Cardiovascular Pathology Etc. Flashcards
Vascultis associated with neutrophils
Behcet DZ
Vasculitis associated with eosinophils
Churg-Strauss DZ
Which is the only vasculitis involving the aorta?
Giant Cell Arteritis
“Pauci-immune” means
Abs direted against cellular constituents and do NOT form circulating immune complex
3 major histological features of Giant cell arteritis
Multinucleated giant cells
Fragmented elastic lamina and intimal thickening
Patchy and focal sites of involvement
What kind of vasculitis is Takayasu arteritis?
Granulomatosus vasculitis of medium and large arteries.
-Pulmonary, Coronary, Renal aa.
Age group common for Takayasu arteritis
<50 y/o
What causes the pulseless disease in Takayasu arteritis?
Narrowing of brachiocephalic, carotid and subclavian aa.
In what disease are pulmonary vessels spared from vasculitis?
PAN
What is Segmental Transmural necrotization?
In which diseases is it found?
Necrotizing inflammation w/ a predilection for branch points.
PAN and Microscopic polyangiitis
SX of PAN
Rapidly accelerating HTN
Abdominal pain an bloody stool
Diffuse myalgia and neuritis.
Renal involvement is major COD.
How can Kawasaki DZ lead to acute MI?
Affected sites may form aneurysms -> thrombosis or rupture -> acute MI
Presenting SX of Kawasaki DZ
What is the Tx?
Erythema of conjunctiva, oral mucosa, palms and soles.
Desquamative rash.
IVIg and aspirin.
What are the major targets of Microscopic polyangiitis?
Renal glomerulus and lung capillaries
SX of Microscopic polyangiitis
Hemoptysis
Hematuria, proteinuria
Cutaneous purpura
Major COD of Churg-Strauss syndrome
What are SX?
Cardiomyopathy
Asthma, rhinitis, hypereosinophilia.
Purpura, GI bleed, renal DZ.
Genetic marker of Behcet DZ
HLA-B51
What is the major COD for Behcet DZ?
Disease mortality related to severe neurological involvement and rupture of aneurysms.
Age group of Granulomatosis w/ polyangiitis
M>F, avg. age of 40
SX of Granulomatosis w/ polyangiitis
Pneumonitis
Sinusitis
Renal DZ
Nasopharyngeal ulceration
morphology of Granulomatosis w/ polyangiitis
URT: granulomas w/ geographic patterns of central necrosis and vasculitis.
LRT: cavitory granulomas.
Major fashions for vascular invasion of infectious vasculitis (2)
Local tissue invasion
Hematogenous spread
Primary Raynaud phenomenon
Induced by cold or emotion w/ symmetric involvement of digits.
Mostly young women. Benign course.
Secondary Raynaud phenomenon
A component of arterial DZ like SLE, scleroderma, thromboangiitis, etc.
Asymmetric involvement of digits. Worsens w/ time.
“Cardiac Raynaud”
Excessive vasoconstriction of myocardial aa. or arterioles that may cause ischemia or infarct.
Primary lymphedema
Isolated congenital defect leads to Lypmphedema (Milroy DZ)
Secondary lymphedema
Blockage of previously normal lymphatic flow. Due to cancer, surgery, radiation, infection, etc.
Markers to check via IHC for vascular malignancies
CD31 or von Willebrand factor (vWF)
Telangiectasia
Permanent dilation of preexisting small vessels that form red lesions usually in skin or mucous membranes.
These are not true neoplasms.
Sturge-Weber syndrome
A special form of nevus flammeus (port wine stain) in the distribution of the trigeminal n.
Capillary hemangioma
Thin-walled capillaries tightly packed together (most common).
Cavernous hemangioma
Irregular, dilated vascular channels making a lesion w/ indistinct border.
More likely to involve deep tissue or bleed.
Pyogenic granuloma
Type of capillary hemangioma.
Grow fast in oral mucosa. May develop from trauma.
Ex: Granuloma gravidarum: gingiva of pregnant women (tumors)
Cavernous lymphangioma
Cystic hygroma.
Usually in neck or axilla of kids.
Turner syndrome.
Simple lymphangioma
Appear similar to capillary hemangiomas, just no RBC.
What happens morphologically in bacillary angiomatosis?
Vascular proliferation to G- Bartonella. Forms capillaries w/ plump endothelial cells.
Who does Bacillary angiomatosis affect?
What kind of lesions form?
What stain is used commonly?
What is a good Tx?
Immunocompromised pts.
Red papules.
Warthin-Starry stain.
Macrolide abx.
Morphology of Epithelioid hemangioendothelioma
neoplastic endothelial cells are cuboidal and resemble epithelium. Vascular channels may be hard to recognize.
Most common inducer of Angiosarcoma
Where is the cancer found typically?
Radiation
Skin, ST, breast and liver
What disease is associated with Berry aneurysms?
AD polycystic kidney DZ
Renovascular HTN
Fibromuscular dysplasia of renal aa.
What kinds of HTN are hyaline and hyperplastic arteriosclerosis associated with?
Hyaline - chronic HTN
Hyperplastic - severe HTN
Nephrosclerosis
Chronic HTN leadnig to hyaline arteriosclerosis and impairment of renal blood supply and glomerular scarring
Necrotizing arteriolitis
Fibrinoid deposits and vessel wall necrosis (usually in kidney) that leads to onion skinning and hyperplastic arteriorsclerosis as a result of malignant HTN.
Where does a plaque generally exist?
Above the internal elastic lamina (between lamina and inner media).
