Vascular Path Flashcards by Brock Wiley

Diagnosis?

Thick walled arteries and veins
Pulsitile tangle of worm like vessels
Can occur in the skin, GI tract, lungs and brian

Arteriovenous malformation

*Large AVMs shunt blood from A to V circulation, leading to high output cardiac failure

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Diagnosis?

Focal dilation of an artery due to a defect in the media
Typically appear in Circle of Willis (pt presents with worst headache of their life with N/V, diplopia, loss of consciousness
Risk factors are HTN and smoking
Seen in polycystic kidney disease, Marfan, Ehlers-Danlos

Berry (saccular) aneurysm

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Diagnosis?

Sceptic emboli from infective endocarditis
Extension of infectious process into the artery

Mycotic aneurysm

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Diagnosis?

Young woman
String of beads angiography of renal arteries
Renovascular hypertension (epigastric bruits)

Fibromuscular dysplasia

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Diagnosis?

Idiopathic HTN
Unmodifiable risk factors: age, genetics
Modifiable risk factors: stress, obesity, physical activity, salt consumption

Primary (essential) HTN

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Diagnosis?

HTN due to
Renovascular disease
Endocrine disorder (primary hyperaldosteronism, Cushjing, pheochromocytoma)
Cardiovascular abnormality (coarctation of the aorta)

Secondary HTN

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Diagnosis?

HTN with hypokalemia
Idiopathic or aldosterone secreting tumor or glomerulosa cells directly responsive to ACTH

Primary Hyperaldosteronism

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Diagnosis?

HTN
Woman with round race, fat belly/neck/back, stretch marks
Pituitary tumor secreting ACTH or tumor secreting cortisol or paraneoplastic

Cushing syndrome

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Diagnosis?

HTN
Paroxysms of HTN, tachy, palpitations, headache, diaphoresis, tremors
Urinary or plasma metanephrines elevated

Pheochromocytoma

*catecholamine secreting tumor

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Some causes for renal artery stenosis that lead to HTN

Atherosclerosis
Fibromuscular dysplasia
Polycystic kidney disease, pylonephritis

*HTN decreases GFR leading to CKD

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Diagnosis?

HTN in the upper extremities with hypotension in the lower extremities

Coarctation of the aorta

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Affects of HTN on the heart and aorta (3)

Cardiac hypertrophy and chronic heart failure
Ischemic heart disease
Acute aortic dissection

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Affects of HTN on the kidney

Renal dysfunction and failure

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Affects of HTN on the brain and eye

Cerebrovascular hemorrhage, Stroke
Papilledema
Retinopathy

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Leading causes of death in untreated HTN

1/2 die from ischemic heart disease or CHF

- 1/3 die of stroke

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Histologic changes or arteries in chronic HTN

Luminal narrowing due to homogenous pink (hyaline) thickening of vessel wall

*kidney will show this histology and be shrunken with a granular surface

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BP reading in hypertensive crisis

Systolic >180 and/or Diastolic >120

*Hypertensive emergency occurs when there is end organ damage

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Histologic changes of vessels in severe HTN

Concentric lamellations “onion skinning”

*kidney will show this histology and have numerous petechial hemorrhages

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Diagnosis?

Myocardial infarct due to exogenous cocaine/caffeine, endogenous catecholamines, elevated thyroid hormones

Myocardial vessel vasospasm

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Diagnosis?

Emotional distress causes sudden surge of catecholamines which leads to ischemic cardiomyopathy and sometimes death
Almost exclusively in elderly women

Takotsubo cardiomyopathy (broken heart syndrome)

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Diagnosis?

Marked dilation of veins of the head, neck, chest wall and arms with cyanosis
Facial swelling and neurologic manifestations
Respiratory distress if pulm vessels compressed
Classically due to neoplasm (lung tumor, lymphoma)

Superior vena cava syndrome

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Diagnosis?

Marked lower extremity edema with distension of superficial lower abdominal veins
Classically due to neoplasm (HCC, renal cell carcinoma, DVT)

Inferior vena cava syndrome

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Diagnosis?

Venous thrombosis and inflammation, typically in the deep veins of the legs
Edema, cyanosis, erythema and pain
Due to prolonged inactivity/immobilization and hypercoagulable states

Thrombophlebitis

*most serous complication is PE

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Diagnosis?

Inflammation of lymphatic channels
Commonly caused by GAS

Lymphangitis

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Diagnosis? Swelling caused by fluid buildup due to lymphatic blockage Primary is congenital defect Secondary occurs in previously normal lymphatic drainage (peau d'orange in breast cancer)

Lymphedema

Vascular ectasias (blood vessel dilations): Birth mark, most will regress

Nevus simplex

Vascular ectasias (blood vessel dilations): Progressive ectasia of vascular plexus that persists into adulthood

Port-wine stain (nevus flammeus)

Vascular ectasias (blood vessel dilations): Trigeminal nerve region port wine stain of face Intellectual disability, neurological deficits Leptomeningeal capillary-venous malformation Skull radio-opacities Eye abnormalities (glaucoma)

Sturge-Weber syndrome

Diagnosis? Permanent dilation of small vessels Looks like spider

Spider telangectasia

Diagnosis? Numerous telangectasias and AV malformations of the skin, mucous membranes, organs Also presents with nosebleeds, GI bleeds and hematuria

Hereditary Hemorrhagic Telangectasia (Osler-Weber-Rendu disease) *caused by mutation in TGF-B signaling pathway

