Cardiovascular Pathology

• Systolic:
• -Mitral/Tricuspid Regurgitation (MR/TR)
• -Aortic Stenosis (AS)
• -VSD
• -Mitral Valve Prolapse (MVP)

• Diastolic:
• -Aortic Regurgitation (AR)
• -Mitral Stenosis (MS)

• Continuous:
• -PDA

• Reflux of blood from LV into LA during systole
• 
• Characteristics:
• -Holosystolic
• -High-pitched
• -"Blowing"

• Location:
• -loudest at apex

• Radiation:
• -toward axilla

• Maneuvers that Enhance:
• -↑ TPR (squatting, hand grip)
• -↑ LA return (expiration)

• Etiology:
• -often due to ischemic heart disease
• -mitral valve prolpase
• -LV dilation (LHF)
• -acute rheumatic heart disease (vs. chronic → stenosis)
• -infective endocarditis
• -papillary muscle rupture (after MI)

• Complications:
• -volume overload and L-sided HF

• Reflux of blood from RV to RA during systole
• 
• Characteristics:
• -Holosystolic
• -High-pitched
• -"Blowing"

• Location:
• -loudest at tricuspid area

• Radiation:
• -right sternal border

• Maneuvers that Enhance:
• -↑ RA return (inspiration)

• Etiology:
• -RV dilation
• -rheumatic fever (MR or TR)
• -infective endocarditis (MR or TR)

• Narrowing of aortic valve orifice: fibrosis and calcification (<1cm²)
• 

• Characteristics:
• -Systolic ejection murmur
• -Ejection click (due to abrupt halting of valve leaflets)
• -Crescendo-Decrescendo

• Radiation:
• -carotids/heart base

• Etiology:
• -"wear and tear" (presents in late adulthood)
• -increased risk with bicuspid aortic valve
• -chronic rheumatic heart disease (coexisting MS and fusion of aortic valve commissures)

• Complications:
• -prolonged asymptomatic stage
• -Syncope
• -Angina
• -Dyspnea on exertion
• "SAD"

• -concentric LVH
• -microangiopathic hemolytic anemia (schistocytes)

• Treatment:
• -valve replacement when complications develop

• 
• Characteristics:
• -Holosystolic
• -"Harsh"

• Location:
• -loudest at tricuspid area

• Maneuvers that Enhance:
• -↑ Afterload (hand grip)

• Ballooning of mitral valve into LA during systole
• -Most frequent valvular lesion: seen in 2-3% of US adults
• 

• Characteristics:
• -Late systolic
• -Midsystolic click (due to sudden tensing of chordae tendinae)
• -Crescendo murmur

• Location:
• -loudest over apex
• -loudest at S2

• Maneuvers that Enhance:
• -↓ Venous return (Standing, Valsalva)

• Etiology:
• -unknown
• -myxomatous degeneration
• -rheumatic fever
• -chordae rupture
• -Marfan Syndrome
• -Ehlers-Danlos syndrome

• Complications:
• -rare, usually asymptomatic
• -predispose to infective endocarditis
• -arrhythmia
• -severe mitral regurgitation

Backflow of blood from aorta into the LV during diastole

• 
• Characteristics:
• -diastolic murmur
• -decrescendo
• -high-pitched
• -"blowing"

• Maneuvers that Enhance:
• -↑ Afterload (Hand grip)

• Manuevers that ↓ Intensity:
• -Vasodilators

• Clinical Features:
• -wide pulse pressure
• -bounding pulses
• -head bobbing
• -pulsating nail beds

• Etiology:
• -aortic root dilation (syphilitic aneurysm and aortic dissection)
• -bicuspid aortic valve
• -endocarditis (valve damage)
• -rheumatic fever

• Complications:
• -LV dilation and eccentric hypertrophy

Narrowing of the mitral valve orifice

• 
• Characteristics:
• -delayed, late diastolic
• -follows OS (abrupt halt of leaflet motion in diastole after rapid opening due to fusion at leaflet tips)
• -"rumbling"

