AAA Clinical presentation• Pain: most common, at hypogastrium or back, not AAA affected by movement• Asymptomatic• Bruit (+/-) Beyond Grey Hair• Rupture triad: and Back Pain ♠ abdominal or back pain; ♠ palpable/ pulsatile abdominal mass; ♠ hypotension (<1/3 cases)
Essential Elements of Aortic Imaging Reports 1. The location at which the aorta is abnormal. 2. The maximum diameter of any dilatation, measured from the external wall of the aorta, perpendicular to the axis of flow, and the length of the aorta that is abnormal. 3. For patients with presumed or documented genetic syndromes at risk for aortic root disease measurements of aortic valve, sinuses of Valsalva, sinotubular junction, and ascending aorta. 4. The presence of internal filling defects consistent with thrombus or atheroma. 5. The presence of intramural hematoma (IMH), penetrating atherosclerotic ulcer (PAU), and calcification. 6. Extension of aortic abnormality into branch vessels, including dissection and aneurysm, and secondary evidence of end-organ injury (eg, renal or bowel hypoperfusion). 7. Evidence of aortic rupture, including periaortic and mediastinal hematoma, pericardial and pleural fluid, and contrast extravasation from the aortic lumen. 8. When a prior examination is available, direct image to image comparison to determine if there has been any increase in diameter.Note: This is Table 5 in the full-text version of the TAD Guideline
AAA Management• Medications : Control of hyperlipidemia, hypertension (β- blockers), cigarette smoking• CT follow up every 3—6 months• Surgical indication: >rupture; >size >5.5 cm; >expanding rapidly (>0.5 cm/year)• Coronary angio (before surgery)
AAA Repair• Two types of repair performed OPEN ENDOVASCULARFirst performed 1951 First performed 1991•Now involves • Less invasive, doneplacement of Dacron or through femoral vesselsPTFE graft • Only certain types of•2-4% operative death AAA can be repairedrate,•5-10% complicationrate
Genetic Syndromes Associated WithThoracic Aortic Aneurysm and Dissection Genetic Common Clinical Features Defect Diagnostic Test Comments on Aortic Disease Syndrome Genetic Marfan Skeletal features (see text); FBN1 Ghent diagnostic Surgical repair when the aorta reaches syndrome Ectopia Lentis; Dural ectasia mutations* criteria 5.0 cm unless there is a family history of AoD at <5.0 cm, a rapidly expanding DNA for aneurysm or presence or significant sequencing aortic valve regurgitation Loeys-Dietz Bifid uvula or cleft palate; TGFBR2 or DNA for Surgical repair recommended at an syndrome Arterial tortuosity; Hypertelorism; TGFBR1 sequencing aortic diameter of ≥4.2 cm by TEE mutations (internal diameter) or 4.4 to ≥4.6 cm by Skeletal features similar to MFS; CT and/or MR (external diameter) Craniosynostosis; Aneurysms and dissections of other arteries Ehlers- Thin, translucent skin; COL3A1 DNA for Surgical repair is complicated by Danlos Gastrointestinal rupture; Rupture of mutations sequencing friable tissues syndrome the gravid uterus; Rupture of Dermal fibroblasts Noninvasive imaging recommended (vascular form) medium-sized to large arteries for analysis of type 3 collagen Turner Short stature; Primary amenorrhea; 45,X karyotype Blood (cells) for AoD risk is increased in patients with syndrome karyotype analysis bicuspid aortic valve, aortic Bicuspid aortic valve; Aortic coarctation, hypertension, or pregnancy coarctation; Webbed neck, low-set ears, low hairline, broad chest* The defective gene at a second locus for MFS is TGFBR2 but the clinical phenotype as MFS is debated.AoD = aortic dissection; COL3A1, type III collagen; FBN1, fibrillin 1; MFS, Marfan syndrome;TGFBR1, transforming growth factor-beta receptor type 1; and TGFBR2, transforming growth factor beta receptor type 2.
