title: Hypertrophic cardiomyopathy: part 1 - introduction, pathology and pathophysiology.
abstract: Hypertrophic cardiomyopathy (HCM) is the most common genetic cardiovascular disease with many genotype and phenotype variations. Earlier terminologies, hypertrophic obstructive cardiomyopathy and idiopathic hypertrophic sub-aortic stenosis are no longer used to describe this entity. Patients present with or without left ventricular outflow tract (LVOT) obstruction. Resting or provocative LVOT obstruction occurs in 70% of patients and is the most common cause of heart failure. The pathology and pathophysiology of HCM includes hypertrophy of the left ventricle with or without right ventricular hypertrophy, systolic anterior motion of mitral valve, dynamic and mechanical LVOT obstruction, mitral regurgitation, diastolic dysfunction, myocardial ischemia, and fibrosis. Thorough understanding of pathology and pathophysiology is important for anesthetic and surgical management.
authors.affiliation: Department of Cardiac Surgery, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, Kerala, India.
authors.forename: Praveen Kerala
authors.initials: PK
authors.lastname: Varma
authors.forename: Praveen Kumar
authors.initials: PK
authors.lastname: Neema
mesh_terms: D002312:Cardiomyopathy, Hypertrophic / Q000235:genetics / Q000473:pathology* / Q000503:physiopathology* / Q000628:therapy; D006801:Humans; D014694:Ventricular Outflow Obstruction / Q000209:etiology