title: The Role of Cardiac MRI in the Diagnosis and Risk Stratification of Hypertrophic Cardiomyopathy.
abstract: 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.
authors.affiliation: Hypertrophic Cardiomyopathy Institute, Division of Cardiology, Tufts Medical Center, Boston, MA; Chanin T. Mast Center for Hypertrophic Cardiomyopathy, Morristown Medical Center, Morristown, NJ, USA.
authors.forename: Ethan J
authors.initials: EJ
authors.lastname: Rowin
authors.affiliation: Hypertrophic Cardiomyopathy Institute, Division of Cardiology, Tufts Medical Center, Boston, MA; Chanin T. Mast Center for Hypertrophic Cardiomyopathy, Morristown Medical Center, Morristown, NJ, USA.
authors.forename: Martin S
authors.initials: MS
authors.lastname: Maron
keywords: cardiovascular magnetic resonance; hypertrophic cardiomyopathy; sudden death