• Maneuvers that Enhance:
• -↑ LA return (expiration)

• Etiology:
• -chronic rheumatic valve disease

• Complications:
• -chronic MS can result in LA dilation
• -Pulmonary congestion (edema and alveolar hemorrhage)
• -Pulmonary HTN (w/ eventual RHF)-Atrial fibrillation (increased risk for mural thrombi)

• Characteristics:
• -continuous
• -"machine-like"
• -loudest at S2

• Location:
• -best heard at left infraclavicular area

• Etiology:
• -congenital rubella
• -prematurity

Released from atrial myocytes in response to ↑ blood volume and atrial pressure

• Function:
• -causes generalized vascular relaxation and ↓ Na+ reabsorption at medullary collecting tubule
• -constricts efferent renal arterioles
• -dilates afferent renal arterioles (cGMP mediated)
• -promotes diuresis and contributes to "escape from aldosterone" mechanism

BP ≥ 140/90 mmHg

• Risk Factors:
• -age
• -obesity
• -diabetes
• -smoking
• -genetics
• -black > white > Asian

• Primary HTN:
• -unknown etiology
• -90% of HTN
• -related to increased CO or TPR
• -risk factors listed above

• Secondary HTN:
• -due to an identifiable etiology
• -5-10% of HTN
• -most common cause is renal artery stenosis (JGA activation of RAAS → ATII vasoconstriction and Aldo Na/H2O retention)
• -elevated plasma renin
• -unilateral atrophy (due to low blood flow)
• -due to atherosclerosis (elderly men) and fibromuscular dysplasia (young females)

• Malignant HTN:
• -severe (>180/120) and rapidly progressing
• -most cases of HTN are benign, 5% are malignant
• -presents with acute end-organ damage (AKI, HA, Papilledema)
• -medical emergency

• Predisposes To:
• -athersclerosis
• -LVH
• -stroke
• -CHF-renal failure
• -retinopathy
• -aortic dissection

• Atheromas
• -plaques in blood vessel walls

• Xanthomas
• -plaques or nodules composed of lipid-laden histiocytes in the skin
• -especially the eyelids (Xanthelasma)

• Tendinous Xanthoma
• -lipid deposit in tendon
• -esp Achilles

• Corneal Arcus
• -lipid deposit in cornea, nonspecific (arcus senilis)

• "Hard arteries"
• -due to thickening of blood vessel wall

• 3 Types
• -Monckeberg
• -Arteriolosclerosis
• -Atherosclerosis

• Calcification in the media of the arteries
• -especially radial and ulnar
• -usually benign
• -Does not obstruct blood flow
• -Intima not involved

"pipe stem" arteries

• Hyaline:
• -thickening of small arteries in essential HTN or DM
• -caused by proteins leaking into the vessel wall → vascular thickening
• -Complications: classically produces glomerular scarring that progresses to renal failure

• Hyperplastic:
• -onion skinning
• -consequence of malignant HTN
• -may lead to fibrinoid necrosis
• -classically causes acute renal failure (flea-bitten appearance)

Fibrous plaques and atheromas form in intima of arteries

Disease of elastic arteries and large and medium sized muscular arteries

• Modifiable Risk Factors:
• -smoking
• -HTN
• -Hyperlipidemia
• -DM

• Non-Modifiable Risk Factors:
• -age
• -gender (↑ in men and postmenopausal women)
• -positive FHx

• Plaque:
• -necrotic lipid core with fibromuscular cap

• Progression:
• -Inflammation is important in pathogenesis
• 1. Endothelial cell dysfunction with LDL accumulation in intima
• 2. LDL is oxidized and consumed by MPs
• 3. Foam cell formation
• 4. Fatty streaks (present in most teenagers)
• 5. SM cell migration (involved PDGF and FGF), proliferation and ECM deposition
• 6. Fibrous plaque
• 7. Complex atheroma

• 
• Complications:
• *account for > 50% of disease in the western world
• -Aneurysms
• -Ischemia
• -Infarcts
• -Peripheral vascular disease
• -Thrombus
• -Emboli

