B P5 C42 Diseases of the Aorta Flashcards
Begins at the aortic valve and extends to the sinotubular junction. This supports the bases of the aortic valve leaflets.
Aortic root
The right and left coronary arteries arise from the __________________
Sinuses of Valsalva
Begins at the sinotubular junction and rises to join the aortic arch. The proximal portion of the ascending aorta lies within the pericardial cavity, anterior to the pulmonary artery bifurcation
Upper portion of the ascending aorta
Gives rise to the innominate artery, the left common carotid artery, and the left subclavian artery.
Aortic arch
Begins distal to the left subclavian artery.
Descending thoracic aorta
Marks the point at which the aortic arch joins the descending aorta
Ligamentum arteriosum
Marks the site of transition between the relatively mobile ascending aorta and the fixed descending aorta making it vulnerable to deceleration trauma.
Aortic isthmus
Gives rise to the celiac artery and the superior mesenteric artery anteriorly, followed by the posterolateral origins of the left and anterolateral right renal arteries.
Abdominal aorta
This segment of the aorta is called the suprarenal or visceral segment. The infrarenal aorta lies anterior to the lumbar spine, where paired lumbar artery branches arise posteriorly.
The aorta ends by bifurcation into _________________
Common iliac arteries
In the proximal aortic segments, the vasa vasorum supply additional nutrients to the outer third of the thoracic aortic media. The ____________ aorta normally lacks an independent microvascular supply.
Infrarenal
The aortic wall pressure-diameter relationship is nonlinear; a more distensible component is demonstrated at lower pressures and a stiffer component at higher pressures, with the transition from distensible to stiff behavior occurring at pressures higher than _________________
80 mm Hg
Aortic diameter is generally less than __________________ at the root and becomes smaller distally.
< 40 mm
The bifurcation typically occurs at the level of the umbilicus and the _______________
L4 vertebral body.
refers to a pathologic segment of aortic d tation that expands and can eventually rupture or dissect. One criterion for abnormal aortic dilatation is a diameter of at least 50% greater than expected for the same aortic segment or l dilation 50% greater than the adjacent normal aorta
Aortic aneurysm
The more common type, are symmetrically dilated with involvement of the entire aortic circumference
Fusiform
Exhubits focal outpouching. These both are “true” aneurysms with an intact aortic wall involving all layers.
Saccular
Bleeding has occurred through the aortic wall r ing in a contained periaortic hematoma in continuity with the aortic lumen; may result from trauma, infection or contained rupture of an aortic aneurysm, dissection, or penetrating ulcer.
Pseudoaneuyrsm
Defined by an abdominal aorta greater than 3.0 cm in diameter
AAA
The most common form of aortic aneurysms, being present in 2.3% of those 75 to 79 years old.
AAA
Most AAA (>80%) arise in the ______________
Infrarenal aorta
AAAs are approximately five times more prevalent in _____ than in women, and are associated strongly with age, with most occurring in those ______________ and even higher risk in those older than 75 years.
Men
>60 years
AAAs strongly are associated with ______________
Cigarette smoking
Current smokers are seven times more likely to have an AAA than nonsmokers with duration and quantity of smoking increasing risk. Smoking also increases AAA growth rate. Other risk factors include emphysema, hypertension, and hyperlipidemia.
A family history is a potent risk factor for AAA being present in about 20%
Matrix-degrading enzymes released by inflammatory cells lead to ___________________ and play a role in dilation and rupture.
Medial degeneration
AAA formation associates with chronic aortic wall inflammation, increased local expression of proteinases, and degradation of structural connective tissue proteins
Enzymes including ____________________ and elastolytic cathepsins degrade arterial matrix constituents contributing to aneurysm expansion and rupture.
Matrix metalloproteinases (MMPs)
In the absence of vasa vasorum, the nutrient supply to the media of the distal aorta depends on __________________, which may be jeopardized by intimal thickening and atherosclerotic plaque.
