AHA Guidelines - Thoracic Aortic Disease 2022 (old is 2010)

Question 1

What is included in the aortic root?

Answer 1

aortic valve annulus, aortic valve cusps, sinuses of Valsalva

Question 2

What is the extent of the ascending aorta?

Answer 2

STJ to the brachiocephalic artery

Question 3

What’s the normal thoracic aorta size - root, ascending, descending?

Answer 3

~3.5 for the root
~2.8 for the ascending
~2.5 for the descending and beyond

Question 4

How do you measure the aortic diameter?

Answer 4

CT: External aortic diameter. Centerline of flow.

Lumen size may not accurately reflect the external aortic diameter in the setting of intraluminal clot, aortic wall inflammation, or AoD.

In contrast, echo measurements are internal diameter size as the possibility of mural thrombus is less likely in the ascending aorta.

Question 5

Describe the protocol for an aortic CT?

Answer 5

Noncontrast study - can detect changes of IMH.
Contrast study - delineate the presence and extent of the dissection flap, identify regions of malperfusion, demonstrate contrast leak.
Imaging is extended all the way to the femoral arteries - provide sufficient info to plan surgical or endovascular tx.
CT is gated to ensure coronaries and root can be well evaluated.

Question 6

What does the image demonstrate?

Answer 6

IMH: Precontrast image demonstrates a high-attenuation aortic hematoma indicating an acute aortic event. Bottom, Images obtained with intravenous contrast material demonstrate the contrast-filled aortic lumen and the hematoma as a relatively lower attenuation band.

Question 7

What does the image show?

Answer 7

Mimic of aortic dissection created by motion of the aortic root. Top left, Image at the level of the right pulmonary artery demonstrates a normal descending thoracic aorta and pseudodissection of the ascending aorta due to motion artifact that occurs on non–ECG-gated CT examinations (arrow). Top right, Image at the aortic root shows a double contour to the aortic root that may simulate a dissection flap (arrow). Bottom, Still image through the thoracic aorta further delineates the extent of the motion artifact.

Question 8

What does the image show?

Answer 8

Arch aneurysm with dissection flap.
Top, Arch dissection, 2-dimensional view.
Bottom, Arch dissection (arrow) with color-flow Doppler margination.

Question 9

What does the image show?

Answer 9

Artifact mimicking dissection. Top left, 2-D view.
Top right, Color-flow Doppler without margination.
Bottom, Artifact not seen in this view.

Question 10

What study should be done at the time of diagnosis of Marfan syndrome to eval the aortic root and ascending aortic diameters?
How should they be followed?

Answer 10

Echocardiogram.
At diagnosis and q6 mo.

Question 11

Are there any imaging recommendations for pts with Turner syndrome?

Answer 11

Any cross-sectional imaging - eval heart, aorta. Check for bicuspid aortic valve, coarctation of the aorta, or dilation of the ascending thoracic aorta.

If normal, repeat every 5-10 yrs.

Question 12

What should pts w/ Loeys-Dietz syndrome or a confirmed genetic mutation known to predispose to aortic aneurysms and aortic dissection undergo?

Answer 12

Complete aortic imaging at initial diagnosis and 6 mo thereafer to establish if enlargement is occurring.

Question 13

It is reasonable to consider surgical repair of the aorta in all adult patients with Loeys-Dietz syndrome or a confirmed TGFBR1 or TGFBR2 mutation and an aortic diameter of what by TEE and/or CT?

Answer 13

4.2 cm or greater by transesophageal echocardiogram (internal diameter) or 4.4 to 4.6 cm or greater by computed tomographic imaging and/or magnetic resonance imaging (external diameter).

Question 14

For women with Marfan syndrome contemplating pregnancy, it is reasonable to prophylactically replace the aortic root and ascending aorta if the diameter exceeds?

Answer 14

4.5 cm

Question 15

In Marfan patients, if the maximal cross-sectional area in square centimeters of the ascending aorta or root divided by the patient’s height in meters EXCEEDS WHAT RATIO, surgical repair becomes reasonable? Why?

