Septal Defects University of OttawaAV间隔缺损渥太华大学课件.ppt

HCM - diastolic dysfunction ventricular hypertrophy myocyte disarray interstitial fibrosis myocardial microinfarcts Cardiomyopathy - summary Gross and histopathologic findings are non- specific but may be diagnostic Most require clinicopathological correlation Many mimics and secondary diseases Molecular diagnosis / genetics developing Aortic valve - normal Mitral valve - normal Aortic stenosis - causes Aortic stenosis causing LVH Mitral stenosis - rheumatic Floppy mitral valve - mitral valve prolapse (MVP) Hypertension PRIMARY (ESSENTIAL) Genetic and environmental factors Defects in sodium homeostasis, vascular smooth muscle structure, regulation of vascular tone SECONDARY renal disease vascular disease endocrinopathies drugs neurogenic etc… Reno-vascular hypertension Hypertension - complications enhance other diseases (risk factor) small vessel changes scarring/sclerosis microaneurysms large vessel changes ectasia / aneurysms / aortic regurgitation dissection vessel rupture cardiac hypertrophy etc… Arteriolo-nephrosclerosis Brain hypertensive bleed Hypertensive brain stem bleed LVH (look familiar?) Cardiomyopathy - definition Heterogenous group of diseases of the myocardium associated with mechanical or electrical dysfunction that usually (but not invariably) exhibit inappropriate ventricular hypertrophy or dilation and are due to a variety of causes that frequently are genetic. Cardiomyopathies are either confined to the heart or are part of generalized systemic disorders often leading to cardiovascular death or progressive heart failure related disability. Circulation 2006 113:1807-1816 Cardiomyopathy types(clinical/functional/morphologic patterns) Dilated (DCM) – 90% Hypertrophic (HCM) Restrictive Primary cardiomyopathy (confined to the heart) - etiology Genetic e.g. HCM, ARVC, mitochondrial defects, channelopathies (e.g. LQTS) Acquired e.g. due to myocarditis (inflammation of the myocardium) Mixed Idiopathic Secondary cardiomyopat