主動脈疾病英文

1、主動脈疾病-英文-精品文檔主動脈疾病-英文-精品文檔Categories of aortic diseaseAneurysmPseudoaneurysmDissectionPenetrating atherosclerotic ulcerIntramural hematomaAtherosclerotic diseaseCoarctationCategories of aortic diseaseAn主動脈疾病-英文-精品文檔Thoracic aortic aneurysmsAortopathy due to heritable diseases (20% of aneurysms)Marfa

2、n syndrome Ehlers-Danlos syndromeFamilial Annuloaortic ectasia Turner syndrome Noonan syndrome Osteogenesis imperfecta Bicuspid aortic valve Coarctation of the aorta Metabolic disorders (eg, homocystinuria, familial hypercholesterolemia)Aortic arteritis (Takayasus, giant-cell)TraumaPregnancySyphilis

3、/infectiousCrack cocaine useIatrogenic causes (eg, cardiac catheterization)AtherosclerosisHypertensionThoracic aortic aneurysmsAortoMarfan SyndromeAutosomal dominant inheritance Missense mutation in the fibrillin gene on chromosome 15Prevalence is 1 in 10,000 (25% sporadic)Leading cause of premature

4、 death is aortic root aneurysm leading to dissection (cystic medial degeneration)Marfan SyndromeAutosomal dominMarfan SyndromeGhent CriteriaIndex caseMajor criteria in 2 different organ systems and involvement in a third organ systemFamily historyMajor criterion in family/genetics and 1 major criter

5、ion in an organ system and involvement in a second organ systemMarfan SyndromeGhent CriteriaMarfan SyndromeSkeletalMajor (at least 4 items)Pectus carinatumPectus excavatum requiring surgeryDecreased upper to lower segment ratio OR increased arm spam to height ratioWrist and thumb signsScoliosis 20 d

6、egreesDecreased elbow extensionPes planusProtrusio acetabulaeMinorPectus excavatum (moderate)Joint hypermobilityHigh arched palate with crowding of teethFacial appearanceOcularMajorEctopia lentisMinorAbnormally flat corneaIncreased axial length of globeHypoplastic iris or ciliary muscleSkin and inte

7、gumentMinor onlyStriae not associated with weight change, pregnancy or stressRecurrent or incisional herniasPulmonaryMinor onlySpontaneous pneumothoraxApical blebsDuraMajor onlyLumbosacral dural ectasiaCardiovascularMajorDilatation of the ascending aorta involving at least the sinuses of ValsalvaDis

8、section of the ascending aortaMinorMitral valve prolapse with or without MRMitral annular calcification before age 40Dilation or dissection of descending aorta before age 50Dilation of the PA without cause before age 40Familial/geneticMajor onlyFirst degree relative who meets criteria independentlyP

9、resence of mutation in FBN1 known to cause MFSPresence of haplotype around FBN1, inherited by descent, known to be associated with unequivocally diagnosed MFS in familyMarfan SyndromeSkeletalPulmonaMarfan SyndromeSkeletalMajor (at least 4 items)Pectus carinatumPectus excavatum requiring surgeryDecre

10、ased upper to lower segment ratio OR increased arm spam to height ratioWrist and thumb signsScoliosis 20 degreesDecreased elbow extensionPes planusProtrusio acetabulaeMinorPectus excavatum (moderate)Joint hypermobilityHigh arched palate with crowding of teethFacial appearanceOcularMajorEctopia lenti

11、sMinorAbnormally flat corneaIncreased axial length of globeHypoplastic iris or ciliary muscleSkin and integumentMinor onlyStriae not associated with weight change, pregnancy or stressRecurrent or incisional herniasPulmonaryMinor onlySpontaneous pneumothoraxApical blebsDuraMajor onlyLumbosacral dural

12、 ectasiaCardiovascularMajorDilatation of the ascending aorta involving at least the sinuses of ValsalvaDissection of the ascending aortaMinorMitral valve prolapse with or without MRMitral annular calcification before age 40Dilation or dissection of descending aorta before age 50Dilation of the PA wi

13、thout cause before age 40Familial/geneticMajor onlyFirst degree relative who meets criteria independentlyPresence of mutation in FBN1 known to cause MFSPresence of haplotype around FBN1, inherited by descent, known to be associated with unequivocally diagnosed MFS in familyMarfan SyndromeSkeletalPul

14、monaFamilial TAA SyndromesAt least 19% of patients with thoracic aortic aneurysms (without overt connective tissue disorders) have a family historyTend to present younger than those presenting sporadicallyGenetically heterogeneousFamilial TAA SyndromesAt leastBicuspid aortic valveInitially, ascendin

