肥厚型心肌病患者左心室纤维化特点及延迟钆增强心脏磁共振的临床应用现状

Features of left ventricle fibrosis in the patients with hypertrophic cardiomyopathy and the current status of clinical evaluation for the late gadolinium enhancement cardiac magnetic resonance

肥厚型心肌病(hypertrophic cardiomypathy,HCM)是最常见的心脏遗传性疾病,因其高致死率而备受关注。该疾病的心肌纤维化在病理、临床进展和影像学上有着独特性。多项研究显示HCM患者的不良预后与左心室纤维化程度密切相关。延迟钆增强心脏磁共振(late gadolinium enhancement cardiac magnetic resonance,LGE-CMR)是评估HCM患者左心室纤维化程度的金标准,有效运用LGE-CMR定量评估HCM患者纤维化程度有望成为HCM诊治的重要手段。

Hypertrophic cardiomyopathy(HCM)is the most common genetic heart disease,attracted much attention because of high mortality. The features of pathology,clinical progress and imaging of myocardial fibrosis in HCM patients are unique. Several studies have shown that the poor prognosis of HCM patients is closely related to the degree of left ventricular fibrosis. The late gadolinium enhancement cardiac magnetic resonance(LGE-CMR)is the gold standard to assess the degree of left ventricle fibrosis. Effecive utilization of LGE-CMR in the assessment of myocardial fibrosis in HCM patients is expected to be an important means of diagnosis and treatment of HCM.