Epidemiology• 2-3X more in male• Majority between 50-70 years of age• Relatively rare before 40 y/o except in association with : Marfan’s syndrome, Ehlers-Danlos syndrome, congenital heart disease, familial incidence, pregnancy, coarctation of aorta, Turner’s syndrome and trauma• History of systemic hypertension occur in more than 2/3 of patients
Risk Factors for Development of Thoracic Aortic Dissection Conditions Associated With Increased Aortic Wall Stress • Hypertension, particularly if uncontrolled • Pheochromocytoma • Cocaine or other stimulant use • Weight lifting or other Valsalva maneuver • Trauma • Deceleration or torsional injury (eg, motor vehicle crash, fall) • Coarctation of the aortaNote: Information on this slide is adapted from Table 9 in full-text version of TAD Guidelines
Risk Factors for Development of Thoracic Aortic Dissection (continued)Conditions Associated With Aortic Media Abnormalities Genetic• Marfan syndrome• Ehlers-Danlos syndrome, vascular form• Bicuspid aortic valve (including prior aortic valve replacement)• Turner syndrome• Loeys-Dietz syndrome• Familial thoracic aortic aneurysm and dissection syndrome Note: Information on this slide is adapted from Table 9 in full-text version of TAD Guidelines
Risk Factors for Development of Thoracic Aortic Dissection (continued) Conditions Associated With Aortic Media Abnormalities (continued) Inflammatory vasculitides • Takayasu arteritis • Giant cell arteritis • Behçet arteritis Other • Pregnancy • Autosomal dominant polycystic kidney disease • Chronic corticosteroid or immunosuppression agent administration • Infections involving the aortic wall either from bacteremia or extension of adjacent infectionNote: Information on this slide is adapted from Table 9 in full-text version of TAD Guidelines 2010
Pathophysiology and pathoanatomy• Medial degeneration (cystic medial necrosis is no longer used) : a process of degeneration characterized by loss of smooth muscle cells and elastic tissue that is accompanied by scarring, fibrosis, hyalin-like changes. > mechanism: medial degeneration, repeated flexion of the aorta, and hydrodynamic stresses on the aortic intima, an aortic dissection occurs• 2 important factors determine the continued dissection of aorta: > degree of hypertension and > the steepness (slope) of the pulse wave (dP/dT)
Proximal DistalAcute: less than 2 weeksChronic: more than 2 weeks
Physical Examination– Pulse deficits and discrepancies in BP between limbs are key diagnostic clues– Pulse deficits (50%)– Aortic regurgitation (50%)– Neurologic findings (20%): altered sensorium, hemiplegia, hemianesthesia, gaze preference to the affected side
Estimation of Pretest Risk of Thoracic Aortic Dissection High Risk Conditions • Marfan Syndrome 1 • Connective tissue disease* • Family history of aortic disease • Known aortic valve disease • Recent aortic manipulation (surgical or catheter-based) • Known thoracic aortic aneurysm • Genetic conditions that predispose to AoD†* Loeys-Dietz syndrome, vascular Ehlers-Danlos syndrome, Turner syndrome, or other connective tissue disease.†Patients with mutations in genes known to predispose to thoracic aortic aneurysms and dissection, such as FBN1, TGFBR1, TGFBR2, ACTA2, and MYH11.