15、g thoracic aortic aneurysms in BAV thought to be due to poststenotic dilatation75% with cystic medial necrosis on pathology compared with 14% of those with tricuspid aortic valvesBicuspid aortic valveInitiallyBicuspid aortic valveNistri et al demonstrated 52% incidence of aortic dilatation in patien

16、ts with normally functioning bicuspid aortic valveBicuspid aortic valveNistri etSyphilitic (luetic) aortitisLatent period from initial spirochetal infection averages 10-30 years (range 5 to 40 years)Due to direct infection of aortic media leading to an obliterative endarteritis of the vasa vasorum a

17、nd destruction of collagen and elastic tissues (“tree barking”)Syphilitic (luetic) aortitisLaTurner SyndromeAssociated with bicuspid aortic valve (30%) and coarctation.42% of screened patients by one study with aortic root dilatationAortic root dilatation can be seen with or without BAV, but when bo

18、th are present risk of dissection is probably compoundedScreening recommended every 5yrsTurner SyndromeAssociated withAortic arteritisTakayasus arteritisTypically causes obliterative luminal changes15% of patients may have aortic dilatation/aneurysmsGiant-cell arteritisUp to 20% can have thoracic or

19、 abdominal aortic aneurysmsAortic arteritisTakayasus artThoracic aortic aneurysmsSymptoms:Majority are asymptomatic at diagnosisChest and/or back painHoarsenessCough, dyspnea, stridorDysphagiaSigns:Distended neck vein(s)Aortic regurgitationFixed vocal cordSystemic and/or cerebral embolismThoracic ao

20、rtic aneurysmsSymptThoracic aortic aneurysmsDiagnosisChest X-ray (poor sensitivity)Widened mediastinumEnlarged aortic knobTracheal deviationCT/MR angiographyAccurate detection and sizing of thoracic aortic aneurysmsMR may be preferably for involvement of aortic rootTTE/TEETTE generally not recommend

21、ed for diagnosing and sizing thoracic aortic aneurysms except in patients with Marfan syndromeInvasive nature of TEE prevents it from being imaging modality of choiceThoracic aortic aneurysmsDiagnThoracic aortic aneurysmsNatural historyLongitudinal data suggests mean increase of 0.1-0.25 cm/yrGreate

22、r for descending vs. ascendingGreater for dissected vs. nondissectedGreater for Marfan vs. non-MarfanCritical sizes (initial aneurysm size)Ascending: 6.0 cm; 31% risk of ruptureDescending: 7.0 cm; 43% risk of ruptureYearly event rate (6.0 cm): 14.1%Davies et al. Ann Thorac Surg 2019; 73:17-28.Thorac

23、ic aortic aneurysmsNaturThoracic aortic aneurysmsSurgical managementAscending thoracic aortic aneurysms: 5.5cmThose with increased operative risk: 6cmMarfan/BAV: 5cm or less (prophylactic replacement with aortic valve surgery if 4cm or greater)Descending thoracic aortic aneurysms: 6cmSymptoms; rapid

24、 enlargementThoracic aortic aneurysmsSurgiThoracic aortic aneurysmsMedical managementBlood pressureBeta-blockerIn randomized study of adult with Marfan syndrome, propranolol over 10 years resulted in slower rate of aortic dilatation, fewer aortic events, and lower mortalityOther antihypertensivesTar

25、get SBP 120 mmHg or lessThoracic aortic aneurysmsMedic70 adolescents and young adults with MFSPropranolol vs placeboSome nonrespondersNEJM 1994;330:1335-134170 adolescents and young adultThoracic aortic aneurysmsMedical managementAnti-oxidantsSome studies suggest benefit of adding statins and/or ang

26、iotensin II type I receptor blockers (no clinical trials)Exercise limitationsNo strenuous isometric exertionAerobic exercise generally safe provided there is no hypertensive response to exercise (180 mmHg)Discontinue tobaccoThoracic aortic aneurysmsMedicThoracic aortic aneurysmsSerial ImagingAll pat

27、ients should get at least one baseline imaging study that includes the abdominal aortaRepeat imaging 6 months after initial diagnosisDepending on rate of change, subsequent studies can be performed at 3, 6 or 12 month intervalsThoracic aortic aneurysmsSeriaAbdominal aortic aneurysmsAbdominal aortic

28、aneurysmsAbdominal aortic aneurysmsVA 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 olderIt is estimated that only 50% of AAAs are currently diagnosedHigh mortality 65-85% for ruptured abdominal aortic aneurysmsRu

29、pture of abdominal aortic aneurysms causes 15,000 deaths per year in the USLederle et al, Arch Intern Med 2000; 160:1425-30.Abdominal aortic aneurysmsVA CAbdominal aortic aneurysmsRisk factorORSmoking history5.17Family history1.9Increased age, per 7 years1.7Established atherosclerosis1.6Black race0.