Diffusion from the lumen
Only ___________ of AAAs are noted on physical examination, although aneurysms larger than 5 cm can be detected in approximately 75% of patients, depending on body habitus
30% to 40%
Patients with AAA may have coexisting ________ (25%) and have an increased prevalence of iliac and popliteal aneurysms
TAA disease
_________________ can detect AAAs with high accuracy and is preferred over CT in screening for AAAs
Abdominal ultrasound
________________ is more accurate than ultrasound; especially useful in demonstrating the relationship of the AAA to the renal, visceral, and iliac arteries and patterns of mural thrombus, calcification, or coexisting occlusive atherosclerosis; useful for follow up in larger AAA
CT angiography (CTA)
The U.S. Preventive Services Task Force recommends a one-time ultrasound screening for AAAs in ____________________ and selective screening for those who never smoked
Men 65 to 75 years of age with a history of smoking
The Society for Vascular Surgery (SVS) recommends a one-time screening for AAAs in _________________ of AAAs.
All men ≥65 years and for women ≥65 years with a history of tobacco use or a family history
The Centers for Medicare and Medicaid Services will currently reimburse screening for ________________ of AAA.
Men 65 to 75 who ever smoked and men and women 65 to 75 with a family history
AAAs expand gradually and variably with an average growth rate of ___________________ per year and larger aortas grow more rapidly
2.2 mm (range 1 to 5 mm) per year
AAA ___________ is most important in predicting rupture
Diameter
The ________________ may predict risk better in women.
Aortic size indexed to body size
Factors associated with risk of AAA rupture include
Current smoking
Female gender
Emphysema
Hypertension
Immunotherapy after organ transplantation
Repair should be considered for asymptomatic aneurysms greater than
5.0 to 5.5 cm in diameter
Symptomatic aneurysms and those with rapid growth (_________) require more urgent consideration.
> 1 cm/year
AAA repair at smaller size (closer to ___________) should be considered for women due to increased risk of rupture.
5 cm
In patients with AAAs larger than 4.5 cm, _________ is preferred over ultrasound for more accurate measurement.
CT
The following surveillance imaging strategy for AAAs of various sizes has been proposed:
2.5 to 2.9 cm
3.0 to 3.9 cm
4.0 to 4.9 cm
5.0 to 5.4 cm
2.5 to 2.9 cm, every 7 years
3.0 to 3.9 cm, every 3 years
4.0 to 4.9 cm, every 1 year
5.0 to 5.4 cm, every 3 to 6 months
True or False
Patients with small AAAs should exercise regularly because moderate physical activity does not adversely influence the risk for rupture and may limit AAA growth.
True
AAA repair is recommended when the diameter exceeds _______ with earlier repair considered for those with rapid expansion, young age, and in women.
5.4 cm
AAA is treated surgically by either open surgical repair (OSR) or EVAR, with _______ associated with a threefold less perioperative mortality
EVAR (1.5% with EVAR vs. 4.2% to 5.2% for OSR)
Perioperative medical management to reduce cardiac risk may include continuation of beta blockers, statins, and/or aspirin.
Selection of the approach depends on the AAA anatomy, age, and risks associated with anesthesia and surgery,
Late complications develop in as many as _______ of patients after OSR for AAAs, including hernia and bowel obstruction, perianastomotic aneurysms (including pseudoaneurysms at suture lines and true aneurysms proximally), graft infection, graft-enteric fistula, and graft limb occlusions with lower extremity ischemia.
15% to 30%
OSR for AAAs should optimally be performed at centers with demonstrable operative mortality rates lower than 5%
After OSR patients should generally have annual clinical follow-up with CT at 5-year intervals, or more frequently if there are small aneurysms in adjacent vessels
In patients with suitable anatomy, EVAR offers a less invasive alternative to OSR. EVAR requires adequate ________________ attachment sites.
Nonaneurysmal proximal and distal
Long-term, multicenter, randomized, controlled trial comparing open repair with endovascular repair in 351 patients with an abdominal aortic aneurysm of at least 5 cm in diameter who were considered suitable candidates for both techniques. The primary outcomes were rates of death from any cause and reintervention
Six years after randomization, endovascular and open repair of abdominal aortic aneurysm resulted in similar rates of survival. The rate of secondary interventions was significantly higher for endovascular repair.
DREAM trial (Long-Term Outcome of Open or Endovascular Repair of Abdominal Aortic Aneurysm)
Long-term, multicenter, randomized, controlled trial comparing open repair with endovascular repair in 351 patients with an abdominal aortic aneurysm of at least 5 cm in diameter who were considered suitable candidates for both techniques. The primary outcomes were rates of death from any cause and reintervention
Six years after randomization, endovascular and open repair of abdominal aortic aneurysm resulted in similar rates of survival. The rate of secondary interventions was significantly higher for endovascular repair.
