular hypertrophy with and without obstruction. The effects of left ventricular outflow tract (LVOT) obstruction based on different anatomies may be hemodynamically relevant and influence therapeutic decision making. Cardiovascular magnetic resonance (CMR) provides anatomical information. We aimed to identify different shapes of LVOT-obstruction using Cardiovascular Magnetic Resonance (CMR). The study consisted of two parts: An in-vivo experiment for shape analysis and in-vitro part for the assessment of its hemodynamic consequences. In-vivo a 3D depiction of the LVOT was created using a 3D multi-slice reconstruction from 2D-slices (full coverage cine stack with 7 slices and a thickness of 5-6 mm with no gap) in 125 consecutive HOCM patients (age = 64.17 +/- 12.655; female n = 42). In-vitro an analysis of the LVOT regarding shape and flow behavior was conducted. For this purpose, 2D and 4D measurements were performed on 3D printed phantoms which were based on the anatomical characteristics of the in-vivo study, retrospectively. The in-vivo study identified three main shapes named K- (28.8%), X- (51.2%) and V-shape (10.4%) and a mixed one (9.6%). By analyzing the in-vitro flow measurements every shape showed an individual flow profile in relation to the maximum velocity in cm/s. Here, the V-shape showed the highest value of velocity (max. 138.87 cm/s). The X-shape was characterized by a similar profile but with lower velocity values (max. 125.39 cm/s), whereas the K-shape had an increase of the velocity without decrease (max. 137.11 cm/s). For the first time three different shapes of LVOT-obstruction could be identified. These variants seem to affect the hemodynamics in HOCM.

摘要

肥厚型心肌病(HCM)是最常见的遗传性心脏病之一,其特征是左心室肥厚伴有或不伴有梗阻的不同表型。基于不同解剖结构的左心室流出道(LVOT)梗阻的影响可能具有血流动力学相关性,并影响治疗决策。心血管磁共振(CMR)提供解剖学信息。我们旨在使用心血管磁共振(CMR)识别不同形状的 LVOT 梗阻。该研究包括两部分:一部分是用于形状分析的体内实验,一部分是用于评估其血流动力学后果的体外部分。在体内,使用二维切片的三维多切片重建(具有 7 个切片的全覆盖电影堆栈,厚度为 5-6mm,无间隙)对 125 例连续 HOCM 患者(年龄=64.17 +/- 12.655;女性 n=42)的 LVOT 进行三维描绘。在体外,对 LVOT 的形状和流动行为进行了分析。为此,对基于体内研究的解剖学特征的三维打印模型进行了二维和四维测量,这些模型是回顾性的。体内研究确定了三种主要形状,分别命名为 K-(28.8%)、X-(51.2%)和 V-形(10.4%)以及一种混合形状(9.6%)。通过分析体外流动测量结果,每种形状在与最大速度 cm/s 相关的关系中都显示出独特的流动曲线。在这里,V 形显示出最高的速度值(最大 138.87cm/s)。X 形的特点是速度相似但速度值较低(最大 125.39cm/s),而 K 形的速度增加而没有减少(最大 137.11cm/s)。这是第一次识别出三种不同形状的 LVOT 梗阻。这些变异似乎会影响 HOCM 的血液动力学。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3523/11561129/efbffc33d0a7/10554_2024_3242_Fig1_HTML.jpg

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