Diseases of The Aorta Diagnosis and Management

Diseases of The Aorta – Diagnosis and Management August 2010 Gian M. Novaro, MD Cleveland Clinic Florida, Weston, FL Histology • Large elastic arte...
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Diseases of The Aorta – Diagnosis and Management

August 2010 Gian M. Novaro, MD Cleveland Clinic Florida, Weston, FL

Histology • Large elastic artery • Serves as conduit, reservoir Intima- includes the single layered endothelium

Media- elastic tissue, collagen, smooth muscle cells

Adventitia- loose connective tissue, vasa vasorum

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Pathology • Cystic medial necrosis

Connective tissue disorders • Marfan, Ehlers-Danlos

• Loeys-Dietz syndrome

• Atherosclerosis – Abdominal aorta • Inflammatory – Infectious – Non-infectious

Hypertension, Increasing age Congenital, other

• Bicuspid aortic valve • Coarctation • Turner’s, Noonan’s

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Diseases • Aortic Dissection • Intramural Hematoma • Penetrating Ulcer • Aortic Aneurysm – Symptomatic • Atheromatous disease • Inflammatory Aortopathies

Acute Aortic Syndromes

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Case – 55 year old male with long standing history of hypertension – Sudden onset of tearing anterior chest pain, progressing to interscapular pain

– Blood pressure 160/90, HR 92 bpm – Chest clear. S1, S2, S4. No pulse deficits. No murmur heard – CXR revealed a widened mediastinum – TEE performed at bedside Intensive Review of Cardiology

TEE – Aortic dissection

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TEE – Aortic dissection

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Aortic dissection • Incidence, US - 2000/year • Intimal tear • False lumen • Antegrade propagation • Classification – Acute, Chronic (>2 wks) – DeBakey, Stanford

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Classification of aortic dissections

• DeBakey – Type I – Type II – Type III • Stanford – Type A – Type B

Type I

Type II

Type A

Type III

Type B

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Aortic dissection • Clinical Presentation – 5th - 7th decades of life – HTN history, male preponderance

– Patients under age 40 – Congenital abnormal aortic valve, aortic coarctation – Connective tissue disorders (Marfan, LDS, ED) – Pregnancy, often in third trimester

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Aortic dissection • Clinical Presentation – Chest pain

– Sudden onset – Anterior Pain – Inter-scapular – Migratory

– Hypertensive – Hypotensive, pseudo-hypotension

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Aortic dissection • Clinical Presentation – Pulse deficits – Aortic insufficiency – Myocardial ischemia/infarction – Pericardial effusion/tamponade – Cerebral, peripheral ischemia

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Aortic dissection – IRAD Incidence

in Type A

in Type B

Any Pain

95.5%

93.8%

98.3%

Hypertensive

49.0%

35.7%

70.1%

Normotensive

34.6%

39.7%

26.4%

Hypo/Shock

16.4%

24.6%

3.8%

AI murmur

31.6%

44%

12%

Pulse deficit

15.1%

18.7%

9.2%

CVA

4.7%

6.1%

2.3%

CHF

6.6%

8.8%

3.0%

Syncope

9.4%

12.7%

4.1%

Hagan et al; JAMA 2000: 897897-903

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Aortic dissection – Diagnostic tests • Chest X-ray – Mediastinal widening – Pleural effusions, commonly left – Enlarged cardiac silhouette – Normal

• ECG – Non-specific ST-T changes, can have ST elevation – Normal

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Aortic dissection – IRAD Incidence

in Type A

in Type B

Normal

12.4%

11.3%

15.8%

Wide medias

61.6%

62.6%

56.0%

Pleural eff

19.2%

17.3%

21.8%

Normal

31.3%

30.8%

32.1%

NST-T ∆’s

41.4%

42.6%

42.8%

ST ↑/new Q

3.2%

4.8%

0.7%

CXR

ECG

Hagan et al; JAMA 2000: 897897-903

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CT and MRI evaluation – Aortic dissection

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Aortic dissection – Imaging Modality

Advantages

Disadvantages

Pitfalls

TEE

Portability, Valvular, LV function No contrast

Blind spot Great vessels Intramural Hematoma

Reverberation Artifacts

CTA

Assess great vessels

MRI

Detailed resolution No nephrotoxicty

Not portable Access, cost

Angio

Assess coronaries

Invasive Risk Contrast

Not portable Streak and pulsation IV contrast Artifacts LV, valvular function Artifacts related to cardiac, aortic pulsation

