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Diseases of the Aorta Fred Wu, M.D. April 19, 2006 Categories of aortic disease nAneurysm nPseudoaneurysm nDissection nPenetrating atherosclerotic ulcer nIntramural hematoma nAtherosclerotic disease nCoarctation Thoracic aortic aneurysms nAortopathy due to heritable diseases (20% of aneurysms) nMarfan syndrome nEhlers-Danlos syndrome nFamilial Annuloaortic ectasia nTurner syndrome nNoonan syndrome nOsteogenesis imperfecta nBicuspid aortic valve nCoarctation of the aorta nMetabolic disorders (eg, homocystinuria, familial hypercholesterolemia) nAortic arteritis (Takayasus, giant-cell) nTrauma nPregnancy nSyphilis/infectious nCrack cocaine use nIatrogenic causes (eg, cardiac catheterization) nAtherosclerosis nHypertension Marfan Syndrome nAutosomal dominant inheritance nMissense mutation in the fibrillin gene on chromosome 15 nPrevalence is 1 in 10,000 (25% sporadic) nLeading cause of premature death is aortic root aneurysm leading to dissection (cystic medial degeneration) Marfan Syndrome nGhent Criteria nIndex case nMajor criteria in 2 different organ systems and involvement in a third organ system nFamily history nMajor criterion in family/genetics and 1 major criterion in an organ system and involvement in a second organ system Marfan Syndrome nSkeletal nMajor (at least 4 items) nPectus carinatum nPectus excavatum requiring surgery nDecreased upper to lower segment ratio OR increased arm spam to height ratio nWrist and thumb signs nScoliosis 20 degrees nDecreased elbow extension nPes planus nProtrusio acetabulae nMinor nPectus excavatum (moderate) nJoint hypermobility nHigh arched palate with crowding of teeth nFacial appearance nOcular nMajor nEctopia lentis nMinor nAbnormally flat cornea nIncreased axial length of globe nHypoplastic iris or ciliary muscle nSkin and integument nMinor only nStriae not associated with weight change, pregnancy or stress nRecurrent or incisional hernias nPulmonary nMinor only nSpontaneous pneumothorax nApical blebs nDura nMajor only nLumbosacral dural ectasia nCardiovascular nMajor nDilatation of the ascending aorta involving at least the sinuses of Valsalva nDissection of the ascending aorta nMinor nMitral valve prolapse with or without MR nMitral annular calcification before age 40 nDilation or dissection of descending aorta before age 50 nDilation of the PA without cause before age 40 nFamilial/genetic nMajor only nFirst degree relative who meets criteria independently nPresence of mutation in FBN1 known to cause MFS nPresence of haplotype around FBN1, inherited by descent, known to be associated with unequivocally diagnosed MFS in family Marfan Syndrome nSkeletal nMajor (at least 4 items) nPectus carinatum nPectus excavatum requiring surgery nDecreased upper to lower segment ratio OR increased arm spam to height ratio nWrist and thumb signs nScoliosis 20 degrees nDecreased elbow extension nPes planus nProtrusio acetabulae nMinor nPectus excavatum (moderate) nJoint hypermobility nHigh arched palate with crowding of teeth nFacial appearance nOcular nMajor nEctopia lentis nMinor nAbnormally flat cornea nIncreased axial length of globe nHypoplastic iris or ciliary muscle nSkin and integument nMinor only nStriae not associated with weight change, pregnancy or stress nRecurrent or incisional hernias nPulmonary nMinor only nSpontaneous pneumothorax nApical blebs nDura nMajor only nLumbosacral dural ectasia nCardiovascular nMajor nDilatation of the ascending aorta involving at least the sinuses of Valsalva nDissection of the ascending aorta nMinor nMitral valve prolapse with or without MR nMitral annular calcification before age 40 nDilation or dissection of descending aorta before age 50 nDilation of the PA without cause before age 40 nFamilial/genetic nMajor only nFirst degree relative who meets criteria independently nPresence of mutation in FBN1 known to cause MFS nPresence of haplotype around FBN1, inherited by descent, known to be associated with unequivocally diagnosed MFS in family Familial TAA Syndromes nAt least 19% of patients with thoracic aortic aneurysms (without overt connective tissue disorders) have a family history nTend to present younger than those presenting sporadically nGenetically heterogeneous Bicuspid aortic valve nInitially, ascending thoracic aortic aneurysms in BAV thought to be due to poststenotic dilatation n75% with cystic medial necrosis on pathology compared with 14% of those with tricuspid aortic valves Bicuspid