Answer 15

10.

Surgical repair is reasonable because shorter patients have dissection at a smaller size and 15% of patients with Marfan syndrome have dissection at a size less than 5.0 cm

Question 16

What is recommended for first-degree relatives of patients with thoracic aortic aneurysm and/or dissection to identify those with asymptomatic disease?

Answer 16

Aortic imaging (vague)

Question 17

If the mutant gene (FBN1, TGFBR1, TGFBR2, COL3A1, ACTA2, MYH11) associated with aortic aneurysm and/or dissection is identified in a patient, first-degree relatives should undergo what?

Answer 17

Counseling and testing. Then, only the relatives with the genetic mutation should undergo aortic imaging.

Question 18

If one or more first-degree relatives of a patient with known thoracic aortic aneurysm and/or dissection are found to have thoracic aortic dilatation, aneurysm, or dissection, then should imaging of second-degree relatives be done?

What gene sequencing should be considered?

Answer 18

It’s reasonable.

Also, sequencing of the ACTA2 gene is reasonable in patients with a family history of thoracic aortic aneurysms and/or dissections to determine if ACTA2 mutations are responsible for the inherited predisposition.

Sequencing of other genes known to cause familial thoracic aortic aneurysms and/or dissection (TGFBR1, TGFBR2, MYH11) may be considered in patients with a family history and clinical features associated with mutations in these genes.

Question 19

What screening recommendations are there for relatives of pts w/ bicuspid aortic valve, premature onset of thoracic aortic disease with minimal risk factors, and/or a familial form of thoracic aortic aneurysm and dissection?
What modality should be chosen?

Answer 19

First-degree relatives should be evaluated for BAV and asx aortic disease.
TTE is first line. CTA or MRI should be used if TTE is incomplete.

Question 20

All patients with a bicuspid aortic valve should have what evaluated?

Answer 20

both the aortic root and ascending thoracic aorta evaluated for evidence of aortic dilatation

Question 21

Describe aberrant R SCA. What is the anatomy like? What symptoms can it cause?
Any associations?
How is it managed?

Answer 21

Arises as the fourth branch from the aorta, courses behind the esophagus in approximately 80% of patients and causes dysphagia (lusoria) in many patients.

In most adult patients, the aorta is also abnormal and is prone to aneurysm formation, dissection, and rupture.

Surgical treatment in adults involves resection of the aneurysmal segment of the subclavian artery (the diverticulum) and the adjacent aorta and replacement of the aorta with a graft.

Question 22

Describe the adult presentation of problems with a repaired coarctation of the aorta.

What should be checked in these patients?

Answer 22

heart failure, intracranial hemorrhage, hypertension particularly with exercise, aneurysm formation, AoD, rupture of old repairs, undersized grafts of previous repairs, and infections

It is of particular importance in previously treated patients with some aortic narrowing to check for a gradient across the stenosis and for hypertension during exercise testing.

Question 23

What is pseudo-coarctation of the aorta? What problems can it cause?

Answer 23

The adult aorta may be redundant and kinked opposite the ligamentum arteriosum without any pressure gradient, the so-called pseudocoarctation. Aneurysms that require surgical treatment may develop proximal and distal to the kinked area.

Question 24

What kind of problems can a right aortic arch cause?

Answer 24

Dysphagia or asthma symptoms - CT or MR can show tracheal or esophageal compression.

Question 25

A patient presents w/ the following:
Age of onset <40 y.
Intermittent claudication.
Diminished brachial artery pulse.
Subclavian artery or aortic bruit.
SBP variation of >10 mm Hg between arms.
Aortographic evidence of aorta or branch stenosis.

What is the diagnosis? Tx?

Answer 25

≥3 criteria are present (sensitivity 90.5%; specificity 97.8%) -> Takayasu arteritis.
Initial tx w/ high dose steroids (prednisone 40-60 daily).
Periodic eval to determine activity w/ PE and ESR or CRP.
CTA of thoracic aorta and arch branches should be obtained.
Elective revascularization should be delayed until inflammatory state is treated and quiescent.

