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The Hypertrophic Cardiomyopathy Phenotype Viewed Through the Prism of Multimodality Imaging: Clinical and Etiologic Implications
Institution:Hypertrophic Cardiomyopathy Center and Research Institute, Tufts Medical Center, Boston, Massachusetts
Abstract:Noninvasive contemporary imaging with echocardiography and cardiovascular magnetic resonance (CMR) provide comprehensive characterization of the hypertrophic cardiomyopathy (HCM) heart including precise definition of left ventricle (LV) wall thickness and reliable identification of morphologic abnormalities of the mitral valve, LV chamber, and myocardial tissue characterization with late gadolinium enhancement (LGE) (fibrosis). Imaging also contributes to identification of patients at risk for sudden death including novel high-risk features such as LV apical aneurysm and extensive LGE. Exercise (stress) echocardiography should be considered to demonstrate physiologic provocation of LV outflow gradients and to distinguish from patients with nonobstructive HCM. Multimodality imaging identifies patients who are optimal candidates for invasive septal reduction therapy and directs preoperative planning for extended myectomy and to optimize alcohol septal ablation. Contemporary imaging interwoven with current management strategies have resulted in a low HCM-related mortality rate.
Keywords:cardiovascular magnetic resonance  echocardiography  heart failure  hypertrophic cardiomyopathy  sudden death  ACC/AHA"}  {"#name":"keyword"  "$":{"id":"kwrd0040"}  "$$":[{"#name":"text"  "_":"American College of Cardiology/American Heart Association  CMR"}  {"#name":"keyword"  "$":{"id":"kwrd0050"}  "$$":[{"#name":"text"  "_":"cardiovascular magnetic resonance  ESC"}  {"#name":"keyword"  "$":{"id":"kwrd0060"}  "$$":[{"#name":"text"  "_":"European Society of Cardiology  GLS"}  {"#name":"keyword"  "$":{"id":"kwrd0070"}  "$$":[{"#name":"text"  "_":"Global longitudinal strain  HCM"}  {"#name":"keyword"  "$":{"id":"kwrd0080"}  "$$":[{"#name":"text"  "_":"hypertrophic cardiomyopathy  ICD"}  {"#name":"keyword"  "$":{"id":"kwrd0090"}  "$$":[{"#name":"text"  "_":"implantable cardioverter-defibrillator  LA"}  {"#name":"keyword"  "$":{"id":"kwrd0100"}  "$$":[{"#name":"text"  "_":"left atrium  LGE"}  {"#name":"keyword"  "$":{"id":"kwrd0110"}  "$$":[{"#name":"text"  "_":"late gadolinium enhancement  LV"}  {"#name":"keyword"  "$":{"id":"kwrd0120"}  "$$":[{"#name":"text"  "_":"left ventricle  LVH"}  {"#name":"keyword"  "$":{"id":"kwrd0130"}  "$$":[{"#name":"text"  "_":"left ventricular hypertrophy  NYHA"}  {"#name":"keyword"  "$":{"id":"kwrd0140"}  "$$":[{"#name":"text"  "_":"New York Heart Association  RV"}  {"#name":"keyword"  "$":{"id":"kwrd0150"}  "$$":[{"#name":"text"  "_":"right ventricle  SAM"}  {"#name":"keyword"  "$":{"id":"kwrd0160"}  "$$":[{"#name":"text"  "_":"systolic anterior motion
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