Vascular Path Flashcards
Is SBP/DBP more important in determining CV risk?
SBP
Postcapillary Venules
Sites of leukocyte exudation and vascular leakage
The primary regulators of arterial blood pressure are…
arterioles and small muscular arteries
Weibel-Palade Bodies
Membrane bound storage vesicles within endothelial cells that contain vWF
Discontinuous Capillaries are located
Liver
Bone marrow
Spleen
Continuous Capillaries are located
Fat
Muscle
CNS/PNS
Fenestrated Capillaries are located
Intestinal villi
Endocrine glands
Kidney glomeruli
Benign/Essential HTN
Controlled HTN with no short term problems
Hypertensive Urgency
> 220/120 with no organ damage
Hypertensive Emergency
Accelerated HTN
Significant increase in BP with organ damage
Hypertensive Emergency
Malignant HTN
Significant increase in BP with organ damage and papilledema
Licorice is bad because
It is structurally similar to aldosterone and then allows for Na retention
Renal a. Stenosis –>
Increased renin secretion due to perceived hypovolemia
Increased Ang2 –> Increased Aldosterone
Vasoconstriction
Increased BP
Treat with surgery to correct stenosis
ENaC control reabsorption of only
2% of Na
Liddle Syndrome
Recessive mutation in PHA1 which causes an increased ENaC resorb and K secrete
Primary Prevention
Prevent onset of disease in at risk pt –> susceptible
Secondary Prevention
Early diagnosis and risk factor assessment –> asymptomatic
Tertiary Prevention
Prevent recurrences with disease –> symptomatic
Areas prone to atherosclerosis
Turbulent flow and low shear stress
Occlusion must be >__ before Sx occur
70%
Multiple atherosclerotic risk factors…
Are compounding –> 2 lead to 4x risk while 3 lead to 7x the risk
Metabolic Syndrome
Hyperlipiemia Insulin resistance Obesity HTN (hypercoagulable and inflame state)
Young obese woman presents with RUQ pain
Cholelithiasis
Statins 2 functions in cholesterol metabolism
Inhibits HMG-CoA reductase and thus synthesis of cholesterol
Promote synthesis of LDL receptors
Estrogen replacement therapy does/does not decrease risk for MI in post menopausal women
DOES NOT
Best predictor of risk for CVD?
C-Reactive Protein
Infections that may contribute to atherosclerosis
Chylamydia pneumonia
cytomegalovirus
herpesvirus
True aneurysm
Bounded by arterial wall component or myocardium
Saccular aneurysm (True)
appears rounded (berry aneurysm)
Fusiform aneurysm
True
involves long segment of artery, not rounded, common in aorta
False aneurysm (Pseudoaneurysm)
Hematoma secondary to transmural rupture (vessel popped and hematoma outside vessel)
Using NSAIDs does what for CVD risk factors?
Inhibiting COX2 prostacyclin release by endothelium due to NSAIDs use leads to a high proportion of COX1 thromboxane A2 leading to a pro-thrombotic state
When does a AAA require surgery?
When the size become 5cm or greater
What is the mutation in Marfan’s and how does it cause the clinical features?
Loss of function mutation of fibrillin in the FBN1 gene
With decreased fibrillin 1 there is an increased TGF-B activity and serum levels which leads to overgrowth of bones
What was the mutation of Marfan’s once thought to be?