Question 26

A patient presents with the following:
Age >50 y.
Recent-onset localized headache.
Temporal artery tenderness or pulse attenuation.
Elevated erythrocyte sedimentation >50 mm/h.
Arterial biopsy shows necrotizing vasculitis.

What is the diagnosis?

Answer 26

≥3 criteria are present (sensitivity greater than 90%; specificity >90%) -> Giant cell arteritis.

Question 27

What is the diagnosis of the following presentation:
Oral ulceration
Recurrent genital ulceration
Uveitis or retinal vasculits
Skin lesions—erythema nodosum, pseudo-folliculitis, or pathergy

Answer 27

Oral ulceration plus 2 of the other 3 criteria -> Behcet disease

Question 28

What is the diagnosis of the following presentation:
Onset of pain <40 y
Back pain for >3 mo
Morning stiffness
Subtle symptom onset
Improvement with exercise

Answer 28

4 of the diagnostic criteria are present -> Ankylosing spondylitis

Question 29

What are the major causes (Ie from where) of aortic infection?
What is the usual anatomic location of infected aneurysm in relation to other vessels nearby?

Answer 29

First, there may be contiguous spread from adjacent thoracic structures, such a mediastinitis, abscess, infected lymph nodes, infectious pericarditis, empyema, or paravertebral abscess.
Second, there may be septic emboli from underlying bacterial endocarditis.
Third, there may be hematogenous dissemination of bacteria in the setting of sepsis or intravenous drug abuse.

Typically, the sites of infected aneurysms are opposite the great vessels in the aortic arch or opposite the visceral arteries in the abdomen.

Question 30

Most common organisms to infect the aorta?

Answer 30

Staphylococcus aureus and Salmonella are the organisms most commonly identified. Pneumococcus and Escherichia coli are relatively common gram-positive and gram-negative pathogens, respectively.

Question 31

Treponema pallidum most commonly infects what part of the aorta?

Answer 31

Ascending aorta
This is the gram-negative spirochete bacterium that causes syphilis.

Question 32

Are fungal aortic infection common?

What situations are they not likely to occur?

Answer 32

Fungal infections of the aorta, with either Candida or Aspergillus, occur less often.

They typically occur in the setting of impaired immunity, such as patients with systemic illness, human immunodeficiency virus, or prior organ or bone marrow transplant.

Question 33

What are the three acute aortic syndromes?

Answer 33

AoD, IMH, and PAU

Question 34

Describe what’s happening in this image.

Answer 34

Type A Aortic dissection with thrombosed false lumen and left renal artery involvement depicted on axial CT images.
Top left image demonstrates marked narrowing of the true lumen.
Patent right renal artery arising from the true lumen (bottom left, arrow), and narrow left renal artery compressed by thrombus in the false lumen, with secondary decreased enhancement of the left kidney compared with the right kidney.

Top left, At the level of the left main coronary artery. Top right, At the celiac axis. Bottom left, At the right renal artery (arrow). Bottom right, At the left renal artery (arrow). *Thrombus in false lumen.

Question 35

Describe the considerations for type A aortic dissection management.
I.e. What questions are in the algorithm?

Answer 35

Operative candidacy?
Preop testing capability?
Likelihood of CAD?
Arch involvement? Root involvement? AV valve involvement?

Question 36

most commonly recognized cardiac complication of Type A dissection

Answer 36

Acute aortic regurgitation

Question 37

most frequently involved end organ in acute AoD involving the ascending aorta

Answer 37

heart

In distinction to other end-organ pathology, most cardiac complications are a direct result of dissection-related disruption of normal anatomic relationships.

Question 38

Three distinct acute aortic dissection-related mechanisms for acute aortic valve incompetence have been identified, and they can occur in combination. What are they?

Answer 38

1) acute dilatation of the aortic root by an expanding false lumen, resulting in incomplete aortic valve closure
2) a dissection extending into the aortic root and disrupting aortic valve commissural attachments, resulting in valve leaflet prolapse
3) a portion of dissection flap prolapsing through the aortic valve in diastole, preventing adequate leaflet closure.

