Rowin Ethan J, Maron Martin S
Hypertrophic Cardiomyopathy Institute, Division of Cardiology, Tufts Medical Center, Boston, MA; Chanin T. Mast Center for Hypertrophic Cardiomyopathy, Morristown Medical Center, Morristown, NJ, USA.
Arrhythm Electrophysiol Rev. 2016;5(3):197-202. doi: 10.15420/aer.2016:13:3.
Hypertrophic cardiomyopathy (HCM), the most common genetic cardiomyopathy, is a disease characterised by substantial heterogeneity. Although the majority of patients with HCM remain asymptomatic with near-normal longevity, a small, but important, subset remain at risk for a wide range of clinical outcomes including sudden death. Cardiovascular magnetic resonance (CMR), with its high spatial resolution and tomographic imaging capability, has emerged as an imaging modality particularly well suited to characterise the phenotypic expression of HCM. CMR helps in the diagnosis of HCM by identifying areas of hypertrophy not well visualised by echocardiography, providing more accurate wall thickness measurements and differentiating HCM from other causes of left ventricular (LV) hypertrophy. CMR has led to the identification of novel subgroups of patients with HCM, including those with LV apical aneurysms (a subgroup at increased risk for ventricular arrhythmias and thromboembolic stroke), as well as abnormalities that contribute to LV outflow obstruction. Additionally, contrast-enhanced CMR with late-gadolinium enhancement (LGE) has recognised patients with extensive LGE (≥15 % LV myocardium) as individuals who may be at increased risk of sudden death, independent of other high-risk features, with implications on management strategies including consideration for primary prevention implantable cardioverter defibrillator therapy. These observations justify an expanded role of CMR in the routine clinical assessment of patients with HCM.
肥厚型心肌病(HCM)是最常见的遗传性心肌病,是一种具有显著异质性的疾病。虽然大多数HCM患者无症状,寿命接近正常,但一小部分但很重要的患者仍面临包括猝死在内的一系列临床结局的风险。心血管磁共振成像(CMR)具有高空间分辨率和断层成像能力,已成为一种特别适合于表征HCM表型表达的成像方式。CMR通过识别超声心动图难以清晰显示的肥厚区域、提供更准确的室壁厚度测量以及将HCM与左心室(LV)肥厚的其他原因区分开来,有助于HCM的诊断。CMR已促使识别出HCM患者的新亚组,包括那些患有左心室心尖部室壁瘤的患者(这是一个室性心律失常和血栓栓塞性中风风险增加的亚组),以及导致左心室流出道梗阻的异常情况。此外,采用延迟钆增强(LGE)的对比增强CMR已识别出广泛LGE(≥15%左心室心肌)的患者为可能猝死风险增加的个体,与其他高危特征无关,这对管理策略有影响,包括考虑一级预防植入式心脏复律除颤器治疗。这些观察结果证明CMR在HCM患者的常规临床评估中应发挥更大作用。