Maron Martin S
Hypertrophic Cardiomyopathy Center, Division of Cardiology, Tufts Medical Center, Massachusetts 02111, USA.
Zhonghua Xin Xue Guan Bing Za Zhi. 2009 Dec;37(12):1057-63.
Hypertrophic cardiomyopathy (HCM) is the most common genetic cardiomyopathy and the leading cause of sudden death in young people and a major cause of heart failure symptoms at any age. Due to its genetic etiology, there is substantial heterogeneity in the phenotypic expression and clinical course of patients with HCM. Traditionally, two-dimensional echocardiography has been the easiest and reliable technique for establishing a diagnosis of HCM. However, cardiovascular magnetic resonance (CMR) has emerged as a novel, 3-dimensional tomographic imaging technique, which provides high spatial and temporal resolution images of the heart (not limited by thoracic or pulmonary parenchyma), in any plane and without ionizing radiation. As a result, CMR is particularly well suited to provide detailed characterization of the HCM phenotype, including a precise assessment of the location and distribution of LV wall thickening (as well as other myocardial structures such as the right ventricle and papillary muscles). In this regard, CMR has been demonstrated to provide a diagnosis of HCM in cases where the echocardiogram was non-diagnostic. Furthermore, CMR provides an accurate assessment of total LV mass which is a more robust marker of hypertrophy, with potential implications for risk stratification. In addition, with the intravenous administration of gadolinium, first-pass perfusion sequences can identify myocardial perfusion abnormalities, while late gadolinium enhancement sequences can identify areas of myocardial fibrosis/scarring. Although the clinical implications of late gadolinium enhancement in HCM are still uncertain this information may, in the near-future, have important implications with regard to identifying HCM patients at high risk of sudden death and progressive heart failure, including evolution into the end-stage phase of HCM. Therefore, at present, CMR provides important information impacting on diagnosis and clinical management strategies in patients with HCM and will likely have an expanding role in the evaluation of patients with this complex disease.
肥厚型心肌病（HCM）是最常见的遗传性心肌病，是年轻人猝死的主要原因，也是任何年龄段心力衰竭症状的主要原因。由于其遗传病因，HCM患者的表型表达和临床病程存在很大异质性。传统上，二维超声心动图一直是诊断HCM最简单可靠的技术。然而，心血管磁共振成像（CMR）已成为一种新型的三维断层成像技术，它可以在任何平面上提供心脏的高空间和时间分辨率图像（不受胸部或肺实质的限制），且无电离辐射。因此，CMR特别适合于详细描述HCM的表型，包括精确评估左心室壁增厚的位置和分布（以及其他心肌结构，如右心室和乳头肌）。在这方面，已证明CMR可在超声心动图无法诊断的情况下诊断HCM。此外，CMR能准确评估左心室总质量，这是肥大的一个更可靠指标，对风险分层具有潜在意义。此外，通过静脉注射钆，首次通过灌注序列可以识别心肌灌注异常，而延迟钆增强序列可以识别心肌纤维化/瘢痕形成区域。虽然延迟钆增强在HCM中的临床意义仍不确定，但在不久的将来，这些信息可能对识别有猝死和进行性心力衰竭高风险的HCM患者具有重要意义，包括发展到HCM终末期。因此，目前CMR为影响HCM患者诊断和临床管理策略提供了重要信息，并且在评估这种复杂疾病的患者中可能会发挥越来越大的作用。