Question 39

Pericardial pathology is a frequent complication of acute Type A AoD and can occur via 2 distinct mechanisms. What are they?

Answer 39

Most commonly, transudation of fluid across the thin wall of an adjacent false lumen into the pericardial space leads to a hemodynamically insignificant pericardial effusion, which is present in about one third of patients.

Less often, the dissected aorta ruptures directly into the pericardium, leading rapidly to tamponade physiology and hemodynamic compromise.

Question 40

What does the image show?

Answer 40

Penetrating atherosclerotic ulcer of the proximal descending thoracic aorta. Axial CT images at the level of the aortopulmonary window (left) and at the level of the left pulmonary artery (right) demonstrate a small penetrating ulcer (long arrow, U) that extends beyond the expected confines of the aortic lumen.

Question 41

What are high-risk signs and symptoms associated with acute thoracic syndromes?

Answer 41

Pulse deficit. Systolic blood pressure limb differential greater than 20 mm Hg. Focal neurologic deficit. Murmur of aortic regurgitation (new).
High-risk chest, back, or abdominal pain features: pain that is abrupt or instantaneous in onset, pain that is severe in intensity, pain that has a ripping, tearing, stabbing, or sharp quality.

Question 42

Patients presenting with sudden onset of severe chest, back, and/or abdominal pain, particularly those less than 40 years of age, should be questioned about and examed for?

Answer 42

A history and examined for physical features of Marfan syndrome, Loeys-Dietz syndrome, vascular Ehlers-Danlos syndrome, Turner syndrome, or other connective tissue disorder associated with thoracic aortic disease.
A history of aortic pathology in immediate family members as there is a strong familial component to acute thoracic aortic disease.
Any recent aortic manipulation (surgical or catheter-based) or a known history of aortic valvular disease, as these factors predispose to acute aortic dissection.
A focused examination should be performed to identify associated neurologic injury or the presence of pericardial tamponade.

Question 43

All patients presenting with acute neurologic complaints should be questioned about?

Answer 43

the presence of chest, back, and/or abdominal pain and checked for peripheral pulse deficits as patients with dissection-related neurologic pathology are less likely to report thoracic pain than the typical aortic dissection patient

Question 44

What is the recommended workup for pts w/ suspected acute thoracic aortic disease?

Answer 44

EKG - presence of ST-segment elevation suggestive of myocardial infarction should be treated as a primary cardiac event without delay for definitive aortic imaging unless the patient is at high risk for aortic dissection.
CXR - can find alternate dx.
Urgent and definitive imaging of the aorta using transesophageal echocardiogram, computed tomographic imaging, or magnetic resonance imaging - identify or exclude thoracic aortic dissection in patients at high risk for the disease by initial screening.

Question 45

If a high clinical suspicion exists for acute aortic dissection but initial aortic imaging is negative, what should be done?

Answer 45

second imaging study should be obtained

Question 46

Initial management of thoracic aortic dissection should be directed at?

Answer 46

Decreasing aortic wall stress by controlling heart rate and blood pressure as follows:
- In the absence of contraindications, intravenous beta blockade should be initiated and titrated to a target heart rate of 60 beats per minute or less.
- In patients with clear contraindications to beta blockade, nondihydropyridine calcium channel-blocking agents should be used as an alternative for rate control.
- If systolic blood pressures remain greater than 120 mm Hg after adequate heart rate control has been obtained, then angiotensin-converting enzyme inhibitors and/or other vasodilators should be administered intravenously to further reduce blood pressure that maintains adequate end-organ perfusion.
- Beta blockers should be used cautiously in the setting of acute aortic regurgitation because they will block the compensatory tachycardia.
- Vasodilator therapy should not be initiated prior to rate control so as to avoid associated reflex tachycardia that may increase aortic wall stress, leading to propagation or expansion of a thoracic aortic dissection.

