title: Clinical utility of cardiovascular magnetic resonance in hypertrophic cardiomyopathy.
abstract: 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.
authors.affiliation: Hypertrophic Cardiomyopathy Center, Division of Cardiology, Tufts Medical Center, Boston, MA, USA. mmaron@tuftsmedicalcenter.org
authors.forename: Martin S
authors.initials: MS
authors.lastname: Maron
mesh_terms: D024741:Cardiomyopathy, Hypertrophic, Familial / Q000150:complications / Q000175:diagnosis* / Q000235:genetics / Q000401:mortality / Q000473:pathology / Q000503:physiopathology; D003287:Contrast Media; D016757:Death, Sudden, Cardiac / Q000209:etiology; D018450:Disease Progression; D005355:Fibrosis; D020022:Genetic Predisposition to Disease; D006333:Heart Failure / Q000235:genetics; D006801:Humans; D008279:Magnetic Resonance Imaging; D008943:Mitral Valve / Q000473:pathology; D009206:Myocardium / Q000473:pathology*; D010210:Papillary Muscles / Q000473:pathology; D010641:Phenotype; D011237:Predictive Value of Tests; D011379:Prognosis; D016277:Ventricular Function, Left; D020257:Ventricular Remodeling
chemical_list: D003287:Contrast Media