• Location:
• -Abdominal aorta > coronary artery > popliteal artery > carotid artery

• Symptoms:
• -angina
• -claudication
• -MAY BE ASYMPTOMATIC

• Epidemiology:
• -occurs more frequently in hypertensive male smokers > 50 years old

• Pathophysiology:
• -usually arises below the renal arteries but above the aortic bifurcation
• -primarily due to atherosclerosis (weakens vessel wall)
• 
• 
  Presentation:
• -pulsatile abdominal mass
• -grows over time

• Complications:
• -rupture!!! (esp when > 5cm in diameter)
• -compression of local structures
• -thrombosis/embolism

• Etiology:
• -tertiary syphilis (endarteritis of vasa vasorum results in luminal narrowing, decreased flow and atrophy of vessel wall; Tree bark appearance)
• 
• -cystic medial necrosis = weakness of CT in the media (Marfan's Syndrome)
• -HTN

• Complications:
• -dilation of aortic valve root → aortic insufficiency
• -compression
• -thrombosis/embolism

• Pathophysiology:
• -longitudinal tear in the intima
• -blood flow through media of aortic wall
• -false lumen can be limited to the ascending aorta, propagate from the ascending aorta or propagate from the descending aorta
• -occurs in the proximal 10 cm of the aorta (high stress region) with preexisting weakness of the media

• Etiology:
• -most commonly HTN (hyaline arteriolosclerosis of vasa vasorum →weakens media)
• -Marfan Syndrome (defect in fibrillin, foundation for elastin fibers)
• -Ehlers-Danlos Syndrome (defective formation of collagen)
• -cystic medial necrosis
• -bicuspid aortic valve

• Presentation:
• -tearing chest pain radiating to the back
• -CXR: mediastinal widening

• Complications:
• -pericardial tamponade (most common cause of death)
• -rupture with fatal hemorrhage
• -obstruction of branching arteries (ie: coronary or renal)

• Angina
• -CAD narrowing, no myocyte necrosis
• -Types: stable, unstable, Prinzmetal variant

Coronary Steal Syndrome

Myocardial Infarction

Sudden Cardiac Death

Chronic Ischemic Heart Disease

Vasodilator my aggravate ischemia by shunting blood from area of critical stenosis to an area of higher perfusion

• -unexpected death due to cardiac disease
• -occurs without sx or within 1 hour of onset of sx

• Etiology:
• -most commonly due to fatal ventricular arrhythmia
• -up to 70% of cases associated with CAD
• -mitral valve prolapse
• -cardiomyopathy
• -cocaine abuse

Progressive onset of CHF over many years due to chronic ischemic myocardial damage

• Presentation:
• -chest pain that arises with exertion or emotional stress
• -lasts < 20 minutes (no necrosis)
• -radiate to L arm or jaw
• -diaphoresis
• -SOB

• Pathophysiology
• -mostly due to atherosclerosis
• ->70% stenosis required for sx
• -decreased blood flow not able to meet  demands of myocardium during exertion
• -reversible injury to myocytes

• EKG:
• -ST depression (subendocardial ischemia: most susceptible)
• Relieved by:
• -Rest
• -Nitroglycerin (vasodilation decreases VR, decreases preload)

• Presentation:
• -chest pain that occurs at rest

• Pathophysiology:
• -usually due to rupture of an atherosclerotic plaque with thrombosis and incomplete occlusion of a coronary artery
• -reversible injury to myocytes

• EKG:
• -ST depression (subendocardial ischemia)

• Relieved by:
• -Nitroglycerin

**very high risk of progression to MI

• Presentation:
• -episodic chest pain unrelated to exertion

• Pathophysiology:
• -due to coronary artery vasospasm
• -reversible injury to myocytes (no necrosis)

• EKG:
• -ST elevation (transmural ischemia: cut off blood supply to entire wall)

• Relieved by:
• -Nitroglycerin
• -CCB (relieves spasms)