DREAM trial (Long-Term Outcome of Open or Endovascular Repair of Abdominal Aortic Aneurysm)
Randomly assigned 1252 patients with large abdominal aortic aneurysms (≥5.5 cm in diameter) to undergo either endovascular or open repair; 626 patients were assigned to each group. Patients were followed for rates of death, graft-related complications, reinterventions, and resource use until the end of 2009
Endovascular repair of abdominal aortic aneurysm was associated with a significantly lower operative mortality than open surgical repair. However, no differences were seen in total mortality or aneurysm-related mortality in the long term. Endovascular repair was associated with increased rates of graft-related complications and reinterventions and was more costly
EVAR-1 trial (Endovascular versus Open Repair of Abdominal Aortic Aneurysm)
It is reasonable to consider an ________ first strategy for younger, low-risk patients with long life expectancy.
OSR
Persistent blood flow in the aneurysm sac outside the endograft) develop in almost 25% of patients at follow-up with many requiring subsequent therapy
Endoleaks
The most common endoleak, result from retrograde filling of the aneurysm sac by aortic branch vessels, usually by the lumbar or inferior mesenteric arteries
Type II
Types of endoleaks and treatment
loss of complete sealing at the proximal or end of the stent graft, lead to increased pressure in the aneurysm sac and are associated with increased risk for rupture and therefore warrant repair
Type I
Caused by separation of components or disruption of the endograft fabric and require treatment, usually by re-lining with a stent graft.
Type III
Endoleaks are related to blood seeping through porous graft material and are self-limited.
Type IV
An enlarging AAA after EVAR without a demonstrated endoleak and with a diameter increased to greater than 10 mm, usually requires repair
Type V
Imaging with contrast-enhanced CTA is typically performed at ____________ after implantation of the device.
One month and annually
Aortic diameters ____________ in adults generally considered to be enlarged.
Greater than 40 mm
Prolonged _____________ exercise is associated with an increased prevalence of ascending aortic dilatation.
Endurance exercise
Most common (approximately 60%) of TAA
Aortic root or ascending aortic aneurysms
Followed by aneurysms of the descending aorta (approximately 35%) and aortic arch (<10%)
_______________ (approximately 10%) refers to descending thoracic aneurysms that extend distally to involve the abdominal aorta.
Thoracoabdominal aortic aneurysm
Many of the heritable disorders preferentially involve the ___________________, but some may involve the arch and descending aorta.
Aortic root and ascending aorta
Causes of TAAs include heritable disorders, congenital disorders, degenerative (atherosclerotic), mechanical,inflammatory,and infectious diseases.
Up to _______ of patients with AAA have either synchronous or metachronous TAA.
25%
__________ describes degeneration and fragmentation of elastic fibers, loss of SMCs, increase in deposition of collagen, and interstitial “cysts” of mucoid-appearing basophilic-staining extracellular matrix
Cystic medial degeneration (CMD)
Syndromic HTADs
MFS
LDS
vEDS
Nonsyndromic HTADs (also called familial TAA disorders) are due to mutations in multiple genes. 7 Up to 20% of individuals with a TAA will have a family history of TAA or will have an affected first-degree relative.
Give the corresponding gene affected
Marfan Syndrome
Loeys-Dietz Syndrome
Vascular EDS
Marfan Syndrome - FBN1
Loeys-Dietz Syndrome - TGFBR1, TGFBR2
Vascular EDS - COL3A1
MFS, an autosomal dominant disorder of connective tissue, results from abnormal fibrillin-1 due to mutations in the FBN1 gene.
Aortic dilation in MFS affects most prominently the _________________
Sinuses of Valsalva
____________ is the major component of the microfibril, a primary component of the extracellular matrix, and by interaction with lysyl oxidase (encoded by LOX), promotes vascular SMC adhesion and elastin support.
Fibrillin-1
___________mutations in multiple genes in the TGF-β signaling pathway, leads to craniofacial abnormalities (hypertelorism, bifid/broad uvula, cleft palate, craniosynostosis), arterial tortuosity, and aneurysms and dissections of the aorta and branch vessels. Cutaneous features include easy bruisability, visible veins, widened scars, and facial milia.