Estimation of Pretest Risk of Thoracic Aortic Dissection High Risk Pain Features 2Chest, back, or abdominal painfeatures described as pain that:• is abrupt or instantaneous in onset.• is severe in intensity.• has a ripping, tearing, stabbing, orsharp quality.•Patients are restless, cannot getcomfortable Anterior pain only: 90% ascending Interscapular pain only: 90% descending
Estimation of Pretest Risk of Thoracic Aortic Dissection High Risk Examination 3 Features • Pulse deficit • Systolic BP limb differential > 20mm Hg • Focal neurologic deficit • Murmur of aortic regurgitation (new or not known to be old and in conjunction with pain)
Risk-based Diagnostic Evaluation: Patients with Low Risk of TADPatients with no high-risk features of TAD present are considered atlow risk for TAD. The following clinical steps are recommended for low-riskTAD patients: Expedited aortic imaging Proceed with diagnostic • TEE (preferred if clinically unstable) evaluation as clinically • CT scan (image entire aorta: chest to pelvis) indicated by presentation. • MR (image entire aorta: chest to pelvis) Yes Yes Alternative diagnosis No Unexplained identified? hypotension or widened Yes mediastinum on CXR? No Initiate appropriate Therapy. Consider aortic imaging study for TAD based on clinical scenario (particularly in patients with advanced age, risk factors for aortic disease, or syncope)
Risk-based Diagnostic Evaluation: Patients with Intermediate Risk of TADThe following steps for patients with intermediate risk of TAD should be followed when any single high-risk feature is present. EKG consistent Likely primary ACS. In absence of other Yes perfusion deficits, strongly consider with STEMI? immediate coronary re-perfusion therapy. If No PTCA performed, is culprit lesion identified? CXR with clear Yes Alternate diagnosis? Initiate appropriate therapy. No Yes History and physical exam strongly Alternate diagnosis suggestive of specific alternate diagnosis? confirmed by further testing? No Expedited aortic imaging No • TEE (preferred if clinically unstable) • CT scan (image entire aorta: chest to pelvis) • MR (image entire aorta: chest to pelvis)
Risk-based Diagnostic Evaluation: Patients with High Risk of TADPatients at high-risk for TAD are those that present with at least 2 high-risk features.The recommended course of action for high-risk TAD patients is to seek immediate surgical consultation and arrange for expedited aortic imaging. Expedited aortic imaging • TEE (preferred if clinically unstable) • CT scan (image entire aorta: chest to pelvis) • MR (image entire aorta: chest to pelvis)
Radiography (I)• Chest X ray – mediastinal widening(75%) – “calcium sign” -uncommon but highly specific, >5mm – double-density appearance of the aorta – a localized bulge along a normally smooth aortic contour – a disparity in the caliber between the descending and ascending aorta – obliteration of the aortic knob – displacement of the trachea or nasogastric tube to the right by the dissection – pleural effusions(left)
Radiography(II)• Echocardiography – transthoracic approach: M-mode & 2-D=low sensitivity and specificity – transesophageal = more accuracy and very sensitive, can be done in ER (safer).
Radiography (III)• Computed Tomography – dilatation of the aorta – identification of an intimal flap – differential rates of flow in true and false lumina – the clear demonstration of both the true and false lumina
Radiography(IV)• limitations of CT scan: – no information about the presence of AR – no information about the relationship of the dissection to the major branches of the aorta – time-consuming and requires the patient to be outside ER• advantages over aortography: – greater contrast resolution and detects small or delayed differences in the opacification of true and false channels – may be able to detect a thrombosed false lumen despite nonopacification – does not require arterial catheterization
Radiology(V)• Aortography – filling of a false channel or channels with or without an intervening intimal flap – distortion of the true lumen by either a patent or thrombosed false lumen – thickening of the aortic wall by more than 5-6 mm caused by a thrombosed false lumen – displaced intimal calcification
Radiography(VI)• disadvantages of aortography: – most invasive, most expensive – risks of intravenous contrast material – inadequate detection of pleural leak• advantages of aortography: – accurate for determining the site of the initmal tear and extent of the dissection – easily demonstrated aortic regurgitation – the only procedure that demonstrates the extent and location of dissection into aortic side branches
Radiography(VII)• Magnetic Resonance Imaging – shows the site of intimal tear, type and extent of dissection, presence of aortic insufficiency, and differential flow velocities in the true and false channels and in the aortic side branches• advantages: – no contrast material, no ionizing radiation, noninvasive