Necrosis of cardiac myocytes

• Pathophysiology:
• -usually due to rupture of an atherosclerotic plaque with thrombosis and complete occlusion of a coronary artery
• -other causes: vasospasm (Prinzmetal angina/cocaine), emboli, vasculitis (Kawasaki)

• Presentation:
• -severe, crushing chest pain (>20 min)
• -radiates to L arm or jaw
• -diaphoresis
• -fatigue
• -dyspnea (pulmonary edema)
• -nausea/vomiting
• -NOT relieved by nitroglycerin

• EKG:
• -initially shows ST depression (subendocardial necrosis)
• -continued or severe ischemia leads to ST elevation (transmural necrosis)

• Labs:
• -Troponin I
• -CK-MB

• Affected Vessels:
• 1. LAD
• -most common (45%)
• -infarction of anterior wall and anterior septum of LV

• 2. RCA
• -second most commonly affected vessel
• -infarction of posterior wall, posterior septum and papillary muscle of LV

• 3. LCx
• -infraction of lateral wall of LV

• Gross Changes:
• -none

• Microscopic Changes:
• -none

• Complications:
• -Arrhythmia
• -CHF exacerbation (↓ EF)
• -Cardiogenic shock (no perfusion of vital organs)

• Gross Changes:
• -dark mottling
• -pale with tetrazolium stain
• 

• Microscopic Changes:
• -early coagulative necrosis
• -edema
• -hemorrhage
• -wavy fibers
• 

• Complications:
• -Arrhythmia

• Gross Changes:
• -dark mottling
• -pale with tetrazolium stain
• *same as 4-12 hours
• 

• Microscopic Changes:
• -contraction bands (reperfusion injury: increased Ca2+ leads to hypercontraction of myofibrlis)
• -release of necrotic cell content into blood
• 

• Complications:
• -Arrhythmia

• Gross Changes:
• -hyperemia
• 

• Microscopic Changes:
• -extensive coagulation necrosis
• -acute inflammation around infarct
• -NP migration
• 

• Complications:
• -Fibrinous pericarditis (if transmural infarction)

• Gross Changes:
• -hyperemic border
• -central yellow-brown softening (max by 10 days)
• 

• Microscopic Changes:
• -macrophage infiltration
• -granulation tissue at margins
• 

• Complications:
• -free wall rupture → cardiac tamponade
• -papillary muscle rupture
• -ventricular aneurysm
• -interventricular septum rupture
• *due to degradation by MPs

• Gross Changes:
• -gray-white scar
• 

• Microscopic Changes:
• -contracted scar complete (fibrosis)
• 

• Complications:
• -Aneurysm
• -mural thrombus
• -Dressler Syndrome (pericardial inflammation leads to antibody formation against pericardium → autoimmune pericarditis)

• EKG
• -gold standard for first 6 hours
• -ST depression (subendocardial infarct)
• -ST elevation (transmural infarct)
• -Pathologic Q waves (transmural infarct)

• Troponin I
• -most sensitive and specific blood test
• -rises after 4 hours
• -elevated for 7-10 days

• CK-MB
• -predominantly found in myocardium but can be released from skeletal muscle
• -useful in diagnosing reinfarction following acute MI
• -rises at 4-6 hours
• -returns to normal after 48 hours

• Transmural Infarcts:
• -↑ necrosis
• -affects entire wall
• -ST elevation and pathologic Q waves on EKG

• Subendocardial Infarcts:
• -ischemic necrosis of < 50% of ventricular wall
• -subendocardium is especially vulnerable to ischemia
• -ST depression on EKG

• Anterior Wall Infarct
• -LAD
• -Q waves in V1-V4

• Anteroseptal Infarct
• -LAD
• -Q waves in V1-V2

• Anterolateral Infarct
• -LCx
• -Q waves in V4-V6

• Lateral Wall Infarct
• -LCx
• -Q waves in I, aVL

• Inferior Wall Infarct (posterior LV)
• -RCA
• -Q waves in II, III, aVF

• Cardiac Arrhythmia
• -important cause of death before reaching the hospital
• -common in first few days

