USMLE Cardiac Pathology

What is characteristic of Churg-Strauss vasculitis?

It is a varient of Polyarteritis Nodosa with associated bronchial asthma.

Granulomas and Eosinophils** are associated with it

What ANCA is associated with Churg-Strauss?

p-ANCA

What is Raynauds Disease?

PRIMARY small artery vasospasm leading to blanching and cyanosis... Made worse by cold.

What is Raynauds Phenomenon?

Raynauds Disease symptoms, brought about secondary to another disease such as SLE, CREST, Buergers etc..

What is seen microscopically in Kaposi-Sarcoma?

Slit like spaces in the vasculature leading to extravisation of blood which manifests as purpura.

How is AIDS associated Kaposi Sarcoma different from non-AIDS related KS from other immunocompromising situations?

AIDS is more aggressive and can metastasize to various regions of the body

What is an angiosarcoma?

A sarcoma of blood vessels, usually seen in the liver as a hemangiosacroma.. Caused by polyvinyl chloride, thorotrast and arsenic

What is the most common type of vasculitis or medium and large arteries?

Temporal Arteritis, AKA giant cell arteritis. Usually affects branches of the carotid and the temporal artery.

Who does Temporal Arteritis usually effect and what is seen microscopically?

Elderly females...

Focal granulomatous inflammation, esp around fragmentation of the internal elastic lamina

Symptoms of Temporal Arteritis?

Unilateral headache and visual problems..

Jaw Claudication

Elevated ESR is usually seen.

What condition is usually associated with Temporal Arteritis?

Polymyalgia Rheumatica!

Flu like symptoms and PAIN in the pelvic and shoulder girdles

Who is usually at risk for Takayasu's arteritis and what blood vessels are affected?

Young Asian women....

Medium and large arteries, esp the aortic arch and its branches. (can look like Temportal arteritis)

What is Takayasu's arteritis AKA?

Pulseless disease... Weak pulses are often seen in upper extremities, as well as granulomas

Common S/S of Takayasu.s arteritis?

FAN MY SKIN On Wednesday

Fever

Arthritis

Nightsweats

MYalgia

SKIN nodules

Ocular disturbances

Weak pulses in upper limbs

What vessels are commonly affected by Polyarteritis nodosa??

Medium arteries, esp Renal and Visceral vessels BUT NO LUNG INVOLVEMENT!

What is seen microscopically in polyarteritis nodosa?

Segmental necrotic vasculitis dues to immune complex inflammation. Lesions are of different ages.***

What are typical findings of a patient with Polyarteritis nodosa?

HEP B POSITIVITY!!*

Multiple aneurisms and constrictions on arteriogram

NO anca assocation.

Symptoms of PAN?

Fever, wt loss and Headache

Cutaneous eruptions and neurological dysfunction

Myalgia

What vessels are typically affected in Kawasaki disease and who is at risk?

Small and medium vessels, often causing CORONARY ANEURISMS!

Infants and kids, esp of Asian decent. Normally self limiting.

Clinical findings of Kawasaki disease?

Fever, changes to mucosa and lips including

strawberry tongue' and LYMPHADENITIS.

What is Buerger's disease AKA?

Thromboangiitis Obliterans...

What is the only vascular disease that affects Arteries as well as nerves and veins?

Buerger's disease.

Who is at risk for Buerger's disease?

Smokers!!! Stop smoking to treat.

Signs and Symptoms of Buerger's disease?

Intermittent claudication

Cold sensitivity (Raynauds)

Nodular phlebitis.... Can lead to autoamputation

What is the common triad of areas affected by Henoch Schonlein Purpura?

Skin

Joints

GI.... Usually affects small vessels

What is the most common form of childhood systemic vasculitis?

Henoch Schonlein Purpura

What is characteristic of the lesions of Henoch Schonlein Purpura?

Multiple skin lesions of the same age with PALPABLE purpura**

Usually seen on buttocks and legs

What is Henoch Schonlein Purpura often associated with?

IgA immune complexes, seen following upper respiratory infections.

IgA nephropathy

Findings of Henoch Schonlein Purpura?

Palpable Purpura

Arthralgias

Intestinal hemorrhage

Abd pain and melena.

What are the symtoms of Microscopic polyangiitis?

Like Wegener's, but lacks the granulomas.

P-ANCA associated instead of C-ANCA

What is the Triad of Wegeners' Granulomatosis?

Necrotizing vasculitis

Necrotizing granulomas of the lung AND upper airway!

Necrotizing glomerulonephritis.... Will see neutrophilic infiltration.

Findins of Wegeners?

