CHAPTER 22

AORTIC ANEURYSMS and GENETIC DISORDERS

18. It is recently identified autosomal dominant disorder. Patients with this syndrome suffer from aortic
and arterial aneurysms, arterial tortuosity, aortic dissection, mild craniofacial abnormalities, and early
onset osteoarthritis
A. Marfan Syndrome
B. Loey-Dietz Syndrome
C. Aneurysms-Osteoarthritis Syndrome
D. Ehlers-Danlos Syndrome

Answer: C
Marfan Syndrome is an autosomal dominant genetic disorder characterized by a specific connective
tissue defect that leads to aneurysm formation. The aortic wall is weakened by fragmentation of elastic
fibers and deposition of extensive amounts of mucopolysaccharides (a process previously called cystic
medial degeneration or cystic medial necrosis).
2. Loeys-Dietz Syndrome - is phenotypically distinct from Marfan syndrome characterized as an
aneurysmal syndrome with widespread systemic involvement
3. Ehlers-Danlos Syndrome - includes a spectrum of inherited connective tissue disorders of collagen
synthesis
 
19. It is the most common congenital malformation of the heart or great vessels, affecting up to 2% of
Americans patients with bicuspid aortic valve have an increased incidence of ascending aortic aneurysm
formation and, often, a faster rate of aortic enlargement.
A. Familial Aortic Aneurysms
B. Aneurysms-Osteoarthritis Syndrome
C. Congenital Bicuspid Aortic Valve
D. Bovine aortic arch

ANSWER: C
Familial Aortic Aneurysms - Families without the heritable connective tissue disorders described earlier
also can be affected by genetic conditions that cause thoracic aortic aneurysms.
Aneurysms-Osteoarthritis Syndrome - recently identified autosomal dominant disorder. Patients with
this syndrome suffer from aortic and arterial aneurysms, arterial tortuosity, aortic dissection, mild
craniofacial abnormalities, and early onset osteoarthritis.
 Bovine aortic arch - a common origin of the innominate and left common carotid arteries; has been
considered a normal anatomic variant.

20. Diagnostic evaluation for aortic aneurysm that is widely available, provides visualization of the entire
thoracic and abdominal aorta, and permits multiplanar and 3-dimensional aortic reconstructions.
A. Computed Tomography
B. Plain Radiography
C. Magnetic Resonance Angiography
D. Abdominal Ultrasonography

ANSWER: A
Plain Radiography - Plain radiographs of the chest, abdomen, or spine often provide enough
information to support the initial diagnosis of thoracic aortic aneurysm.
 Echocardiography and Abdominal Ultrasonography
  Computed Tomography - Computed tomographic (CT) scanning - is widely available, provides
visualization of the entire thoracic and abdominal aorta, and permits multiplanar and 3-dimensional
aortic reconstructions. It is the most common—and arguably the most useful—imaging modality for
evaluating thoracic aortic aneurysms
Magnetic Resonance Angiography -is becoming widely available and can facilitate visualization of the
entire aorta

21. Illustrations of proximal aortic repairs in which the native aortic root is left intact, EXCEPT:
A. Modified hemiarch with additional graft replacement of the innominate artery.
B. Hemiarch beveled graft replacement, in which the ascending aorta and a portion of the lesser
curvature of the aortic arch are replaced.
C. The branched graft approach, which replaces the brachiocephalic vessels by following their original
anatomic location.
D. The elephant trunk approach with a concomitant island common carotid artery reattachment.

ANSWER: D
The elephant trunk approach with a concomitant island BRACHIOCEPHALIC artery reattachment.

22. Options for graft repair of the aortic aneurysm:

A. Descending replacement
B. Total arch replacement with unattachment of brachiocephalic branches
C. Patch aortoplasty
D. Beveled total arch replacement

ANSWER: C
Patch aortoplasty
Ascending replacement only
Beveled hemiarch replacement
Total arch replacement with reattachment of brachiocephalic branches
Total arch replacement with bypass grafts to the brachiocephalic branches (Y-graft approaches)
Elephant trunk technique with island reattachment
Elephant trunk technique with Y-graft approach

23. True of Hybrid repair

A. Involve some form of “debranching” of the brachiocephalic vessels
B. Endovascular exclusion of some or all of the aortic arc is done
C. Involve sewing a branched graft to the proximal ascending aorta with the use of a partial aortic clamp
D. ALL OF THE ABOVE

ANSWER: D
Hybrid Repair
Unlike purely endovascular approaches, hybrid repairs of the aortic arch have entered the
mainstream clinical arena, although they remain controversial. Hybrid arch repairs involve some form of
“debranching” of the brachiocephalic vessels (which are not unlike Y-graft approaches), followedby
endovascular exclusion of some or all of the aortic arc. Although this technique has many variants, they
often involve sewing a branched graft to the proximal ascending aorta with the use of a partial aortic
clamp. The branches of the graft are then sewn to the arch vessels. Once the arch is “debranched,” the
arch aneurysm can be excluded with an endograft.