Question 47

Acute thoracic aortic dissection involving the descending aorta should be managed how?

Answer 47

medically unless life-threatening complications develop (eg, malperfusion syndrome, progression of dissection, enlarging aneurysm, inability to control blood pressure or symptoms)

Question 48

For all patients diagnosed with ascending thoracic aortic dissection, what should be done for definitive management?

Answer 48

Urgent surgical consultation should be obtained to evaluate for emergent surgical repair because of the high risk of associated life-threatening complications such as rupture.

Question 49

For patients with ascending thoracic aortic dissection, what is the surgical objective?

Answer 49

All of the aneurysmal aorta and the proximal extent of the dissection should be resected. A partially dissected aortic root may be repaired with aortic valve resuspension. Extensive dissection of the aortic root should be treated with aortic root replacement with a composite graft or with a valve sparing root replacement. If a DeBakey Type II dissection is present, the entire dissected aorta should be replaced.

Question 50

When a Type A AoD involves the aortic root, what can be done to preserve the valve? When is replacement necessary?

Answer 50

Resuspension of the valve with preservation of the aortic sinuses, and excision of the sinuses and resuspension of the valve within a polyester graft are suitable options.

If the aortic root is dilated, or if there is extensive dissection and disruption of the aortic sinuses, replacement with a composite graft is necessary.

Question 51

Principles of Treatment for Intramural Hematoma and Penetrating Atherosclerotic Ulcer

Answer 51

The goals of treatment are to prevent aortic rupture or progression to classic AoD, allow patient stabilization before urgent surgery, and reduce complexity of unavoidable aortic surgery. Aggressive medical treatment usually includes, particularly in symptomatic patients, beta blockers and other antihypertensive therapy. Indications for open or endograft treatment are based on the anatomic features of the lesion, clinical presentation and course, patient comorbidities, and anatomic constraints related to endograft technology. Treatment by endografts or open aortic reconstruction can be discussed in the context of 3 overlapping aortic lesions: intimal defect without IMH, intimal defect with IMH, and IMH without an intimal defect.

Question 52

An aortic Intimal Defect Without Intramural Hematoma is found. What are the considerations, and what do you do?

Answer 52

These are localized lesions and may involve a limited segment of the aorta. They are often an incidental finding. By imaging criteria, they include uncomplicated aortic ulcers, blebs, and eccentric or saccular aneurysms of the aorta. They are treated as saccular aneurysms based on their maximum diameter and clinical feature.
These lesions can be treated with open reconstruction and are the most suitable of the 3 groups for treatment by endografts, if in the descending thoracic aorta. They involve a limited segment, which can easily be excluded from the circulation, as long as there is an adequate distance from a critical branch artery. When these limited dissections involve the ascending aorta, emergency surgery is indicated as for other types of AoD because rupture or cardiac tamponade can occur.

Question 53

A (Type B) aortic Intimal Defect WITH Intramural Hematoma is found. What do you do?

Answer 53

The intimal defect again presents a target lesion for endovascular treatment in the descending thoracic aorta, but the associated IMH involves a longer segment of aorta than those without IMH.
If the patient becomes asymptomatic in response to aggressive medical treatment, it may be possible to delay endovascular or open reconstruction until the IMH has reabsorbed and organized. (Of note, some writing committee members have observed healing of IMH such that immediate reconstruction was not required but have continued to follow that small number of patients closely.)
Two considerations affect the length of aorta bordering the intimal defect, which is to be included in the segment targeted for treatment. Evidence of adjacent atheromatous wall should favor more extensive treatment of the aorta with longer endografts, because radiographic imaging underestimates shallow ulcerated atheromas, and the ulcer typically arises in a bed of atheromatous intima. Treatment with longer endografts provides a safety margin against undertreating the intimal defect.
The second consideration is the extent of associated IMH. The self-expanding endograft may tear through the intimal surface into underlying thrombosed false lumen. When treatment of this lesion in the acute stage is clinically necessary (eg, persisting pain, evidence for expansion or rupture, compromise of critical branches), it is preferable to anchor the endograft in the noninvolved wall above and below the intimal defect.