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Aortic dissection – Therapy • Medical Therapy – Initiated as soon as diagnosis is entertained – Intravenous beta-blockade (need to decrease dp/dt) and intravenous nitroprusside

– If not hypertensive, intravenous beta-blockade – Be wary of pseudo-hypotension (secondary to brachial occlusion by flap)

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Aortic dissection – Therapy • Ascending aortic dissection (Type A) – Surgery for acute dissection – 1-2%/hour mortality for first 24-48 hours – Pericardial effusion/tamponade should NOT be evacuated percutaneously

– Pericardial access obtained in O.R. with cardiopulmonary bypass

Isselbacher et al; Circulation 1994: 23752375-8

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Aortic dissection – Therapy • Descending aortic dissection (Type B) – Medical therapy – Surgery in acute setting associated with high mortality and paraplegia rate

– Surgery reserved for organ ischemia (malperfusion syndrome- spinal, mesenteric, renal), persistent pain, saccular aneurysm, expansion (>5.5-6.0 cm), retrograde dissection

– Subsequent management of some debate (INSTEAD trial)

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Iatrogenic dissection • Type A - related to cardiac surgery • Type B - related to percutaneous procedures (cath, EVSG)

– Some very small series – Risks of early surgery include bleeding on antithrombotic or antiplatelet agents

– Conservative management in limited retrograde dissection

– In-hospital mortality remains high, at ~30-40%

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Diseases • Aortic Dissection • Intramural Hematomas • Penetrating Ulcers • Aortic Aneurysms • Atheromatous disease • Inflammatory Aortopathies

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Case – 72 year old male, presented with acute onset of chest and back pain. ECG revealed T wave inversions

– Catheterization performed emergently. Revealed severe 3 vessel disease. Aortogram- dilated aortic root, but no dissection

– Transferred for CABG. Persistent chest pain unresponsive to nitrates

– TEE performed

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TEE

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Dissection variants • Intramural Hematoma – Hematoma in aortic wall without intimal tear – Rupture of vasa vasorum – More commonly affect descending aorta, but may involve the ascending

– Can be a difficult diagnosis by TEE

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Dissection variants • Intramural Hematoma – Absence of dissection flap – Regional crescent-shaped thickening of aortic wall >0.7 cm

– Central displacement of intimal calcium

– Differential: atheroma, thrombosed false lumen or thrombosed aneurysm

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Intramural hematoma – Natural hx Meta-analysis of 168 cases Ascending

Descending

Dissection

25%

13%

Aortic rupture

28%

9%

Stabilization

28%

76%

Surgical mortality

18%

33%

vonKodolitsch et al. Z Kardiol 1998;87:9171998;87:917-27

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Case – 75 year old male with hypertension, carotid and peripheral vascular disease

– Presented with sudden onset of sharp chest pain – Chest clear. RRR. S1, S2, S4. No murmur. No pulse deficits – ECG, CXR unremarkable – TEE, cath performed

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Penetrating aortic ulcer

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Penetrating aortic ulcer

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Dissection variants • Penetrating aortic ulcer – Atheromatous plaque erodes/ruptures inward, penetrating the elastic lamina towards the aortic media (crater-like outpouching)

– Atheroma usually limits expansion along the media – Disease of elderly hypertensive men, preferentially involving the descending aorta

– Can result in formation of IM hematoma, saccular aneurysm, pseudoaneurysm or even complete aortic rupture (↑ rupture rate versus dissection)

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Penetrating aortic ulcer – Natural hx

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Dissection variants (PAU, IMH) – Therapy • Ascending aorta – Surgical therapy - considered the standard of care

• Descending aorta – Initial medical therapy as in classic dissection

– Surgical indications: pseudoaneurysm, ↑pleural effusion, expansion, and persistent/recurrent pain – Natural history not well defined – Reported outcomes differ in Asian populations

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Diseases • Aortic Dissection • Intramural Hematomas • Penetrating Ulcers • Aortic Aneurysms • Atheromatous disease • Inflammatory Aortopathies

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Case – 42 year old male. No significant past medical history – Presents with complaints of progressive exertional dyspnea – Exam remarkable for a Grade III/VI decrescendo diastolic murmur heard along the sternal border

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Aortic root aneurysm with AI