24. Which of the following describes aortic aneurysm?

A. It is a permanent, localized dilatation of the aorta to a diameter that is at least 30% greater than is
normal at that anatomic level
B. Aneurysms of the thoracic aorta consistently decrease in size and eventually progress to cause serious
complications
C. Aneurysms can be localized to a single aortic segment, or they can involve multiple segment.
D. ALL DESCRIBES AORTIC ANEURYSM

ANSWER: C
Aortic Aneurysm - is defined as a permanent, localized dilatation of the aorta to a diameter that is at
least 50% greater than is normal at that anatomic level.
- annual incidence : 5.9 per 100,000 persons
- Aneurysms of the thoracic aorta consistently increase in size and eventually progress to cause
serious complications.These include rupture, which is usually a fatal even.
- Aneurysms can be localized to a single aortic segment, or they can involve multiple segment.

25. Causes of aortic aneurysm, except:

A. Aortitis
B. Uncontrolled hypertension
C. Infection
D. Trauma

ANSWER: B
Causes:
 Degenerative disease of the aortic wall
 aortic dissection
 aortitis
 infection
 trauma
26. ANATOMY: Area from which the brachiocephalic branches arise
A. Ascending aorta
B. Descending thoracic aorta
C. Transverse aortic arch
D. Aortic arch

ANSWER: C
Ascending aorta - begins at the aortic valve and ends at the origin of the innominate artery
Descending thoracic aorta - begins distal to the origin of the left subclavian artery and extends to the
diaphragmatic hiatus, where it joins the abdominal aorta

27. What is the most common cause of thoracic aortic disease?

A. Nonspecific Medial Degeneration
B. Aortic dissection
C. Marfan Syndrome
D. Familial Aortic Aneurysms

ANSWER: A
Nonspecific Medial Degeneration - nonspecific medial degeneration is the most common cause of
thoracic aortic disease.
Aortic Dissection - usually begins as a tear in the inner aortic wall, which initiates a progressive
separation of the medial layers and creates two channels within the aorta.
Marfan Syndrome - an autosomal dominant genetic disorder characterized by a specific connective
tissue defect that leads to aneurysm formation.
Familial Aortic Aneurysms - Families without the heritable connective tissue disorders described earlier
also can be affected by genetic conditions that cause thoracic aortic aneurysms.

28. Seen in the thoracic aorta usually represent chronic leaks that are contained by surrounding tissue
and fibrosis
A. Primary infection
B. Aortitis
C. Pseudoaneurysm
D. Fusiform aneurysms

ANSWER: C
Infection - Primary infection of the aortic wall resulting in aneurysm formation is rare. Although these
lesions are termed mycotic aneurysms, the responsible pathogens usually are bacteria rather than fungi.
 Aortitis - In patients with preexisting degenerative thoracic aortic aneurysms, localized transmural
inflammation and subsequent fibrosis can develop
 Fusiform aneurysms - can be described as symmetrical dilatations of the aorta.
29. Preoperative assessment and preparation done prior to surgical intervention for aortic aneurysm,
except:
A. CARDIAC
B. VASCULAR
C. PULMONARY
D. RENAL

ANSWER: B
Preoperative Assessment and Preparation
 Cardiac Evaluation
 Pulmonary Evaluation
 Renal Evaluation

30. Options for open surgical repair of proximal aortic aneurysms and for treating aortic valve disease,
EXCEPT:
A. Aortic valve replacement with mechanical or biologic prosthesis
B. Composite valve graft
C. Stentless porcine root
D. Aortic allograft

ANSWER: D
Options for open surgical repair of proximal aortic
aneurysms:
A. Options for treating aortic valve disease
 Aortic valve annuloplasty (annular plication)
 Aortic valve replacement with mechanical or biologic prosthesis
 Aortic root replacement
 Composite valve graft
 Aortic homograft
 Stentless porcine root
 Pulmonary autograft (Ross procedure)