Recommendations for Screening Tests (continued)I IIa IIb III Urgent and definitive imaging of the aorta using transesophageal echocardiogram, computed tomographic imaging, or magnetic resonance imaging is recommended to identify or exclude thoracic aortic dissection in patients at high risk for the disease by initial screening.I IIa IIb III A negative chest x-ray should not delay definitive aortic imaging in patients determined to be high risk for aortic dissection by initial screening. http://content.onlinejacc.org/cgi/content/full/j.jacc.2010.02.015/DC1
Differential Diagnosis– Chest pain is the most common symptom in AD– Acute myocardial infarction • pain is more typically pressurelike but may radiate to the arms or neck • pain does not typically migrate over time • CK-MB levels are elevated– Pulmonary embolus • pain is generally respirophasic • hypoxemia secondary to ventilation/perfusion mismatch– Pericarditis • pain typically changes with position • auscultation may reveal a pericardial friction rub • EKG is common diagnostic(ST-segment elevation prominent in V5-6 and lead I)
Acute AoD Management PathwaySTEP 1: Immediate post-diagnosis management and disposition considerations • Arrange for definitive management: – Appropriate surgical consultation – Inter-facility transfer if indicated based on institutional capabilities • If transfer required, initiate aggressive medical management until transfer occurs. http://content.onlinejacc.org/cgi/content/full/j.jacc.2010.02.015/DC1
Acute AoD Management PathwaySTEP 2: Initial management of aortic wall stress • Obtain accurate blood pressure prior to beginning treatment. • Measure in both arms. • Base treatment goals on highest blood pressure reading.
Acute AoD Management PathwaySTEP 2: Initial management of aortic wall stress Intravenous rate and pressure control No Hypotension Rate/Pressure Control 1 or shock state? Intravenous beta blockade or Labetalol (If contraindication to beta blockade Yes substitute diltiazem or verapamil) Titrate to heart rate <60 + Anatomic based management Pain Control 2 Intravenous opiates Titrate to pain control Systolic BP >120mm HG? Secondary pressure control BP Control 3 Intravenous vasodilatorTitrate to BP <120mm HG (Goal is lowest possibleBP that maintains adequate end organ perfusion)
Acute AoD Management PathwaySTEP 2: Initial management of aortic wall stress Anatomic based management Type A dissection Type B dissection 1 Urgent surgical consultation 1 Intravenous fluid bolus + •Titrate to MAP of 70mm HG Arrange for expedited or Euvolemia operative management (If still hypotensive begin intravenous vasopressor agents)2 Intravenous fluid bolus •Titrate to MAP of 70mm HG 2 Evaluate etiology of or Euvolemia hypotension (If still hypotensive begin • Review imaging study forintravenous vasopressor agents) evidence of contained rupture • Consider TTE to evaluate Review imaging study for: cardiac function3 • Pericardial tamponade • Contained rupture 3 Urgent surgical consultation • Severe aortic insufficiency
Acute AoD Management PathwaySTEP 3: Definitive management • Depending on the results from the pressure control or anatomic based management, continued treatment will involve either: – ongoing medical management, or – operative or interventional management.
Acute AoD Management Pathway STEP 3: Definitive managementBased on results from intravenous Based on results from anatomicrate and pressure control: based management: No Dissection involving Etiology of hypotension the ascending aorta? Amenable to operative management? Ongoing medical management Close hemodynamic monitoring Maintain systolic BP < 120mm Hg (Lowest BP that maintains end organ perfusion) Complications requiring operative Operative or Operative or or interventional management? interventional Yes Yes interventional management Limb or mesenteric ischemia management Progression of dissection Aneurysm expansion Uncontrolled hypertension
Acute AoD Management Pathway STEP 4: Transition to outpatient management and disease surveillance • If no complications present requiring operative or interventional management, transition to: – Oral medications (beta blockade/ antihypertensives regimen) – Outpatient disease surveillance imagingNote: For full algorithm, see Figure 26 in full-text version of TAD Guidelines
• Definite Therapy Treatment(II) – Type A: acute aortic dissections require surgical treatment – the only contradiction to immediate surgical repair of a type A dissection is the simultaneous occurrence of a progressing stroke – Type B : medical management mortality is 15-20%(same as surgery done) – Surgery indications: persistent pain, uncontrolled hypertension, occlusion of a major arterial trunk, frank aortic leaking or rupture, or development of a localized anerysm. – Chronic aortic dissection: control of blood pressure using beta-blocking agents(most common)
Acute Surgical Management Pathway for AoD The following steps outline ascending TAD by imaging study. STEP 1: Determine patient suitability for surgery • If not suitable, begin medical management. STEP 2: Determine stability for pre-op testing • If not sufficiently stable, proceed with urgent operative management.