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Aortic root aneurysm with AI

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Thoracic aortic aneurysm – Incidence 6 cases per 100,000 patient years – Many patients asymptomatic at time of diagnosis – Physical findings may be absent – When present signs and symptoms are due to mass effect (SVC syndrome, stridor, dysphagia, hoarseness)

– Chest pain – Aortic root dilatation → AI (annuloaortic ectasia)

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Thoracic aortic aneurysm Regression Analysis % Increase in Risk 35 30 25 20 15 10 5 0 4

5

6

7

Aneurysm size (cm) Coady et al; J. Thorac Cardiovasc Surg. 1997;4761997;476-491

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Thoracic aortic aneurysm – Indications for elective surgery

All Ascending - >5.0-5.5 cm

Descending - >6.0 to 6.5 cm For ‘genetic’ aortas (i.e., Marfan, BAV, familial): Ascending - >5.0 cm; >4.5 cm if BAV undergoing AVR Rapid expansion (>0.5-1.0 cm/year), symptoms, traumatic etiology are also indications

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Abdominal aortic aneurysm – Most common form of arterial aneurysm – AAA is 10th leading cause of death in older males in the U.S.

– Atherosclerosis a dominant risk factor – 75% are infrarenal – Men are 10x more likely than women to have AAA >4.0 cm – Familial predisposition (15-29% prevalence in 1st degree relatives)

– Incidence estimated at 36 cases/100,000 pt yrs

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Abdominal aortic aneurysm – Asymptomatic – Pain – Atheroembolic events – Less than a 1/3 of patients with rupture present with classic triad of pain, pulsatile abdominal mass, hypotension

– 10% will have inflammatory component- pain, weight loss, constitutional symptoms, ↑ ESR

– Ultrasonography, CT, MRI, aortography

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Abdominal aortic aneurysm – Exam • EASY to do • 76-82% sensitivity for AAA >5.0 cm • Ideally in those with: – Waist 7.0 cm

AAA diameter UK Small Aneurysm Trial. Lancet 1998;352:16491998;352:1649-55 ADAM VA Cooperative Study Group. NEJM 2002;346:14372002;346:1437-44

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Abdominal aortic aneurysm – Screening

SVS, AAVS, SVMB (consensus statement): • Recommends AAA screening for: • All men 60-85 years • All women 60-85 years with CV risk factors • All men and women >50 years with family history United States Preventative Services Task Force (January 2005): • Recommends one-time screening by ultrasound in men aged 6575 who have ever smoked • Screening NOT recommended in women and nonsmokers • Potential to save 15,000 lives in U.S. annually Intensive Review of Cardiology

Abdominal aortic aneurysm – Indications for elective surgery – 5.0-5.5 cm has been used as an indication for surgery in asymptomatic patients, who are suitable surgical candidates

– 4.5-5.0 cm in patients of shorter height and women, who are suitable surgical candidates

– Rapidly enlarging (>1.0 cm/yr), symptomatic, infectious, traumatic are also indications for surgery

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Aortic aneurysm • Beta-blockade – Slow rate of expansion and complications in Marfan patients with –

ascending aortic aneurysms Slow rate of progression in large AAA in hypertensive patients

• ARB / ACE-inhibitor therapy • Endovascular stent graft repair

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Aortic aneurysm – medical Rx

Ahimastos et al. JAMA 2007; 298:1539-47

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Abdominal aortic aneurysm – EVAR Advantages

Disadvantages

• Minimally invasive • Regional anesthesia

• High incidence of endoleak • Lifelong surveillance

• Blood loss minimized • Hospital stay reduced

• High rates of re-intervention

• Rapid return to daily function

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AAA – The UK EVAR 1 trial

Kaplan-Meier Estimates for Total Survival and Aneurysm-Related Survival during 8 Years of Follow-up

The United Kingdom EVAR Trial Investigators

UK EVAR Investigators. N Engl J Med 2010; 362:1863

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Diseases • Aortic Dissection • Intramural Hematomas • Penetrating Ulcers • Aortic Aneurysms • Atheromatous disease • Inflammatory Aortopathies

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Case

– 65 year old woman undergoing elective CABG for 3 vessel disease

– Prior history of CVA, from which she has no residua – Carotid Dopplers revealed mild stenosis only – Intraoperative TEE performed