LV failure and pulmonary edema

• Cardiogenic shock
• -large infarct has a high risk of mortality
• -failure of CO: no organ perfusion

• Ventricular Free Wall Rupture
• -cardiac tamponade

• Papillary Muscle Rupture
• -severe mitral regurg

• Interventricular Septum Rupture
• -VSD

• Ventricular Aneurysm
• -decreased CO
• -risk of arrhythmia
• -embolus from mural thrombus
• -greatest risk approximately 1 week post MI

• Postinfarction Fibrinous Pericarditis
• -friction rub
• -1-3 days post MI

• Dressler's Syndrome
• -autoimmune phenomenon resulting in fibrinous pericarditis
• -several weeks post- MI

• 1. Dilated (congestive)
• 2. Hypertrophic
• 3. Restrictive (obliterative)

• Epidemiology:
• -most common cardiomyopathy (90%)

• Etiologies:
• -often idiopathic
• -up to 50% familial
• -chronic Alcohol abuse
• -wet Beriberi
• -Coxsackie B virus myocarditis
• -chronic Cocaine use
• -Chaga's disease
• -Doxorubicin toxicity
• -hemochromatosis
• -peripartum cardiomyopathy (pregnancy)

"ABCCCD"

• Pathophysiology:
• -all four chambers of heart are dilated
• -systolic dysfunction
• -eccentric hypertrophy (sarcomeres added in series)
• -biventricular CHF

• Findings:
• -S3
• -dilated heart on U/S
• -balloon appearance on X-ray

• Treatment:
• -Na+ restriction
• -ACEI
• -diuretics
• -digoxin
• -heart transplant

• Etiology:
• -60-70% of cases are familial, AD (commonly mutation in β-myosin heavy chain)
• -associated with Friedreich's ataxia

• Pathophysiology:
• -massive hypertrophy of left ventricle
• -asymmetric concentric hypertrophy (sarcomeres added in parallel)
• -diastolic dysfunction (can't fill hypertrophied LV → ↓CO)
• -hypertrophied interventricular septum obstructions outflow through mitral valve (causes murmur and syncope)
• 

• Histology:
• -disorganized, tangled, hypertrophied myocardial fibers

• Presentation:
• -Decreased CO
• -Sudden death (major cause in young athletes)
• -syncope (mitral valve obstruction)

• Findings:
• -normal sized heart
• -S4
• -apical impulses
• -systolic murmur (mitral obstruction)

• Treatment:
• -β-blocker
• -non-DHP CCB (Verapamil)

• Etiology:
• -sarcoidosis
• -amyloidosis
• -postradiation fibrosis
• -endocardial fibroelastosis (children)
• -Loffler's Syndrome
• -hemochromatosis

• Endocardial Fibroelastosis:
• -thick fibroelastic tissue in endocardium
• -young children

• Loffler's Syndrome:
• -endomyocardial fibrosis with a prominent eosinophilic infiltrate

• Pathophysiology:
• -diastolic dysfunction

• Presentation:
• -CHF
• -low-voltage EKG with diminished QRS amplitude

• Causes:
• -ischemia
• -HTN
• -MI
• -dilated cardiomyopathy (causes greater ventricular end-diastolic volume)
• -restrictive cardiomyopathy

• Pathophysiology:
• -pulmonary congestion (pulmonary edema)
• -decreased forward perfusion (kidneys)
• -decreased kidney perfusion leads to activation of RAAS (vasoconstriction and Na+/H2O retention)
• -exacerbates CHF

• Pulmonary Edema:
• -↑ pulmonary venous pressure → pulmonary venous distention and transundation of fluid
• -hemorrhage leads to hemosiderin-laden MPs (Heart Failure cells)

• Clinical Presentation:
• -dyspnea
• -paroxysmal nocturnal dyspnea (increased VR when laying flat)
• -orthopnea (increased VR when laying flat, exacerbates pulmonary vascular congestion)
• -crackles
• -hemosiderin laden MP in lungs (HF cells)