C-ANCA positive

Chest x-ray can show large nodular densities

Hematuria with red cells

Hemoptysis

CAUSE of Raynaud's phenomenon?

Arteriolar vasospasm due to the cold

What is Characteristic of Sturge-Weber disase?

Port Wine stain on the face

Leptomeningeal angiomatosis (intracerebral AVM)

Vascular disorder affecting capillaries

Who is affected by Hemangiomas and what is the usual outcome?

Children...

Two types are Capillary and Cavernous.

Most spontaneously regress

Where are capillary hemangiomas usually found?

In children, superficially.

Called Strawberry hemangiomas.

Where are Cavernous hemangiomas usually found?

In children, commonly in the liver.

Caused by massive dilation of vessels

What conditions are hemangioblastomas typically associated with?

RCC

and

von Hippel Lindau

Where in the body are hemangioblastomas typically seen on the USMLE?

Cerebellum and Retina

What is a Glomus tumor or Glomangioma?

Tumor of blood vessels seen under the nailbeds!

PAINFUL**

How are DVT's diagnosed?

With doppler studies

What predisposes one to DVTs?

Virchow's Triad!

Blood stasis

Endothelial damage

Hypercoagulable state! (seen often in cancer pts)

Why are TED hose used for DVTs?

Compress the superficial veins to increase the bloodflow to the deep vein.

What are varicose veins?

Superficial tortuous and dilated veins with incompetent valves.

Complications of Varicose Veins?

Stasis dermatitis, itchy...

NOT EMBOLIZATION.

What BP ranges characterize Prehypertension?

120-139

or

80-89 diastolic

What BP ranges characterize Stage 1 Hypertension?

140-159

or

90-99 diastolic

What BP ranges characterize Stage 2 HTN?

160 +

or

100 + diastolic

What BP ranges characterize malignant HTN?

Greater than 180 systolic

or

Greater than 120 diastolic...

What are clinical findings of malignant HTN?

Flea bitten appearance to the kidney

Papilledema

Onion-skinning arteriolosclerosis due to hyperplasia of endothelial cells

What are two physiological factors that are increased in essential HTN?

Increased cardiac output or increased total peripheral resistance

What type of arteriolosclerosis does HTN predispose to?

Hyaline type

What is the Monckenberg classification of Arteriosclerosis?

Calcification in the MEDIA or arteries, esp radial or ulnar....

Gives pipestem appearance.

DOES NOT OBSTRUCT BLOOD FLOW AND INTIMA IS NOT INVOLVED

What is arteriolosclerosis?

Hyaline thickening of the small arteries in essential HTN... Leads to onion skin appearance in malignant HTN

What is the result of Atherosclerosis?

Fibrous plaques and atheromas forming in the INTIMA of arteries, esp after damage to the vessel.

What is being referred to when there is a COMPLICATED PLAQUE in BV pathology?

A problem with the plaque itself, such as calcification, thromobosis or rupture.

COMPLICATIONS of a plaque are MI, Stroke, gangrene etc..

Risk factors of atherosclerosis?

Smoking

HTN

Diabetes

Hyperlipidemia

What are the steps in the progression of atherosclerosis?

Endothelial cell damage

OXIDIZED LDL accumulation and macrophage phagocytosis....

Foam cells form and rupture, leading to fatty streaks

Smooth muscle cell migration involving PDGF and FGF-Beta leads to fibrous plaques and atheromas

Are fatty streaks reversible?

Yes, seen as early as in children...

If there is fibrous CAP on the atheroma, it is permanent.

What are the top 4 locations of Atherosclerotic plaques?

Abdominal Aorta>

Coronary Artery>

Popliteal Artery>

Carotid Artery

What is Stable angina?

Chest pain, usually secondary to atherosclerosis, caused during exertion and relieved by rest or nitroglycerine.

What is classically seen on an EKG of Stable angina?

ST depression

What is Prinzmetal's Angina?

Occurs at rest secondary to CORONARY ARTERY SPASM.

What is classically seen on an EKG of Prinzmetal's Angina?

ST elevation

What is Unstable or Crescendo angina?

Pain caused at rest, that progressively worsens... Thromosis commonly occurs

What is characteristic of Unstable angina on EKG?

ST depression... precursor to MI

Who are most at risk for Ischemic heart disease and Angina?

Elderly males and postmenopausal women.

What type of necrosis is seen on MI and what is the typical histological findings of this necrosis?

Coagulative!

Presevation of the borders of the cell, but no nuclei

What is sudden cardiac death?

Death from cardiac causes within 1 hour of onset of symptoms USUALLY CAUSED BY A LETHAL ARRHYTHMIA!

Silent MIs may result of the MI is restricted to...