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Mobile atheroma on TEE

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Aortic atheromatous disease • Source of cerebral, peripheral emboli • TEE – Plaques > 4 mm in thickness – Mobile components – Non-calcified plaque – Role of anticoagulation

• Cardiac surgery - cannulation site, aortic repair

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Aortic atheromatous disease • Cholesterol embolization syndrome – Showering of emboli from descending aorta

– Livedo reticularis, blue toes, palpable pulses, renal insufficiency – Increased ESR, transient eosinophilia – Angiography, warfarin therapy, spontaneous – Supportive Tx – AAA resection if source

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Diseases • Aortic Dissection • Intramural Hematomas • Penetrating Ulcers • Aortic Aneurysms • Atheromatous disease • Inflammatory Aortopathies

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Inflammatory Aortopathies • Giant-cell arteritis – Common vasculitis, medium sized vessels/aorta – 2:1 male-female, age > 55 – Temporal artery involvement (scalp tenderness, jaw claudication, constitutional symptoms)

– Aorta and great vessels can be involved- leading to occlusion, aneurysm formation and dissection

– Corticosteroids

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Inflammatory Aortopathies • Takayasu’s (pulseless arteritis) – 8:1 female-to-male, under age 40 – Sclerotic phase, occlusive inflammation – Acquired coarctation, renal artery involvement – Corticosteroids (acute), surgical bypass

• Syphilitic – Tertiary syphilis, 10-30 years after initial – Saccular aneurysm, aortic insufficiency

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Inflammatory Aortopathies • Systemic inflammatory diseases – Reactive arthritis, ankylosing spondylitis, rheumatoid arthritis, enteropathic athropathies – Proximal aortitis, aneurysm formation

• Mycotic aneurysms, infected plaque – More common in descending aorta – In endocarditis cases or isolated aortitis

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Questions…

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Question 1 • A 70 year old male presents with the sudden onset of tearing chest pain. On presentation, he has a heart rate of 130 with a systolic blood pressure of 80 mm Hg. A bedside TEE demonstrates the presence of a proximal aortic dissection. There is also a pericardial effusion present with partial diastolic collapse of the right ventricle. Significant respiratory variation is noted across mitral and tricuspid inflows. Appropriate treatment would be:

– a) Immediate percutaneous pericardiocentesis to relieve the

tamponade, followed by surgery to replace the ascending aorta

– b) Proceed immediately to operating room – c) Emergent angiography to define coronary anatomy, followed by surgery

– d) Intra-aortic balloon pump to stabilize hemodynamics, followed by surgery

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• b) Proceed immediately to operating room – These patients should be taken to the operating room immediately. Percutaneous drainage has been associated with increased mortality in this setting. Given the hemodynamic status, there is no time to proceed with angiography first. Balloon pumps are contraindicated with aortic dissection

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Question 2 • A 60 year old hypertensive male presents with tearing back pain. MRI confirms the presence of a descending thoracic dissection originating beyond the left subclavian artery. Appropriate initial treatment would include:

– a) Immediate surgery to replace the descending aorta – b) Intravenous nitroprusside followed by immediate surgery – c) Intravenous nitroprusside alone. Surgery for persistent pain, involvement of renal or mesenteric arteries.

– d) Intravenous beta-blockade, nitroprusside. Surgery for persistent pain, involvement of renal or mesenteric arteries.

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• d) Intravenous beta-blockade, nitroprusside. Surgery for persistent pain, involvement of renal or mesenteric arteries.

– Initial therapy for descending dissections is medical, with surgery reserved for special circumstances. The goal in treatment is reduction in blood pressure, as well as reduction in dp/dt. Both betablockade and nitroprusside should be used.

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Question 3 • A 56 year old male presents for screening physical examination. He is asymptomatic. Vital signs reveal a heart rate of 80 with a blood pressure of 160/90. His exam is remarkable only for a pulsatile mass in the abdomen. Ultrasound reveals the presence of a 3.9 cm AAA. Appropriate management would include:

– a) Immediate referral for surgery – b) Start him on a beta blocker and repeat ultrasound in 6 mos – c) Refer for stenting of the AAA

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• b) Start him on a beta blocker; repeat ultrasound in 6 mos – Asymptomatic aneurysms of that size have a very small risk of rupture. He should be followed by serial examination to assess for size and rate of expansion. Control of his hypertension with beta-blockers may delay the growth of the aneurysm. There is no data as of yet that endovascular stents will lower the threshold for intervention for these aneurysms.