aortic valve nNistri et al demonstrated 52% incidence of aortic dilatation in patients with normally functioning bicuspid aortic valve Syphilitic (luetic) aortitis nLatent period from initial spirochetal infection averages 10-30 years (range 5 to 40 years) nDue to direct infection of aortic media leading to an obliterative endarteritis of the vasa vasorum and destruction of collagen and elastic tissues (“tree barking”) Turner Syndrome nAssociated with bicuspid aortic valve (30%) and coarctation. n42% of screened patients by one study with aortic root dilatation nAortic root dilatation can be seen with or without BAV, but when both are present risk of dissection is probably compounded nScreening recommended every 5yrs Aortic arteritis nTakayasus arteritis nTypically causes obliterative luminal changes n15% of patients may have aortic dilatation/aneurysms nGiant-cell arteritis nUp to 20% can have thoracic or abdominal aortic aneurysms Thoracic aortic aneurysms nSymptoms: nMajority are asymptomatic at diagnosis nChest and/or back pain nHoarseness nCough, dyspnea, stridor nDysphagia nSigns: nDistended neck vein(s) nAortic regurgitation nFixed vocal cord nSystemic and/or cerebral embolism Thoracic aortic aneurysms nDiagnosis nChest X-ray (poor sensitivity) nWidened mediastinum nEnlarged aortic knob nTracheal deviation nCT/MR angiography nAccurate detection and sizing of thoracic aortic aneurysms nMR may be preferably for involvement of aortic root nTTE/TEE nTTE generally not recommended for diagnosing and sizing thoracic aortic aneurysms except in patients with Marfan syndrome nInvasive nature of TEE prevents it from being imaging modality of choice Thoracic aortic aneurysms nNatural history nLongitudinal data suggests mean increase of 0.1-0.25 cm/yr nGreater for descending vs. ascending nGreater for dissected vs. nondissected nGreater for Marfan vs. non-Marfan nCritical sizes (initial aneurysm size) nAscending: 6.0 cm; 31% risk of rupture nDescending: 7.0 cm; 43% risk of rupture nYearly event rate (6.0 cm): 14.1% Davies et al. Ann Thorac Surg 2002; 73:17-28. Thoracic aortic aneurysms nSurgical management nAscending thoracic aortic aneurysms: 5.5cm nThose with increased operative risk: 6cm nMarfan/BAV: 5cm or less (prophylactic replacement with aortic valve surgery if 4cm or greater) nDescending thoracic aortic aneurysms: 6cm nSymptoms; rapid enlargement Thoracic aortic aneurysms nMedical management nBlood pressure nBeta-blocker nIn randomized study of adult with Marfan syndrome, propranolol over 10 years resulted in slower rate of aortic dilatation, fewer aortic events, and lower mortality nOther antihypertensives nTarget SBP 120 mmHg or less n70 adolescents and young adults with MFS nPropranolol vs placebo nSome nonresponders NEJM 1994;330:1335-1341 Thoracic aortic aneurysms nMedical management nAnti-oxidants nSome studies suggest benefit of adding statins and/or angiotensin II type I receptor blockers (no clinical trials) nExercise limitations nNo strenuous isometric exertion nAerobic exercise generally safe provided there is no hypertensive response to exercise (180 mmHg) nDiscontinue tobacco Thoracic aortic aneurysms nSerial Imaging nAll patients should get at least one baseline imaging study that includes the abdominal aorta nRepeat imaging 6 months after initial diagnosis nDepending on rate of change, subsequent studies can be performed at 3, 6 or 12 month intervals Abdominal aortic aneurysms Abdominal aortic aneurysms nVA Cooperative Study found a 1.3% incidence of abdominal aortic aneurysms larger than 4.0 cm on screening of 125,000 patients age 50 or older nIt is estimated that only 50% of AAAs are currently diagnosed nHigh mortality 65-85% for ruptured abdominal aortic aneurysms nRupture of abdominal aortic aneurysms causes 15,000 deaths per year in the US Lederle et al, Arch Intern Med 2000; 160:1425-30. Abdominal aortic aneurysms Risk factorOR Smoking history5.17 Family history1.9 Increased age, per 7 years1.7 Established atherosclerosis1.6 Black race0.59 Diabetes mellitus0.50 Female gender0.18 Risk factors for presence of abdominal aortic aneurysm Lederle et al, Arch Intern Med 2000; 160:1425-30. Abdominal aortic aneurysm nAtherosclerosis breakdown of collagen, elastin nSmoking, hypertension, hyperlipidemia nConnective tissue disorders nMarfan Syndrome nEhlers-Danlos syndrome nVascular inflammation nAortic arteritis (Takayasus, giant-cell) nBehcets nTuberculosis nBrucellosis, salmonellosis nTrauma nIatrogenic