主动脉疾病-英文精选.pptVIP

Diseases of the Aorta Fred Wu, M.D. April 19, 2006 Categories of aortic disease Aneurysm Pseudoaneurysm Dissection Penetrating atherosclerotic ulcer Intramural hematoma Atherosclerotic disease Coarctation Thoracic aortic aneurysms Aortopathy due to heritable diseases (20% of aneurysms) Marfan syndrome Ehlers-Danlos syndrome Familial Annuloaortic ectasia Turner syndrome Noonan syndrome Osteogenesis imperfecta Bicuspid aortic valve Coarctation of the aorta Metabolic disorders (eg, homocystinuria, familial hypercholesterolemia) Aortic arteritis (Takayasu’s, giant-cell) Trauma Pregnancy Syphilis/infectious Crack cocaine use Iatrogenic causes (eg, cardiac catheterization) Atherosclerosis Hypertension Marfan Syndrome Autosomal dominant inheritance Missense mutation in the fibrillin gene on chromosome 15 Prevalence is 1 in 10,000 (25% sporadic) Leading cause of premature death is aortic root aneurysm leading to dissection (cystic medial degeneration) Marfan Syndrome Ghent Criteria Index case Major criteria in 2 different organ systems and involvement in a third organ system Family history Major criterion in family/genetics and 1 major criterion in an organ system and involvement in a second organ system Marfan Syndrome Skeletal Major (at least 4 items) Pectus carinatum Pectus excavatum requiring surgery Decreased upper to lower segment ratio OR increased arm spam to height ratio Wrist and thumb signs Scoliosis 20 degrees Decreased elbow extension Pes planus Protrusio acetabulae Minor Pectus excavatum (moderate) Joint hypermobility High arched palate with crowding of teeth Facial appearance Ocular Major Ectopia lentis Minor Abnormally flat cornea Increased axial length of globe Hypoplastic iris or ciliary muscle Skin and integument Minor only Striae not associated with weight change, pregnancy or stress Recurrent or incisional hernias Pulmonary Minor only Spontaneous pneumothorax Apical blebs Dura Major only Lumbosacral dural ectasia Cardiovascular Major Dilatation