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Question 4 • A 76 year old woman with hypertension presents with severe chest pain. Her blood pressure is 200/110. Electrocardiogram reveals non-specific ST-T changes. Chest X-ray is unremarkable. CT scan demonstrates the presence of a penetrating ulcer in the ascending aorta. There is no dissection flap seen. Appropriate management would include:

– a) Start intravenous beta-blocker, nitroprusside while plans are being made for surgery

– b) Intravenous beta-blocker and nitroprusside, with surgery only if complications develop

– c) Intravenous nitroprusside alone with surgery only if complications develop

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• a) Start intravenous beta-blocker, nitroprusside while plans are being made for surgery

– Present practice is that penetrating ulcers involving the ascending aorta are treated like dissections with prompt referral for surgery.

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Question 5 • A 23 year old patient with Marfan syndrome presents for routine evaluation. He is asymptomatic. Work-up includes a CT scan, which reveals the presence of a 4.2 cm ascending aorta. Appropriate management would include:

– a) Refer for surgery

– b) Start on beta-blocker and re-image in 6-12 months – c) Re-image in 6-12 months

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• b) Start on beta-blocker and re-image in 6-12 months – His aorta has not yet reached a size which would be considered for surgery in the absence of symptoms. There is data that beta-blockers can slow the rate of expansion of these aneurysms and improve survival.

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Question 6 • The same patient returns for follow-up in 12 months. The aorta now measures 5.0 cm in size. He remains asymptomatic. Appropriate management would include:

– a) Refer for surgery

– b) Continue beta-blocker, reassess in 6 months – c) Reassess in 3 months

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• a) Refer for surgery – There has been rapid growth in the size of the aneurysm (0.8 cm in 1 year). He should be referred for surgery.

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Question 7 • Which of the following disorders is NOT associated with involvement of the aorta?

– a) Marfan syndrome

– b) Giant cell arteritis – c) Ankylosing spondylitis – d) Syphilis – e) All of the above can have aortic involvement

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• e) All of the above can have involvement of the aorta

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Question 8 • Which of the following statements is NOT true regarding transesophageal findings of atheroma?

– a) Plaques greater than 2 mm in the ascending aorta are associated with increased risk of stroke

– b) Plaques greater than 4 mm in the ascending aorta are associated with increased risk of stroke

– c) Mobile components are associated with an increased risk of stroke

– d) There is some limited data to suggest that these patients may benefit from anticoagulation therapy with coumadin

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• a) Plaques greater than 2 mm in the ascending aorta are associated with increased risk of stroke

– Plaques greater than 4 mm have been associated with cerebral embolic events. The role of anticoagulation needs to be more clearly defined, but there is some data to support their use.

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Question 9 • A 28 year old male presents to your office for a second opinion

regarding his hypertension. On examination, he is in no acute distress. Blood pressure 160/90, symmetric in both arms. Pulse rate 75 bpm. Cardiac exam reveals a non-displaced PMI. S1 is normal, followed by a high pitched sound widely transmitted throughout the precordium. A short II/VI systolic ejection murmur is heard. S2 is normal. Femoral pulses are weak. You order a diagnostic test, and plan to see him again in 1 week.

• He presents to the emergency room 3 days later with chest pain.

Blood pressure 80/60. Heart rate 125 bpm. Bilateral crackles are present. S1 is now soft. P2 is loud. An S3 is present. A short decrescendo diastolic murmur is heard at the sternal border. ECG reveals inferior ST segment elevation. A brief echocardiogram is performed at the bedside. The study is difficult, but reveals premature closure of the mitral valve. There is hypokinesis of the inferoposterior walls.

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Question 9 – con’t • Which of the following would be your next course of action? – a) Transesophageal echocardiogram

– b) Intra-aortic balloon pump, followed by emergent angiography

– c) Thrombolytic therapy

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• a) TEE, surgical consultation – The patient has a bicuspid aortic valve with coarctation (ejection click is heard, weak femoral pulses). These patients have an associated aortopathy.

– He presents with acute severe AR (short diastolic murmur, soft S1 from premature mitral valve closure, low output state with pulmonary edema). In this setting, this is dissection until proven otherwise. The dissection flap likely involves the ostium of the right coronary artery. Thrombolytics and IABP should not be used until dissection rule out.

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