Once thought to be a dominant negative mutation but it’s not!! Autosomal dominant and sporadic pattern
Mutations in TGF-B receptors is called
Loeys-Dietz syndrome
Giant Cell Arteritis
Temporal arteritis Granulomatous inflamm Large aa. vasculitis >50 y/o Assc. polymyalgia rheumatica Permanent blindness/diplopia 2/3 have anti-end or anti-SM Ab
Takaysu Arteritis
"Pulseless disease" Granulomatous inflamm of aorta and major branches <40 Weakening of pulses Reduced BP in UE
Polyarteritis Nodosa
Medium sized vessel vasculitis Necrotizing inflamm Young adults Renal aa. involvement w/o glomerulonephritis 30% with HepB Ag/Ab complex palpable purpura Malaise, HTN, melena, infarcts, weight loss, Abd pain, fever Tx: steroids and cyclophosphamide
Kawasaki Disease
80%<4y/o Anti-endo and anti-SM Ab Involves coronary aa. Strawberry tongue Erythema/desquam rash on soles and palms Mucocutaneous lymph node syndrome conjunctival and oral erythema
Microscopic polyangitis
Leukocytoclastic vasculitis Necrotizing vaculitis (Pauci-Immune) p-ANCA (MPO-ANCA) Palpable purpura Necrotizing glomerulonephritis Pulmonary capillaries Hemoptysis
Churg-Strauss
Eosinophil-rich granulomatous necrotizing vasculitis
Involves RT, strong assc. w/allergic rhinitis, bronchial asthma, lung infiltrates
Blood esosinophilia
p-ANCA (MPO-ANCA)
Coronary artertitis
Eosinophilic myocarditis both cause morbidity and mortality
Behcet Disease
Neutrophilic vasculitis 1) Recurrent oral aphthous ulcers 2) Genital ulcers 3) Uveitis HLA-B51 \+ pathergy test (minor trauma results in exaggerated rxn)
Granulomatosis with polyangiitis
Wegener’s granulomatosis
1) Acute necrotizing granulomas of URT LRT
2) Granulomas of vessels in lungs, upper airways and eyes/skin
3) Renal disease with focal necrotizing, crescentic, glomerulonephritis with hematuria/proteinuria/renal failure
c-ANCA(PR3-ANCA)
Proteinase 3 in azurophilic granules
Granulomatosis with Polyangitis Clinical Featuers
M>F ~40 yrs Persistent pneumonitis, bilateral nodular and cavitary infiltrates Chronic sinusitis Mucosal ulcerations of nasopharynx Renal disease Tx: immunosuppresion
Lymphatoid Granulomatosis
Not a vasculitis Often presents similar to Wegner's Pulmonary infiltrates with lymphoid and plasmacytoid cells and cellular atypia Infiltrates invade vessel walls Evolving lymphoproliferative disorder 50% develop lymphoidmalignancy
Thomboangitis Obliterans
Segmental, thrombosing, acute and chronic inflammation which can involve veins and nerves
Often occurs in heavy smokers s
Severe pain even at rest due to nerves
Rheumatoid vasculitis
Clincially significant aortitis
Visceral Infarction
Infective Vasculitis
Direct invasion of pseudomonas or Aspergillus and mucromycosis
Can result in mycotic aneurysm
Hypothermia
Vasoconstriction and increased permeability leads to edema
Alcohol inducing vasodilation
Frostnip
Vasoconstriction involving nose, ears, hands and feet
Chillblain (Perniones/Perniosis)
Nonfreezing temperatures and damp conditions
Chronic recurrent vasculitis with red raised lesions
Immersion
Trench Foot
Feet wet but not freezing
May not heal, chronic pain, edema, blotchy discoloration
Superficial moist, liquefactive gangrene
Frostbite
Sudden sharp drops in temperature that are persistent
Vasoconstriction and increased viscosity
Hyperemia, edema, large clear blisters, vesicles filled with hemorrhagic fluid to complete gangrene
Raynaud Phenomenon
Primary due to emotion or cold induced vasoconstriction
Secondary due to arterial insufficiency caused by other conditions (SLE, systemic sclerosis, atherosclerosis, Buergers)
Red –> White –> Blue (fingertips)
Chronic Venous Insufficiency
Bilateral dusky brown coloring
Chronic edema
Ulcerations due to increased pressure
Chronic congestion
Phlebosclerosis
Elastic tissue degeneration and spotty calcifications in the media
Varicose veins
Dilated tortuous veins (elevated intraluminal pressure and loss of vessel wall support) Vavlvular incompetence Pedal edema >50 yrs old, obese, women Can develop stasis dermatitis Variation in thickness of vein wall