Loeys-Dietz Syndrome
___________ may have a more aggressive phenotype than TGFBR1 mutations and aortic surgery is recommended at aortic root dimensions of 4 to 4.5 cm.
TGFBR2 mutations
___________ due to SMAD3 mutations, involves severe osteoarthritis and osteochondritis dissecans, in addition to the vascular, skeletal, and cutaneous features of LDS and may merit aortic surgery at relatively small aortic root diameters and mutations in TGFB2 share features of MFS and LDS.
Aneurysms-osteoarthritis syndrome (AOS or LDS3)
____________ due to mutations in COL3A1 causing abnormal type III procollagen synthesis, associates with aortic and branch vessel aneurysm, rupture, and/or dissection and rupture of visceral organs at a young age leading to reduced lifespan
vEDS
Aortic root disease is less common in vEDS, with more frequent involvement of the _________________
Descending and abdominal aorta and branch vessel
Individuals with vEDS have risk for spontaneous arterial dissection and rupture, often involving medium-sized arteries that did not exhibit significant dilation.
_______________ are inherited as an autosomal dominant trait with decreased penetrance and variable expression (especially in women) and are more common than syndromic aortopathies.
Nonsyndromic HTADs (familial TAA)
__________ affects 1% of the population, associates with ascending aortic aneurysm, coarctation of the aorta, and aortic dissection
BAV
____________ due to helical flow patterns in the setting of BAVs may underlie the aortopathy of BAV disease
Abnormal aortic wall shear stress
The BAV exhibits abnormal leaflet folding, wrinkling, and increased leaflet doming, which can result in turbulence even in the absence of a stenotic or regurgitant lesion.
The aortic dilatation in BAV disease occurs most often in the ___________
Proximal to mid-ascending aorta
Ascending TAAs a ated with BAVs may develop independent of valve function and may develop late after aortic valve replacement (AVR).
________ underlies the aortic aneurysm and risk for dissection associated with BAVs.
CMD
When BAV and TAA coexist, CMD is more pronounced in ____________ than stenotic BAV.
Regurgitant
The lifetime risk of aortic dissection for the BAV patient is ____________ higher than the risk in the general population, but the absolute risk is very low unless aortic aneurysm is present.
4 to 8 times
____________ results from complete or partial loss of a second sex chromosome (XO, Xp)
Turner Syndrome
Patients with TS have an estimated ______________ for aortic dissection than do age-matched controls
100-fold greater risk
Women with TS but without risk factors for aortic dissection should undergo reevaluation of the aorta every _____________ or when clinically indicated (such as when contemplating pregnancy).
5 to 10 years
Most women with TS who suffer aortic d tion have risk factors, including aortic dilatation, BAV, coarctation of the aorta, or hypertension
TS patients have increased aortic diameter relative to body surface area and a higher risk for dissection at ________________.
smaller absolute aortic diameters
Degenerative TAAs occur most commonly in the _____________, have a _______ predominance (1.7 to 1), present at an average age of ________, and are associated with aortic __________________.
Descending aorta
Male
65 years
Atherosclerosis
These aneurysms tend to originate just distal to the origin of the left subclavian artery, may be either fusiform or saccular, and may extend into the abdominal aorta or coexist with AAAs.
Aneurysm formation develops during the chronic stage of dissection, being most common in the ________________
Proximal descending aorta
____________ the embryologic remnant located at the origin of an aberrant subclavian artery that may lead to aneurysmal dilatation, rupture, or aortic dissection.
Kommerell diverticulum
Surgical intervention is considered when the diverticulum diameter exceeds ________ and/or the diameter of the descending aorta adjacent to the diverticulum exceeds ___________
30 mm
50 to 55 mm.
Cardiovascular syphilis occurs in the tertiary stage and typically involves the _________________
Ascending aorta and arch
Pathologic features include lymphocytic and plasma cell inflammation in the adventitia, with the classic appearance of a “tree bark” or wrinkled appearance of the aortic intima.
Tertiary syphilis may cause aortic valvulitis, aortic regurgitation (AR), and coronary ostial stenosis.