Question 54

Management recommendations for Intramural Hematoma Without Intimal Defect

Answer 54

It is reasonable to treat intramural hematoma similar to aortic dissection in the corresponding segment of the aorta.

Question 55

A patient with type A aortic dissection is clinically stable, but you are at an institution that does not regularly treat this disease. What should be considered?

Answer 55

transfer to a high-volume aortic center to improve survival; especially for those with extensive aortic disease, those who might benefit from complex open and endovascular repairs, and for those with multiple comorbidities in whom intervention is considered

Question 56

The operative repair of a type A aortic dissection should entail at least what?

Answer 56

an open distal anastomosis rather than just a simple supracoronary interposition graft

Question 57

In patients with aneurysms of the aortic root or ascending aorta, or those with aortic dissection, what is the screening recommendation for relatives?

Answer 57

first-degree relatives should be screened with imaging

Question 58

What are some of the key findings on CT/MRI in discussing thoracic aortic disease?

Answer 58

maximal aortic diameters, wall changes, luminal stenosis, findings suggestive of acute aortic syndrome, involvement of branch vessels, presence of prior repair, comparison with previous examinations, and impressions regarding disease classification

Question 59

What is the cutoff age for those with thoracic aortic disease that should receive genetic testing?

Answer 59

<60

Question 60

Rapid growth of the thoracic aorta is defined as? What about for Marfan syndrome?
What about bicuspid aortic valve?

Answer 60

nsHTAD (nonsyndromic heritable thoracic aortic disease) or sporadic TAA: ≥0.5 cm/yr or ≥0.3 cm/yr for 2 consecutive years.

MFS: ≥0.3 cm/yr.
BAV: ≥0.3 cm/yr.

Question 61

A Marfan or Loeys-Dietz patient has a thoracic aneurysm not meeting cutoff for surgery. What is the medical mgmt?

Answer 61

either a beta-blocker or an angiotensin receptor antagonist (ARB) in maximally tolerated doses

Question 62

A Marfan patient has a thoracic aneurysm. When is surgery to replace the aortic root and ascending aorta recommended?

Answer 62

surgery with an experienced surgeon is reasonable with either an aortic root diameter ≥4.5 cm plus high-risk features (family history of dissection, rapid growth, diffuse aortic root and ascending aorta dilation, or marked vertebral artery tortuosity)
***normally recommended at 5 cm or cross-sectional aortic root area to patient height ratio ≥10 cm2/m

Question 63

What are the cutoff measurements for surgical replacement of the aortic root or ascending aorta in a bicuspid aortic valve (BAV) pt?

Answer 63

Surgery to replace the aortic root and/or ascending aorta is recommended with an aortic diameter ≥5.5 cm. Surgical intervention is reasonable with a cross-sectional area to height ratio ≥10 cm2/m, with an aortic diameter 5.0-5.4 cm plus a risk factor for dissection (family history of dissection, rapid growth, aortic coarctation, or “root phenotype” aortopathy), or with an aortic diameter ≥4.5 cm at the time of aortic valve replacement or repair.

Question 64

AAA US screening recommendations?

Answer 64

≥65 years who have ever smoked and in men or women ≥65 years with a first-degree relative with AAA. Ultrasound screening is reasonable in women ≥65 years who have ever smoked.

(I’d need to be screened at 65)

Question 65

What is the recommended medical management for stable AAA patients?

Answer 65

Moderate- or high-intensity statin therapy is recommended in patients with AAA and evidence of aortic atherosclerosis. Low-dose aspirin may be considered in patients with AAA and concomitant atheroma or penetrating aortic ulcer (PAU).

Question 66

What is the recommended AAA surveillance based on size?

Answer 66

Surveillance should be every 3 years in patients with AAA diameter 3.0-3.9 cm, annually in men with AAA diameter 4.0-4.9 cm or women with AAA diameter 4.0-4.4 cm, and every 6 months in men with AAA diameter ≥5.0 cm or women with AAA diameter ≥4.5 cm.