causes (eg, cardiac catheterization or previous surgery) Abdominal aortic aneurysms nUnruptured nUsually asymptomatic, diagnosed incidentally nAwareness of heartbeat in abdomen nChronic vague abdominal/back pain nDistal embolisation nUerterohydronephrosis nRuptured nComplaint of pain nUsually sudden onset nOften in abdomen, but also lower back or flank nMay radiate to buttocks, groin, testicles or leg nMay be constant, throbbing or colicky nShock nPulsatile abdominal mass nSyncope or lightheadedness Abdominal aortic aneurysms nPhysical exam Palpation of supraumbilical area for pulsatile mass n3.0-3.9cmSens 61% n4.0-4.9cmSens 69% n5.0cmSens 82% nImaging (62% of cases found incidentally) nUltrasound should we be screening? nCT/CT angio nMRI/MRA nContrast angiography Abdominal aortic aneurysms 9.4% 10.2% 32.5% n=52 n=85 n=61 JAMA 2002; 287:2968JAMA 2002; 287:2968 Natural historyNatural history Abdominal aortic aneurysms nSurgical indications: nRupture or impending rupture (90% mortality) nIn patients with the clinical triad of abdominal and/or back pain, a pulsatile abdominal mass, and hypotension, immediate surgical evaluation is indicated. (Level of Evidence: B) nClass I nPatients with infrarenal or juxtarenal AAAs measuring 5.5 cm or larger should undergo repair to eliminate the risk of rupture. (Level of Evidence: B) nIn patients with symptomatic (e.g. embolization, ureteral compresion) aortic aneurysms, repair is indicated regardless of diameter. (Level of Evidence: C) Abdominal aortic aneurysms nSurgical indications: nClass IIa: nRepair can be beneficial in patients with infrarenal or juxtarenal AAAs 5.0 to 5.4 cm in diameter (Level of Evidence: B) nRepair is probably indicated in patients with suprarenal or type IV thoracoabdominal aortic aneurysms larger than 5.5 to 6.0 cm. (Level of Evidence: B) nClass III: nIntervention is not recommended for asymptomatic infrarenal or juxtarenal AAAs if they measure less than 5.0 cm in diameter in men or less than 4.5 cm in diameter in women. (Level of Evidence: A) nNot mentioned: nRapid expansion (1.0 cm in 12 months) nInflammatory of infectious etiology Abdominal aortic aneurysms nEndovascular repair: nEliminates need for major transabdominal surgery nCan be performed under regional or local anesthesia nMay be useful in setting of severe cardiopulmonary disease, advanced age, morbid obesity or history of multiple abdominal surgeries Abdominal aortic aneurysms nEndovascular repair: nPatients need adequate length of relatively noral aorta below the renal arteries because of high risk for proximal attachment failure, graft migration, and endoleak nBecause of inflexibility of these grafts, the segment of aorta can not be severely angulated. Abdominal aortic aneurysms nEndograft indications: nClass I: nOpen repair of infrarenal AAAs and/or common iliac aneurysms is indicated in patients who are good or average surgical candidates (Level of Evidence: B) nPeriodic long-term surveillance imaging should be performed to monitor for an endoleak, to document shrinkage or stability of the excluded aneurysm sac, and to determine the need for further intervention in patients who have undergone endovascular repair of intrarenal aortic and/or iliac aneurysms (Level of Evidence: B) Abdominal aortic aneurysms nEndograft indications: nClass IIa: nEndovascular repair in infrarenal aortic and/or common iliac aneurysms is reasonable in patients at high risk of complications from open operations because of cardiopulmonary or other associated diseases. (Level of Evidence: B) nClass IIb: nEndovascular repair of infrarenal aortic and/or common iliac aneurysms may be considered in patients at low or average surgical risk. (Level of Evidence: B) Abdominal aortic aneurysms nMedical therapy: nClass I nIn patients with AAAs, blood pressure and fasting serum lipid values should be monitored and controlled as recommended for patients with atherosclerotic disease. (Level of Evidence: C) nPatients with aneurysms or a family history of aneurysms should be advised to stop smoking and be offered smoking cessation interventions, including behavior modification, nicotine replacement, or bupropion. (Level of Evidence: B) Abdominal aortic aneurysms nMedical therapy: nClass I nPerioperative administration of beta-adrenergic blocking agents, in the absence of contraindications, is indicated to reduce the risk of adverse cardiac events and mortality in patients with coronary artery disease undergoing surgical repair of