30、59Diabetes mellitus0.50Female gender0.18Risk factors for presence of abdominal aortic aneurysmLederle et al, Arch Intern Med 2000; 160:1425-30.Abdominal aortic aneurysmsRiskAbdominal aortic aneurysmAtherosclerosis breakdown of collagen, elastinSmoking, hypertension, hyperlipidemiaConnective tissue d

31、isordersMarfan SyndromeEhlers-Danlos syndromeVascular inflammationAortic arteritis (Takayasus, giant-cell)BehcetsTuberculosisBrucellosis, salmonellosisTraumaIatrogenic causes (eg, cardiac catheterization or previous surgery)Abdominal aortic aneurysmAtherAbdominal aortic aneurysmsUnrupturedUsually as

32、ymptomatic, diagnosed incidentallyAwareness of heartbeat in abdomenChronic vague abdominal/back painDistal embolisationUerterohydronephrosisRupturedComplaint of painUsually sudden onsetOften in abdomen, but also lower back or flankMay radiate to buttocks, groin, testicles or legMay be constant, thro

33、bbing or colickyShockPulsatile abdominal massSyncope or lightheadednessAbdominal aortic aneurysmsUnruAbdominal aortic aneurysmsPhysical exam Palpation of supraumbilical area for pulsatile mass3.0-3.9cmSens 61%4.0-4.9cmSens 69%5.0cmSens 82%Imaging (62% of cases found incidentally)Ultrasound should we

34、 be screening?CT/CT angioMRI/MRAContrast angiographyAbdominal aortic aneurysmsPhysAbdominal aortic aneurysms9.4%10.2%32.5%n=52n=85n=61JAMA 2019; 287:2968Natural historyAbdominal aortic aneurysms9.4%Abdominal aortic aneurysmsSurgical indications:Rupture or impending rupture (90% mortality)In patients

35、 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)Class IPatients with infrarenal or juxtarenal AAAs measuring 5.5 cm or larger should undergo repair to eliminate the risk of rupture.

36、 (Level of Evidence: B)In patients with symptomatic (e.g. embolization, ureteral compresion) aortic aneurysms, repair is indicated regardless of diameter. (Level of Evidence: C)Abdominal aortic aneurysmsSurgAbdominal aortic aneurysmsSurgical indications:Class IIa:Repair can be beneficial in patients

37、 with infrarenal or juxtarenal AAAs 5.0 to 5.4 cm in diameter (Level of Evidence: B)Repair 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)Class III:Intervention is not recommended for asymptomatic infrar

38、enal 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)Not mentioned:Rapid expansion (1.0 cm in 12 months)Inflammatory of infectious etiologyAbdominal aortic aneurysmsSurgAbdominal aortic aneurysmsEndovascular repai

39、r:Eliminates need for major transabdominal surgeryCan be performed under regional or local anesthesiaMay be useful in setting of severe cardiopulmonary disease, advanced age, morbid obesity or history of multiple abdominal surgeriesAbdominal aortic aneurysmsEndoAbdominal aortic aneurysmsEndovascular

40、 repair:Patients need adequate length of relatively noral aorta below the renal arteries because of high risk for proximal attachment failure, graft migration, and endoleakBecause of inflexibility of these grafts, the segment of aorta can not be severely angulated.Abdominal aortic aneurysmsEndoAbdom

41、inal aortic aneurysmsEndograft indications:Class I:Open repair of infrarenal AAAs and/or common iliac aneurysms is indicated in patients who are good or average surgical candidates (Level of Evidence: B)Periodic long-term surveillance imaging should be performed to monitor for an endoleak, to docume

42、nt 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 aneurysmsEndoAbdominal aortic aneurysmsEndograft indication

43、s:Class IIa:Endovascular 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)Class IIb:Endovascular repair of infrarenal aortic and/or com

44、mon iliac aneurysms may be considered in patients at low or average surgical risk. (Level of Evidence: B)Abdominal aortic aneurysmsEndoAbdominal aortic aneurysmsMedical therapy:Class IIn patients with AAAs, blood pressure and fasting serum lipid values should be monitored and controlled as recommend