Question 67

When is AAA repair recommended?

Answer 67

Repair is recommended in men with AAA diameter ≥5.5 cm or women with AAA diameter ≥5.0 cm, and in the setting of symptoms attributable to the aneurysm.

Consider open AAA repair when EVAR is not applicable and comorbidities are not prohibitive, for patients with the following:
Rapidly expanding AAA (>5 mm in 6 months).
Symptomatic AAA regardless of diameter, and anatomy unsuited for EVAR.
Infected AAA.
Conversion after failed EVAR.

Question 68

What is the recommended medical therapy for sporadic/degenerative thoracic aortic aneurysm (TAA)?

Answer 68

1) The use of antihypertensive medications is recommended among patients with sporadic TAA and blood pressure (BP) ≥130/80 mm Hg.
2) In the absence of contraindications, the use of beta-blockers and/or ARB therapy is reasonable.
3) Moderate- or high-intensity statin therapy is reasonable in patients with TAA and clinical or imaging evidence of atherosclerosis.
4) Low-dose aspirin is reasonable in patients with TAA and concomitant atherosclerosis or PAU.

Question 69

A sporadic/degenerative thoracic aortic aneurysm (TAA) is incidentally found. What imaging should be done? What are you looking for?

Answer 69

TTE is recommended at the time of diagnosis to assess aortic valve anatomy and function and thoracic aortic diameters. CT or MRI is reasonable at the time of diagnosis.

Question 70

Surveillance for sporadic degenerative TAA?

Answer 70

Surveillance imaging with TTE, CT, or MRI is reasonable after 6-12 months, and then, if stable, every 6-24 months.

Question 71

For aortic root or ascending aorta aneurysm, surgery is recommended…

Answer 71

for symptoms attributable to the aneurysm, diameter ≥5.5 cm, or rapid growth; and reasonable at the time of tricuspid aortic valve replacement with an aortic diameter ≥5.0 cm. With an experienced surgeon, surgery is reasonable with an aortic diameter ≥5.0 cm (or in a patient with height >1 standard deviation above or below the mean with a cross-sectional aortic area to height ratio ≥10 cm2/m), or at the time of tricuspid aortic valve replacement with an aortic diameter ≥4.5 cm.

Question 72

TEVAR vs open surgery in pts with descending TAA w/o MFS, LDS, or vascular Ehlers-Danlos.

Answer 72

Thoracic endovascular aortic repair (TEVAR) is recommended over open surgery in patients without MFS, LDS, or vascular Ehlers-Danlos syndrome who have descending TAA meeting criteria for intervention and suitable anatomy; consideration for alternative vascular access is recommended for patients with smaller or diseased access vessels.

Question 73

After treatment of TAA with TEVAR, surveillance imaging should be done with what modality and what interval?

Answer 73

CT is recommended after 1 month and 12 months, and then annually if stable; MRI is a reasonable alternative to CT.

Question 74

After open repair of TAA, what is surveillance?

Answer 74

CT or MRI within 1 year and then every 5 years is reasonable in the absence of residual aortopathy; annual imaging is reasonable if there is residual aortopathy or abnormal findings on surveillance imaging.

Question 75

Acute aortic syndrome (AAS; dissection, PAU, IMH) medical therapy?

Answer 75

Patients with AAS should be treated to a systolic BP <120 mm Hg or to the lowest BP that maintains end-organ perfusion, and to a heart rate of 60-80 bpm. Unless contraindicated, intravenous beta-blockers are recommended as initial management, and vasodilators if BP is not adequately controlled with beta-blockers. Long-term beta-blockers are recommended after the initial treatment of AAS.

Question 76

TAAD pt with aortic valve involvement. Repair vs replace?

Answer 76

Aortic valve resuspension is recommended over replacement in patients with type A dissection and no significant aortic valve leaflet pathology; aortic valve replacement with a mechanical or biological valved conduit is recommended if there is extensive destruction of the aortic root, a root aneurysm, or a known genetic disorder. An open distal anastomosis is recommended in the aortic repair.

