Clinical utility of cardiovascular magnetic resonance in hypertrophic cardiomyopathy.
Clinical utility of cardiovascular magnetic resonance in hypertrophic cardiomyopathy.
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DOI：
10.1186/1532-429x-14-13
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发表时间：
2012-02-01
期刊：
影响因子：
--
通讯作者：
Maron MS
中科院分区：
文献类型：
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作者：
Maron MS
肥厚型心肌病（HCM）的特点是大量的遗传和表型异质性，导致相当大的差异，在临床过程中，包括最常见的原因，猝死的年轻人和心力衰竭症状的决定因素，在任何年龄的患者。传统上，二维超声心动图是建立HCM临床诊断的最可靠方法。然而，心血管磁共振（CMR），其高空间分辨率和断层成像能力，已成为一种技术，特别适合于表征这种复杂疾病的不同表型表达。例如，CMR在HCM诊断中通常上级于超声心动图，通过识别节段性肥大区域（即，前外侧壁或心尖）不能通过超声心动图可靠地可视化（或在范围方面被低估）。经CMR确定的高风险HCM患者亚组包括薄壁瘢痕性LV心尖动脉瘤（在HCM中进行CMR成像之前，大部分未检测到）、终末期收缩功能障碍和巨大LV肥大。CMR观察结果还表明，HCM中的心肌病变过程比以前认为的更扩散，延伸到LV心肌以外，包括右心室壁增厚以及乳头肌和二尖瓣的大量形态多样性。这些发现对接受侵入性间隔缩小术的患者的管理策略有意义。在HCM家族成员中，CMR已经确定了在不存在LV肥大的情况下受影响遗传状态的独特表型标志物，包括：心肌隐窝、二尖瓣小叶延长和晚期钆增强。对比增强CMR与晚期钆增强识别心肌纤维化的独特能力提高了人们的期望，即这可能代表一种新的标志物，可增强风险分层。此时，晚期钆增强似乎是与收缩功能障碍相关的不良LV重构的重要决定因素。然而，LGE对猝死的预测意义尚未完全解决，最终未来的大型前瞻性研究可能会对这一问题提供更多的见解。这些观察结果强调了CMR在当代HCM患者评估中的重要作用，提供了影响诊断和临床管理策略的重要信息。
Hypertrophic cardiomyopathy (HCM) is characterized by substantial genetic and phenotypic heterogeneity, leading to considerable diversity in clinical course including the most common cause of sudden death in young people and a determinant of heart failure symptoms in patients of any age. Traditionally, two-dimensional echocardiography has been the most reliable method for establishing a clinical diagnosis of HCM. However, cardiovascular magnetic resonance (CMR), with its high spatial resolution and tomographic imaging capability, has emerged as a technique particularly well suited to characterize the diverse phenotypic expression of this complex disease. For example, CMR is often superior to echocardiography for HCM diagnosis, by identifying areas of segmental hypertrophy (ie., anterolateral wall or apex) not reliably visualized by echocardiography (or underestimated in terms of extent). High-risk HCM patient subgroups identified with CMR include those with thin-walled scarred LV apical aneurysms (which prior to CMR imaging in HCM remained largely undetected), end-stage systolic dysfunction, and massive LV hypertrophy. CMR observations also suggest that the cardiomyopathic process in HCM is more diffuse than previously regarded, extending beyond the LV myocardium to include thickening of the right ventricular wall as well as substantial morphologic diversity with regard to papillary muscles and mitral valve. These findings have implications for management strategies in patients undergoing invasive septal reduction therapy. Among HCM family members, CMR has identified unique phenotypic markers of affected genetic status in the absence of LV hypertrophy including: myocardial crypts, elongated mitral valve leaflets and late gadolinium enhancement. The unique capability of contrast-enhanced CMR with late gadolinium enhancement to identify myocardial fibrosis has raised the expectation that this may represent a novel marker, which may enhance risk stratification. At this time, late gadolinium enhancement appears to be an important determinant of adverse LV remodeling associated with systolic dysfunction. However, the predictive significance of LGE for sudden death is incompletely resolved and ultimately future large prospective studies may provide greater insights into this issue. These observations underscore an important role for CMR in the contemporary assessment of patients with HCM, providing important information impacting diagnosis and clinical management strategies.