The subendocardium

What is Chronic ischemic heart disease?

Progressive onset of CHF over many years due to chronic ischemic myocardial damage that never manifests as a full blown MI.

What is the gold standard for diagnosis of an MI in the first 6 hours of onset?

EKG

What is the most specific protein marker for an MI and when does can it be detected?

Tropinin, esp troponin I!

Rises after 4 hours and peaks shortly thereafter

Present for 7-10 days

What CK enzyme is used to detect MI and when is it detectable?

CK-MB--- Peaks after a day and is only around for 3 days.

If Troponin I is elevated and not CK MB then it must be 4-7 days post MI

WRT MI's, ST elevation is seen in...

Transmural infarcts.. leads to greater degree of necrosis

WRT MIs, ST depressionis seen in...

Subendocardial infarcts...ischemic necrosis of less than 50% of the ventricular wall

When are 'pathological Q waves' seen on EKG?

After a transmural infarct MI

What is the normal ration of LDH enzymes and how can these be used to monitor MIs?

Normally enzyme 2 is greater an enzyme 1..

After an MI the LDH FLIP can occur, leading to enzyme 1>2. Can be present for up to 2 weeks, so if CKMB and Troponin I are absent but this is present, the MI is greater than a week old.

Most common COD in an MI patient before reaching the hospital?

Fatal arrhythmia...common finding in the first few days as well.

When is fibrinous pericarditis seen in an MI patient?

Between 3-5 days post MI--- leads to friction rub and pleuritic chest pain.

What is Dressler's syndrome and when is it observed?

An autoimmune phenomenon RESULTING from fibrinous pericarditis that occurs several weeks post MI.

What is the cause of cardiogenic shock in an MI patient?

Decreased tissue perfusion, commonly seen in large infarcts. High risk of mortality

When is a ventricular free wall rupture post MI most likely and what can it cause?

4-7 days post MI... Cardiac tamponade if blood enters the paricardial sac.

Interventricular septal rupture can lead to a VSD

Papillary muscle rupture can lead to mitral regurgitation.

What are two causes of Degenerative Calcific aortic valve stenosis?

Age related DYSTROPHIC (normal Ca++ levels with damaged tissue) calcification of the aortic valve (70's plus)

OR

Congenital bicuspid aortic valves...appears in younger patients.

What type of hypertrophy results from calcific aortic stenosis and what are common signs or symptoms?

CONCENTRIC LVH

CHF, sycopal episodes, chest pain and palpitations

Who is generally affected by MVP?

Young women or people with Marfan's Syndrome

What is heard on auscultation of someone with MVP?

Mid-systolic click

What is seen on gross appearance of a person with MVP?

Flopply leaflets leading to prolapse

'Ballooning' of 'Parachuting' of the leaflets into the left atrium

What is seen microscopically in MVP?

Myxomatous degeneration

What are some complications of MVP?

Infective endocarditis...subacute, as it is caused by a previously damaged valve

Mitral insufficiency, leading to LVH.

Major criteria for Rheumatic Fever diagnosis?

Must have two major or 1 major and 2 minor.

Majors are PANCARDITIS, Subcutaneous nodules and Sydenham Chorea, as well as migratory polyarthritis and erythma marginatum.

What are Aschoff bodies?

Fibrinoid necrosis, surrounded by macrophages that form in the myocardium...Lesion in the myocardium is most deadly tho can occur in any layer.

What is an Anitschokow cell?

AKA a caterpillar cell....it is the macrophages of the Aschoff body in myocarditis caused by rheumatic fever

How do fibrinous plaques appear regarding the endocarditis seen in Rheumatic Fever?

Involve the mitral and aortic valves, seen along the lines of closure. Small Fibrin vegetations

What are MacCallum plaques?

Left atrial endocardial thickenings...seen in Rheumatic Heart disease.

When is Chronic Rheumatic heart disease seen and what are some manifestation?

In adults 15-20 years after acute RF. Mitral valve thickening and calcification, leading to stenosis and LAH.

Fusion of the valve commisures and a 'fishmouth appearance' or the valves

Short thickened chordae tendinae

Early deaths in Rheumatic heart disease are caused by...

Myocarditis

Rheumatic Fever Mnemonic?

FEVERSS

Fever

Erythema marginatum

Valcular damage

ESR increased

Red hot joints (Migratory polyarthritis)

Subcutaneous nodules (Aschoff bodies)

Sydenham Chorea

Acute endocarditis is caused by..

S. aureus! NORMAL VALVES, it is simple so virulent.

What type of vegetations are seen in Acute endocarditis?

Large beefy red destructive vegetations