Lymphangitis
Infections w/i lymphatics
Group A Beta hemolytic streptoccoci
Cause cellulitis/focal abscesses
Red streaks with painful enlarged regional lymph nodes
Lymphedema
Primary 1) Isolated congenital defects 2) Familial Milroy disease presenting with lymphatic agenesis or hypoplasia 3) lymphedema praecox Secondary Obstructive
Chronic edema leads to
Thickended skin, brawny induration or peau d’orange
Chylous acites
Chylotrhrax
Chylopericardium
Caused by rupture of obstructed dilated lymphatic into the peritoneum, pleural cavity or pericardium
Angiomatosis
Involves large segments of the body
such as entire extremity
Vascular Ectasis
Localized dilation of preexisting vessels
Glomus Tumor
Benign often painful tumor
From modified cells of glomus body
Often presents on distal digit
Speaialized glomus cells painful to touch
Cavernous Lymphangioma
Cystic Hygroma
Presents in children in neck/axillary
Assoicated with Turner syndrome 45X
Cavernous Hemangioma
Large, less circumscribed, involving deep structures
No tendency to regress
Locally destructive
Hemangioblastomas
Present in VHL Disease
Angiomatous lesions of cerebellum, brainstem and eye
Cystic neoplasms of pancreas and liver
Capillary hemangioma
Most common vascular tumor
Juvenile hemangioma
Strawberry type
Occurs in 1/200 newborns
May grow rapidly in first few months and then regresses in 75-90% cases by 7 y/o
Hemangiomas
Malignant transformation rare
Common in skin and liver
Pyogenic granuloma
Rapidly growing red nodule on finger or lips
Bleed easily and ulcerate
Develop after trauma
Can present in gingiva in pregnancy
Telangiectasia
Congenital anomly or acquired permanent exaggeration of preformed vessels
Nevus Falmmeus
Most common form of ectasia
Leptomenignes
Inner two menignes
Pia and Arachnoid mater
Port Wine Stain
May grow proportionately with child with no tendency to fade
Sturge-Weber Syndrome
Ipsilateral port wine stain in trigeminal nerve distribution
Assc. Sx: MR, seizures, hemiplegia, radiopacities in skill
Venous angiomatous masses in leptomeninges
Spider Telangiectasia
Dilated subQ aa.s around a central core that blanches with pressure
Occurs in states of hyperesterinism (pregnancy & cirrhosis)
Presents on face neck and upper chest
Hereditary Hemorrhagic Telangiectasia
Congenital dilation of capillaries
Presents widespread at birth on skin and mucosa
Pt have tendency to bleed due to dilated capillaries
Autosomal dominant mutation in TGF-B signaling pathway
Bacillary Angiomatosis
Opportunistic infection caused by gram negative Bartonella species (B.hensale and B.quintana)
HIF-1a induction by bacteria causes increased VEGF
Treat with erythromycin
Kaposi Sarcoma
Caused by HHV-8 which infects endo cells G protein induced by virus induces VEGF Hyaline globules may be found in cells Most infxns are asymptomatic Raised red-purple discoloration that progresses form flat lesion to plaque(w/RBC extravasation) to nodule that ulcerates
Epithelioid Hemangioendothelioma
Vascular tumor presents around medium-large vv. in soft tissue
Tumor cells plump and cuboidal
Tx with excision but 40% recur, 20-30% metastasis and 15% of metastasis lead to death
Angiosarcoma
Malignant well differentiated- anaplastic
Local invasion and metastasis common with 30% 5 year survival after metastasis
Induced by radiation, foreign material
Immunohisto: CD31, vWF, CD34 Factor 8
Present in skin, soft tissue, breast, liver
Hepatic angiosarcomas
Caused by carcinogens, arsenic, vinylyl chloride, thorotrast
Lymphangiosarcoma
Arise in chronic lymphedema
Hemangiopericytoma
Derived from pericytes
Staghorn branching pattern
Found pelvic retroperitonel or LE of middle aged women
half malignant half benign
Ballon Angioplasty Complications
Complications
1) Abrupt reclosure
2) Proliferative in-stent restenosis
Vascular Stent Complications
Early thrombosis (use platelet antagonists) Late intimal thickening (use anti proliferative drugs)
Neointima
nothing