2 “models” used to describe how atherosclerosis develops
Hemodynamic model - due to lesions near bifucations.
Circulating lipids - accumulation of choleserol which is metabolized by Mo and a fatty streak is made.
False aneurysm
Defect thru the wall of a vessel that communicates w/ and extravascular hematoma that communicates with the lumen of the vessel.
Creates a pulsating hematoma.
Atherosclerosis causes ischemia of the ______ media
Inner
HTN causes ischemia of the ______ media
Outer
What valvular DZ can accompany tertiary syphilis?
Aortic regurg
Serous pericarditis
Produced by non-infectious inflammatory Dzs, like RF, SLE, and scleroderma.
Purulent/suppurative pericarditis
Active infection caused by microbial invasion of pericardial space.
Hemorrhagic pericarditis
Exudate composed of blood mixed w/ a fibrous or suppurative effusion.
*most commonly caused by malignancy.
Caseous pericarditis
From tuberculosis. Rarely can be from fungi.
Constrictive pericarditis
Heart becomes encased in a dense, fibrous or fibrocalcific scar that limits diastolic expansion and CO (mimics restrictive CM).
Cardiac lipoma
Localized, well-circumscribed benign tumors composed of mature fat cells; occurs in eubendocsrdium, subepicardium or myocardium.
Papillary fibroelastoma
Usually incidental, sea-aneomone-like lesions, mostly found on autopsy. 80% found on valves.
-Resemble Lambl excrescences.
50% of Rhabdomyomas are associated w/ what DZ?
What are the mutations?
Tuberous sclerosis
TSC1 (Hamartin) or TSC2 (Tuberin)
How are primary arrhythmic DOs detected?
Genetic testing
How does the heart morphology of Cor Pulmonale (RSHD) change in chronic vs. acute presentations?
Chronically - RV hypertrophy
Acute - usually from a PE. Marked dilation of RV w/o hypertrophy
What valvular DZ is most commonly caused by chronic RHD?
Mitral stenosis, as well as chordae thickening and cusp fusion
In what settings is functional mitral valve regurg clinically important?
IHD
DCM
Most common causes (2) of mitral regurg
Abnormalities of leaflets and commissures
MV prolapse
Most common cause of aortic stenosis
Calcification of congenitally deformed valve
Most common causes (3) of aortic regurg
Aortic insufficiency
Syphilitic aortitis
Marfan’s syndrome
Age group for calcific aortic stenosis
SX of calcific aortic stenosis
Tx?
Increases w/ age usually from 60-80 y/o
Angina, CHF, syncope, pulm. edema
Surgical repair
Doxyrubicin chemotherapy is associated w/:
DCM
Hereditary hemochromatosis is associated w/:
DCM
Mitral annular calcification is usually ASX, but in some cases it can cause:
Arrhythmias
Regurg
Stenosis
What marker is elevated first in an MI?
Myoglobin > CK-MB > Troponin
What is myxomatous degeneration? And what valvular DZ is it associated w/?
Proteoglycan deposits and elastic fiber disruption of the valve leaflets. They become thickened and rubbery.
MVP
What malformation is associated w/ “nothing” of ribs?
Coarctation of aorta w/ PDA
When does acute RF occur post infection?
10 days to 6 wks
RF SX
Pancarditis
Migratory polyarthritis
Erythema marginatum
Syndenham chorea
Chronic RHD leads to which valvular abnormality?
What happens to the heart structurally as a result?
MV stenosis due to leaflet thickening, commissure fusion, and thickening of cords.
LA enlargment
RHF/pulm congestion -> RV hypertrophy
IE
3 major associations to DCM
EtOH
Genetics
Hemochromatosis
Anitschkow “caterpillar” cells
Immune cells that clump to form Aschoff bodies in pandcarditis due to *acute RF.
Cardiac features of Acute RF (3)
Pancarditis
Fibrinoid necrosis of endocardium and left-sided valves
Verrucae
Acute IE infects a ____ valve
Chronic IE infects a ____ valve
Normal valve
Abn. valve
IE usually affects which sided lesions?
Left-sided
Carcinoid heart DZ usually affects which sided lesions?
Right-sided, as the pulm vascular bed degrades mediators.
Usually liver catabolizes mediators before they affect the heart, so there is a huge hepatic burden.
Titin is associated w/ what 2 disease processes?
DCM
Myocarditis
What exists within fatty streaks early on?
In a plaque?
Foam cells and T cells
Cholesterol clefts and Mo
Fibrillin deficiency is associated w/:
Marfan syndrome
Familial syndromes associated w/ myxomas have activating mutations in the ______ gene or null mutations in ______. Which 2 diseases/complexes are associated w/ each mutation?
GNAS1 - McCune-Albright syndrome
PRKAR1A - Carney complex
3 Left-to-right shunts
ASD
VSD
PDA
Secundum ASD
Primum anomalie ASD
Sinus venosa ASD
Secundum ASD - most common, at center of septum
Primum anomalie ASD - 5%, adjacent to AV valves
Sinus venosa ASD - 5%, near entrance on SVC
Explain what changes take place to the heart 3-4 days post MI, 7-10 days and 10 days
3-4 days: PMNs
7-10 days: Mo
10 days+: granulation
When does cTnT peak?
When does cTnI peak?
cTnT: 12-48 hrs
cTnI: 24 hrs
Both begin to rise approx 3-12 hrs post MI
When does cTnT peak?
When does cTnI peak?
When does CK-MB peak?
cTnT: 12-48 hrs
cTnI: 24 hrs
CK-MB: 24 hrs
All begin to rise approx 3-12 hrs post MI
