Clinical Perspective of Aortic Disease Flashcards
1
Q
4 Main Pathological Processes leading to Disease of the Aorta
A
- Cystic Medial Degeneration
- Atherosclerosis
- Inflammatory Disorders
- Trauma
2
Q
Cystic Medial Degeneration
A
- Very Important predisposing factor to disease of aorta
- Can be genetically determined
- Most important in ascending aortic disease
- Characterized by:
*smooth muscle cell necrosis
*smooth muscle cell apoptosis
*degeneration of elastic fibers within the media
*cystic spaces develop
*extends to the elastic components of the adventitia
- Weakens the aortic wall
- Prone to aneurysm/dissection
3
Q
Genetic Diseases Predisposing to Cystic Medial Degeneration
A
- Marfan syndrome
- Ehlers-Danlos syndrome
- Loeys-Dietz syndrome
- Familial aoritc aneurysm and/or dissection syndromes
- Bicuspid aortic valve
- Autosomal dominant polycystic kidney disease
- Neurofibromatosis
- Turners syndrome
4
Q
Marfan Syndrome
A
- Mutation in fibrillin 1 gene (FBN1)
- Dilation and aneurysm of the aortic root, dilation of the pulmonary artery, and dilation or dissection of the descending throacic or abdominal aorta
- Autosomal dominant disorder of the connective tissue
- 2-3 in 10,000
- Also seen in musculoskeletal and ocular systems, much less frequent in pulmonary, nervous, and integumentary fibrils
- 80% morbidity linked to aortic aneurysm and dissection
- Ghent criteria, Z-scores, genetic testing
5
Q
Ehlers-Danlos Syndrome
A
- Mutations in COL5A1, COL5A2 and COL3A1 genes
- Arterial mid-sized rupture, specially involving thoracic or abdominal vessels. Frequently descending and abdominal aorta
- Autosomal dominant, recessive autosomal, or x-linked patterns
- Prevalence 1 in 10,000 depending on type
- Characterized by articular hypermobility, skin extensibility, tissue fragility
- Classification: 6 types
6
Q
Loeys-Dietz Syndrome
A
- Mutation in TGFBR1 and TGFBR2 genes
- Premature and aggressive aneurysms and dissections. Aneurysms distal to the aortic root
- Dominant autosomal
- No clinical diagnostic criteria
- Genetic testing is helpful
- Mean survival is 37yrs/first vascular procedure age 20
7
Q
Bicuspid Aortic Valve
A
- Most common congenital heart malformation, 1-2% in gen pop.
- Unknown aortic dilation typically involves the aortic root and ascending aorta whereas it is not present in the descending and abdominal aorta
8
Q
Autosomal Dominant Polycystic Kidney Disease
A
- Mutation in PKD1 and PKD2 genes
9
Q
Neurofibromatosis
A
- Aneurismal arterial disease affecting the renal arteries and abdominal aorta
10
Q
Turners Syndrome
A
- Dilation of the aortic root and dissection of the thoracic aorta
11
Q
Aortic Dissection Process
A
- Splitting of aortic wall in the media
- Formation of false lumen that courses along w/ true lumen
- Intimal tear starts the dissection, allows pulsatile high pressure flow into the media
- Also there can be primary rupture of the vaso vasorum and resultant intramural hematoma
12
Q
Aortic Dissection Classifications
A
- DeBakey
- Stanford
13
Q
Aortic Dissection Treatments
A
- Acute type A dissections are surgical emergencies
*very high mortality rates up to 1-2% per hr.
- Beta Blockade +/- sodium nitroprusside for both types
- Type B dissections are treated medically unless complications; assoc. w/ high mortality rates
14
Q
Intramural Hematoma
A
- Non-communicating blood collection in the aortic wall/media
- May be related to rupture of vaso vasorum
- Can communicate w/ adventitial space and lead to rupture or dissection w/ intimal tear
- If small, benign and resolve
15
Q
Penetrating Aortic Ulcer
A
- Develops when aortic atheromatous plaque erodes into the aortic media
- Disruption of internal elastic lamina -> extension into media
- Can form intramural hematoma, saccular aneurysm, pseudoaneurysm, or rupture
- Elderly, hypertensive (>90%) pts
- Depeding on location, observation and resolution
18
Q
Crawford Aortic Aneurysm Classification Illustration
A