45、ed for patients with atherosclerotic disease. (Level of Evidence: C)Patients 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)Ab

46、dominal aortic aneurysmsMediAbdominal aortic aneurysmsMedical therapy:Class IPerioperative 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 undergoi

47、ng surgical repair of atherosclerotic aortic aneurysms. (Level of Evidence: A)Class IIbBeta-adrenergic blocking agents may be considered to reduce the rate of aneurysm expansion in patients with aortic aneurysms. (Level of Evidence: B)Abdominal aortic aneurysmsMediAbdominal aortic aneurysmsSurveilla

48、nce:Class IPatients 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)Class IIaIn patients with AAAs smaller than 4.0 cm in diameter, monitoring by ultrasound examinatio

49、n every 2 to 3 years is reasonable. (Level of Evidence: B)Abdominal aortic aneurysmsSurvAbdominal aortic aneurysmsScreening:Clinical examination lacks sensitivityEarly detection reduces mortalityClass IMen 60 years of age or older who are either the siblings or offspring of patients with AAAs should

50、 undergo physical examination and ultrasound screening for detection of aortic aneurysms. (Level of Evidence: B)Class IIaMen 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

51、 aortic aneurysmsScreAbdominal aortic aneurysmsHypertension and other cardiovascular risk factors should be treated effectively discontinue tobaccoTreat associated coronary and carotid diseaseAneurysm with diameter over 3cm should be monitored every 12 months. When 5cm in man or 4.5cm in woman, shou

52、ld be followed every 6 monthsSurgery when aneurysm exceeds 5.5cm (UK Small Aneurysm Trial, NEJM 2019; 348:1895-1901)About 1% of aneurysms with diameter of 4cm rupture annually compared with 10% of aneurysms 6cm or more. Mortality with rupture is 90%Elderly brothers of patients with known aneurysms s

53、hould be screened (Salo, Ann intern med 2019; 130:637-42.Abdominal aortic aneurysmsHypeInflammatory type2-4% of all AAAClassic triad of abdominal/back pain, weight loss, and elevated ESRObstructive uropathyDiagnosis: ultrasound, CT, MRIUnusually thickened aneurysm wallShiny white perianeurysmal fibr

54、osisIntense adherence to adjacent intraabdominal structuresAccumulation of macrophages and cytokines in aneurysmal aortic tissueTreatment surgical grafting regardless of sizeRole of corticosteroids?Inflammatory typeAcute aortic syndromesAortic dissectionPenetrating aortic ulcerIntramural hematomaAcu

55、te aortic syndromesAortic dAcute aortic syndromesAortic dissection 83-95%Penetrating aortic ulcerIntramural hematoma5-17%Acute aortic syndromesAortic dAcute aortic syndromes9/11/03 6:10pmAfter collapsing at work, a 54yo male is brought to ED with complaints of chest pain, nausea, vomiting and dizzin

56、ess9/11/03 8:00pmInitially diagnosed with MI, patient is brought to cath lab. There a “l(fā)arge ascending aortic aneurysm with severe dissection” is found.9/11/03 10:48pmWhile in the operating room, patient arrests and can not be resuscitated.Acute aortic syndromes9/11/03 Acute aortic syndromes9/3/04Fa

57、mily 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.Claimed damages are in excess of $25 million3/16/06Parties reach settlement for “a large monetary settlement” of

58、an undisclosed amount.Acute aortic syndromes9/3/04Aortic dissectionCommon fatal aortic conditionHigh mortalityWith medical therapy, 1% die per hour in the first 24 hours50% die in first 2 weeksCorrect clinical diagnosis in 15-43% of casesDiagnostic delay of 24 hours in up to 39% of casesKlompas et a

59、l, JAMA 2019; 287:2262-2272.Aortic dissectionCommon fatal Aortic dissectionClinical presentationSudden severe pain - 90%Migrating pain 31%Tearing pain 39% (spec. 95%)No pain 4.5%Hypertension 49%Diastolic murmur 28%Pulse deficits or BP differential 31%Focal neurologic deficits 17%Syncope 13%ECG crite

60、ria for AMI 7%There is no disease more conducive to clinical humility than aneurysm of the aorta. Sir William Osler, c.1900 Klompas et al, JAMA 2019; 287:2262-2272.Nallamothy et al, Am J Med 2019; 113:468-471.Aortic dissectionThere is no dAortic dissectionLong-standing hypertensionSmoking, dyslipide