atherosclerotic aortic aneurysms. (Level of Evidence: A) nClass IIb nBeta-adrenergic blocking agents may be considered to reduce the rate of aneurysm expansion in patients with aortic aneurysms. (Level of Evidence: B) Abdominal aortic aneurysms nSurveillance: nClass I nPatients with infrarenal or juxtarenal AAAs measuring 4.0 to 5.4 cm in diameter should be monitored by ultrasound or CT scans every 6 to 12 months to detect expansion. (Level of Evidence: A) nClass IIa nIn patients with AAAs smaller than 4.0 cm in diameter, monitoring by ultrasound examination every 2 to 3 years is reasonable. (Level of Evidence: B) Abdominal aortic aneurysms nScreening: nClinical examination lacks sensitivity nEarly detection reduces mortality nClass I nMen 60 years of age or older who are either the siblings or offspring of patients with AAAs should undergo physical examination and ultrasound screening for detection of aortic aneurysms. (Level of Evidence: B) nClass IIa nMen who are 65 to 75 years of age who have ever smoked should undergo a physical examination and 1- time ultrasound screening for detection of AAAs. (Level of Evidence: B) Abdominal aortic aneurysms nHypertension and other cardiovascular risk factors should be treated effectively discontinue tobacco nTreat associated coronary and carotid disease nAneurysm with diameter over 3cm should be monitored every 12 months. When 5cm in man or 4.5cm in woman, should be followed every 6 months nSurgery when aneurysm exceeds 5.5cm (UK Small Aneurysm Trial, NEJM 2003; 348:1895-1901) nAbout 1% of aneurysms with diameter of 4cm rupture annually compared with 10% of aneurysms 6cm or more. Mortality with rupture is 90% nElderly brothers of patients with known aneurysms should be screened (Salo, Ann intern med 1999; 130:637-42. nInflammatory type n2-4% of all AAA nClassic triad of abdominal/back pain, weight loss, and elevated ESR nObstructive uropathy nDiagnosis: ultrasound, CT, MRI nUnusually thickened aneurysm wall nShiny white perianeurysmal fibrosis nIntense adherence to adjacent intraabdominal structures nAccumulation of macrophages and cytokines in aneurysmal aortic tissue nTreatment surgical grafting regardless of size nRole of corticosteroids? Acute aortic syndromes nAortic dissection nPenetrating aortic ulcer nIntramural hematoma Acute aortic syndromes nAortic dissection 83-95% nPenetrating aortic ulcer nIntramural hematoma 5-17%5-17% Acute aortic syndromes n9/11/03 6:10pm nAfter collapsing at work, a 54yo male is brought to ED with complaints of chest pain, nausea, vomiting and dizziness n9/11/03 8:00pm nInitially diagnosed with MI, patient is brought to cath lab. There a “large ascending aortic aneurysm with severe dissection” is found. n9/11/03 10:48pm nWhile in the operating room, patient arrests and can not be resuscitated. Acute aortic syndromes n9/3/04 nFamily files lawsuit accusing hospital of negligence, claiming that the patient was misdiagnosed “at least twice and underwent improper and unnecessary procedures” during his hospital stay. nClaimed damages are in excess of $25 million n3/16/06 nParties reach settlement for “a large monetary settlement” of an undisclosed amount. Aortic dissection nCommon fatal aortic condition nHigh mortality nWith medical therapy, 1% die per hour in the first 24 hours n50% die in first 2 weeks nCorrect clinical diagnosis in 15-43% of cases nDiagnostic delay of 95%, spec87% (Class I) nMay be more accurate than MRI or TEE in detection of arch vessel involvement nMRI/MRA nHighest sensitivity and specificity (almost 100%) nOnly practical in stable patients (Class IIa) nTTE/TEE nPreferred modality in unstable patients (Class I) nSens 99%, spec 89% nAortography (rare) (Class IIa) nSens 90%, spec 95% Eur Heart J 2001; 22(18):1642-81. Aortic dissection Aortic dissection JAMA 2000; 283:897-903. Aortic dissection nAcute Type A (DeBakey Type I or II) nSurgical emergency aim to avoid tamponade or rupture (Class I) nImplantation of a composite graft with or without reimplantation of coronary arteries nRestoration of aortic valve competence in patients with AI (resuspension vs. replacement) nWithout surgery, 20% mortality by 24 hours and 30% by 48 hours nOperative mortality varies from 15% to 35% Eur Heart J 2001; 22(18):1642-81. Aortic dissection nAcute Type B (DeBakey Type III) nMedical management - In stable patients, surgery offers no proven benefit (Class I) nInvasive hemodynamic monitoring nBeta-blockade nArterial vasodilators to keep SBP5-6cm nType B (