Hemangiomas: Capillary hemangioma: most common, small red spot Strawberry hemangioma: present at birth and regress Cavernous hemangioma: more likely to be in deep tissues and bleed Pyogenic granuloma: oral, lobar capillary hemangioma common in pregnancy

All benign vascular tumors

Diagnosis? Modified smooth muscle tumor Subungal location, blueish color Painful including with temp changes

Glomus tumor *arise from glomus body, responsible for thermoregulation

Diagnosis? Benign lymphatic neoplasm associated with Turner syndrome Dilated spaces that do not contain RBCs Present in the neck or axilla

Lymphangioma *Simple lymphangioma or cystic hygroma

Diagnosis? Benign red papule Caused by Bertonella bacilli (bacteria on silver stain) Occur in immunocompromised (HIV, transplant)

Bacillary angiomatosis

Diagnosis? Angioproliferative disorder caused by HHV-8 Red-purple nodules or plaques Patch stage => Plaque stage => Tumor stage

Kaposi sacroma Types: * Classic - distal lower extremities, indolent * Endemic african - in african kids * Iatrogenic - immunosuppressant related (transplant pt) * AIDS associated - most common AIDS related malignancy

Diagnosis? Malignant vascular tumor Risk factors: PVC (liver malignancy), lymphadema, radiation

Angiosarcoma

Nonmodifiable risk factors for atherosclerosis

- Genetic (multifactorial) - Family history - Age (40s-60s 5x risk, risk increases with age) - Male (estrogen protective)

Modifiable risk factors for atherosclerosis

- Hyperlipidemia (high LDL, low HDL) - HTN - Smoking - Diabetes - Obesity - Sedentary - Stress - Inflammation - Hyperhomocysteinemia

Diagnosis of metabolic syndrome criteria

3/5 - Abdominal obesity - Hypertriglyceridemia - Decreased HDL - HTN - Insulin resistance

Pathogenesis of atherosclerosis

Endothelial injury => SM migration => Macrophage infiltration and uptake of LDL => Macrophage cytokine release attracts more => SM proliferation and ECM deposition (PDGF, FGF, TGF-a) => Intimal thickening and plaque formation

Diagnosis and risk? Tall lanky person with long extremities Mutation in FBN1, increased TGF-B activity, weakens elastic tissue

Marfan *risk of aortic aneurysm and dissection

Diagnosis and risk? Hyperelastic skin Joint hypermobility Fragile skin and poor wound healing Mutation in collagen 3 gene (COL5A1, COL5A2)

Ehlers Danlos syndrome *risk of large artery rupture, MVP

Most important risk factor for AAA

Atherosclerosis

Aside from atherosclerosis, smoking, age and being male... what else can cause AAA?

IgG4 related disease * inflammatory type with lymphoplasmatic inflammation\ * responds well to steroids

Most important risk factor for thoracic aortic aneurysm

HTN *also may be caused by tertiary syphilis (obliteration of vasa vasorum)

Diagnosis? Thoracic pain, pulse abnormalities, mediastinal widening on CXR

Aortic dissection *casued by HTN and CT disorders (Marfan, Ehlers Danlos)

Most common location for aortic dissection

proximal to right brachiocephalic artery *classified and Stanford Type A or DeBakey 1

Primary Reynaud's phenomenon

Symmetric attack with spontaneous remission

Secondary Reynaud's phenomenon

Asymmetric attack, increasing severity over time, due to another disease (SLE, scleroderma, Bergers vasculitis)

Vasculidities that involve the aorta and often have giant cells

Giant cell arteritis (temporal arteritis | Takayasu arteritis

Vasculitis where granulomas are required for diagnosis

Granulomatosis with polyangitis *also has PR-3 ANCA (c-ANCA)

Vasculitis where eosinophilia is required for diagnosis

Churg-Strauss Syndrome *also has MPO ANCA (p-ANCA)

Vasculitis where neutrophils are required for diagnosis

Behcet's disease

How is restenosis prevented in baloon angioplasty with endovascular stenting?

Drug eluting stents *coated in drug that prevents SM activation and proliferation

Vasculitis diagnosis? Older adults T-cell mediated Headache, occular symptoms (potential vision loss)

Giant cell (temporal) arteritis

Vasculitis diagnosis? ``` Female <50 Autoimmune Granulomatous inflammation Weak upper extremity pulses Occular disturbances ```

Takayasu arteritis

Vasculitis diagnosis? ``` Associated with HBV Fibrinoid necrosis Affects renal vessels, GI tract, skin Spares the lungs Rapidly accelerating HTN ```

Polyarteritis nodosa

Vasculitis diagnosis? ``` Child <5 years old Conjunctivitis Rash Adenopathy (cervical lymphnodes) Strawberry toungue Hand and foot erythema/edema Fever ```

Kawasaki disease (mucocutaneous LN syndrome)

Vasculitis diagnosis? ``` Asthma Eosinophilia Palpable purpura GI bleeding Renal disease ```

Churg-Strauss (eosinophilic/allergic granulomatosis with polyangiitis) *may give rise to cardiomyopathy/myocarditis =>death

Vasculitis diagnosis? Necrotizing granulomatous vasculitis Affects lungs, nose, kidneys PR3-ANCA (c-ANCA)

Granulomatosis with polyangiitis (Wegener's)

Vasculitis diagnosis? Necrotizing vasculitis that involves capillaries, venules Affects kidney, lung, nereves, GI tract (no nose) MPO-ANCA (p-ANCA) No immune complex deposition

Microscopic polyangiitis