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心脏磁共振成像在肥厚型心肌病中的作用。

The role of cardiac magnetic resonance in hypertrophic cardiomyopathy.

作者信息

Duarte Sérgio, Bogaert Jan

机构信息

Serviço de Radiologia, Hospital Garcia de Orta, Almada, Portugal.

出版信息

Rev Port Cardiol. 2010 Jan;29(1):79-93.

Abstract

Hypertrophic cardiomyopathy (HCM) is a common genetic cardiac disorder, typically characterized by a hypertrophied, non-dilated left ventricle (LV). Since the phenotypic expression of HCM varies widely, and other diseases may mimic it, imaging has a central role in its diagnosis. Though transthoracic echocardiography is the first-line imaging modality to study patients with HCM, cardiac magnetic resonance (CMR) imaging has evolved in recent years into a highly useful technique providing valuable additional information in the diagnosis of HCM and in differentiating it from other disorders. Unfortunately this technique is still considered by many clinicians as preclinical with limited use in daily clinical practice. In fact, CMR is currently the standard for morphological and functional cardiac assessment, and can easily evaluate the entire heart without technical or patient-related constraints. Moreover, no other noninvasive imaging technique can currently compete with CMR in terms of tissue characterization. These strengths applied in HCM patients provide an accurate means to morphologically describe phenotypic expression, to evaluate the impact on regional and global function and intraventricular flow patterns, and to quantify gradients and regurgitant fractions. Contrast-enhanced CMR can depict areas of scarring within abnormal myocardial segments, making CMR appealing for risk stratification. Moreover, concomitant pathology of the aortic valve and thoracic aorta, other primary or secondary myocardial disorders, and other conditions that may present with myocardial hypertrophy can be clearly depicted or ruled out. Finally, its non-invasive nature and excellent reproducibility favor CMR as a follow-up technique for evaluation of disease progression. It is our hope that this review paper will provide a better appreciation of the contribution of CMR among other imaging modalities in the diagnosis of HCM.

摘要

肥厚型心肌病(HCM)是一种常见的遗传性心脏疾病,其典型特征是左心室(LV)肥厚且无扩张。由于HCM的表型表达差异很大,且其他疾病可能与之相似,因此影像学在其诊断中起着核心作用。虽然经胸超声心动图是研究HCM患者的一线影像学检查方法,但近年来心脏磁共振(CMR)成像已发展成为一种非常有用的技术,在HCM的诊断以及将其与其他疾病区分开来方面提供了有价值的额外信息。不幸的是,许多临床医生仍认为这项技术尚处于临床前阶段,在日常临床实践中的应用有限。事实上,CMR目前是心脏形态和功能评估的标准,并且可以在没有技术或患者相关限制的情况下轻松评估整个心脏。此外,目前没有其他非侵入性成像技术在组织特征描述方面能与CMR相媲美。这些优势应用于HCM患者,为形态学描述表型表达、评估对局部和整体功能以及心室内血流模式的影响,以及量化梯度和反流分数提供了准确的方法。对比增强CMR可以描绘异常心肌节段内的瘢痕区域,这使得CMR在风险分层方面具有吸引力。此外,可以清晰地描绘或排除主动脉瓣和胸主动脉的合并病变、其他原发性或继发性心肌疾病以及可能表现为心肌肥厚的其他情况。最后,其非侵入性性质和出色的可重复性使CMR成为评估疾病进展的随访技术。我们希望这篇综述文章能让大家更好地认识到CMR在HCM诊断中的贡献以及其他成像方式的作用。

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