Question 77

Procedural intervention is recommended for type B aortic dissection when?

Answer 77

Intervention is recommended in patients with acute type B dissection and rupture, branch artery occlusion and malperfusion, dissection extension, progressive aortic enlargement, intractable pain, or uncontrolled hypertension. In patients with rupture and suitable anatomy, endovascular stent-grafting is recommended over open surgical repair.

Medical therapy is recommended as the initial management in patients with uncomplicated acute type B dissection.

Question 78

IMH management?
Timing?
Type A vs type B IMH?

Answer 78

Urgent repair is recommended in patients with acute type A or type B IMH and complications (malperfusion, periaortic hematoma, pericardial effusion with tamponade, refractory or recurrent pain, or rupture). Prompt surgical repair is recommended in uncomplicated acute type A IMH. Medical therapy is recommended as the initial treatment in patients with uncomplicated acute type B IMH.

Question 79

PAU management?

Answer 79

Urgent repair is recommended in patients with PAU and rupture; urgent repair is recommended for ascending aorta PAU with associated IMH; repair is recommended in patients with uncomplicated PAU and persistent pain.

Question 80

Long-term surveillance imaging after Acute aortic dissection or IMH?

Answer 80

In patients with acute aortic dissection or IMH treated with open or endovascular repair and who have residual disease, and in patients managed only with medical therapy, CT (or MRI) is recommended after 1 month, 6 months, 12 months, and then annually if stable.

Question 81

Long-term surveillance imaging after PAU?

Answer 81

Imaging follow-up in patients with PAU who underwent repair should follow the same guidelines as for patients who underwent repair of TAA. In patients with PAU managed medically, CT is reasonable after 1 month and, if stable, every 6 months for 2 years; and then based on patient age and PAU characteristics.

Question 82

In patients with syndromic HTAD, nsHTAD, Turner syndrome, BAV with aortic dilation, or another aortopathy, what should be done before pregnancy?

Answer 82

both counseling and aortic imaging (with TTE or CT or MRI) is recommended before pregnancy

Question 83

Recommendations for surgery prior to pregnancy in pts with thoracic aortic disease?

Answer 83

Surgery prior to pregnancy is recommended in patients with MFS and aortic root diameter >4.5 cm, nsHTAD and aortic diameter ≥4.5 cm, BAV and aortic diameter ≥5.0 cm, Turner syndrome and ASI ≥2.5 cm/m2, and sporadic aortic root or ascending aorta aneurysm diameter ≥5.0 cm.

Surgery prior to pregnancy is reasonable in patients with LDS attributable to pathologic variants in TGFB2 or TGFB3 and aortic diameter ≥4.5 cm.

Question 84

Management guidelines in patients who are pregnant and have thoracic aortic disease (not undergoing surgery) -
Who should manage patient?
What med should be given?

Answer 84

During pregnancy, patients with aortic aneurysm or increased risk of dissection should be managed by a multidisciplinary team including a maternal fetal medicine specialist and a cardiologist; and hypertension should be treated using guideline-directed therapy.

Among patients with syndromic HTAD or nsHTAD, beta-blocker therapy is recommended during pregnancy and post-partum.

Question 85

Vaginal vs C-section delivery in pregnant TAD pts?
How does size affect this decision?

Answer 85

Delivery by cesarean section is recommended in patients with chronic aortic dissection, and is reasonable in patients with aortic root or ascending aorta aneurysm diameter ≥4.5 cm; if otherwise appropriate, vaginal delivery is recommended in patients with aortopathy and aortic diameter <4.0 cm.

Question 86

During pregnancy, patients with aortic aneurysms or increased risk of dissection should get surveillance; how often?

Does modality change with disease anatomy?

Answer 86

Surveillance TTE is recommended during each trimester and several weeks post-partum;

MRI without gadolinium is recommended over CT among patients in whom surveillance imaging of the arch, descending thoracic aorta, or abdominal aorta is required.