Acute Surgical Management Pathway for AoDSTEP 3: Determine likelihood of coexistent CAD Yes Assess need for Is patient age >40? preoperative coronary angiography Yes No • Known CAD? • Significant risk factors No for CAD? Yes Urgent operative No Significant CAD by management angiography? Yes Plan for CABG if appropriate at time of AoD repair
Acute Surgical Management Pathway for AoD STEP 4: Intra-operative evaluation of aortic valve • Perform intra-operative assessment of aortic valve by TEE. Aortic Regurgitation? or Dissection of aortic sinuses? Yes No STEP 5: Surgical intervention Graft replacement of ascending aorta Graft replacement +/- aortic arch and of ascending aorta repair/ replacement +/- aortic arch of aortic valveNote: For full algorithm, see Figure 22 in full-text version of TAD Guidelines.
Atypical aortic dissectionIntramural hematoma:• rupture of vasa vasorum,• aortic dissection without intimal flap,• 10% type B dissection,• failed diagnosis in aortography,• high risk for aneurysm formation,• medication (distal) or surgery (proximal)
Recommendation for Intramural Hematoma Without Intimal DefectI IIa IIb III It is reasonable to treat intramural hematoma similar to aortic dissection in the corresponding segment of the aorta.
Atypical aortic dissectionPenetrating atherosclerotic ulcer:• old, hypertension• no false lumen,• Aortography is standard• no definite treatment
Aortic atherothrombotic emboli• Age, hypertension, DM, hyperlipidemia, vascular disease• Most common in descending thoracic aorta• Coumadin is for high risk patients to prevent embolic event• Post-operative stroke
Cholesterol embolization syndrome• Cholesterol crystal from ulcerated atheromatous plaques• “blue-toe” or “purple-toe” syndrome• Elevated ESR & eosinophil• Reduced complement level• No specific therapy
Primary tumor of aorta• < 50 Cases• Equal in thoracic and abdomen aorta• Back pain• Aortography, biopsy• Prevent embolization
Peripheral artery diseasesIntermittent claudication:• pain, ache, fatigue, or discomfort in the affected leg during exercise, particularly walking (oxygen demand)• resolved with rest within few minutes• Buttock, hip, thigh• Gastrocnemius muscle is most common• Walking Impairment Questionnaire• Arterial embolism, vasculitis / arteritis, secondary compression, lumbar sacroradiculopathy (neurogenic pseudoclaudication, standing)
Peripheral artery diseasesRest pain• Inadequate blood flow• Skin fissure, ulceration, or necorsis• DM neuropathy or ischemic neuropathy
Peripheral artery diseasesPhysical examination:• Absent pulse distal to the stenotic site• Bruit of the stenotic site• Muscle atrophy, hair loss, cool skin, poor healing, pressure sore,
Peripheral artery diseasesAnkle/brachial index (ABI):• SBP ratio (normal: >=1)• ABI <0.9 : 95% sensitive for PAD• ABI 05—0.8 with claudication: critical limb ischemia• ABI <0.5 or ankle BP <55mmHG: poor ulcer healingMR angiography: 95% sensitivity and specificityContrast angiography
Thromboangitis obliterans• Young smokers• Medium and smalll vessels of the arms• Cause unknown? Type I and III collagen• Pain, digit ulceration, Raynaud phenomenon• Abnormal allen test (2/3)• Tx: Cessation smoking, prostacyclin analogue,