• Treatment:
• -ACEI (mainstay)
• -β-Blockers (except in acute decompensated HF)
• -ARBs and Spironolactone: reduce mortality
• -Thiazide/Loop diuretics: mostly for sx relief
• -Hydralazine with nitrate tx: improves sx and mortality in select patients

• Decreased LV contractility leads to pulmonary venous congestion
• -this causes pulmonary edema and decreased RV output, leading to peripheral edema

• Decreased LV contractility also decreases CO
• -activates RAAS: increased Na+/H2O reabsorption
• -increases sympathetic activity: increases RAAS
• -increased Na+/H2O retention increases systemic venous pressure

• Increase systemic venous pressure leads to:
• -peripheral edema
• -increased preload, increased CO (compensation!)

• Causes:
• -usually due to LHF
• -isolated RHF usually due to cor pulmonale (chronic lung disease)
• -L→R shunt

• Pathophysiology:
• -systemic congestion

• Clinical Presentation:
• -elevated JVP
• -painful hepatosplenomegaly ("nutmeg liver", may lead to cardiac cirrhosis)
• -dependent pitting edema

• Treatment:
• -ACEI (mainstay)
• -β-Blockers (except in acute decompensated HF)
• -ARBs and Spironolactone: reduce mortality
• -Thiazide/Loop diuretics: mostly for sx relief
• -Hydralazine with nitrate tx: improves sx and mortality in select patients

• Epidemiology:
• -most commonly caused by S. viridans
• -mitral valve most frequently involved
• -IV drugs associated with Tricuspid valve endocarditis (S. aureus, Pseudomonas and Candida)
• "IV drugs abuse (don't tri drugs)"

• Acute:
• -S. aureus
• -high virulence
• -large vegetations on previously normal valves
• -rapid onset

• Subacute:
• -viridans strep
• -low virulence
• -smaller vegetations
• -congenitally abnormal or diseased valves
• *sequelae of dental procedures
• -more insidious onset

• Other Causes:
• -S. epidermidis (prosthetic valves)
• -S. bovis (associated with colon cancer)
• -HACEK (Haemophilus, Actinobacillus, Cardiobacerium, Eikenella, Kingella) (negative blood cultures)

• Pathophysiology:
• -damaged endocardial surface develops thrombotic vegetations (platelets and fibrin)
• -transient bacteremia leads to trapping of bacteria in the vegetations

• Clinical Features:
• -Fever
• -Roth's Spots (round white spots on retina surrounded by hemorrhage)
• 
• -Osler's nodes (tender lesions on fingers and toes due to septic emboli)
• 
• -Murmur (new)
• -Janeway lesions (painless erythematous lesions on palm or sole due to septic emboli)
• -Anemia of chronic disease
• -Nail-bed hemorrhage (splinter hemorrhage due to septic emboli)
• 
• -Emboli

"FROM JANE"

• Complications:
• -chordae rupture
• -glomerulonephritis
• -suppurative pericarditis
• -emboli

• Etiology:
• -malignancy
• -hypercoagulable state
• -lupus

• Libmann-Sacks Endocarditis:
• -sterile vegetations
• -present on surface and under-surface of mitral valve (mitral regurg)

• Pathophysiology:
• -systemic complication of pharyngitis due to group A β-hemolytic strep
• -antibodies to M protein cross react with human tissue (molecular mimcry)
• -Type II hypersensitivity (not a direct effect of bacteria)

• Acute Rheumatic Fever:
• -2-3 weeks after strep throat
• -early deaths due to myocarditis
• -mitral regurgitation
• -repeat infections may lead to chronic rheumatic heart disease

Presentation: Two Mnemonics

• FEVERSS:
• -Fever
• -Erytema marginatum
• -Valvular damage (vegetation and fibrosis)
• -ESR elevated
• -Red hot joints (migratory polyarthritis)
• -Subcutaneous nodules
• -St. Vitus' dance (Sydenham's chorea)

• JNES
• -Joints (migratory polyarthritis)
• - Pancarditis
• -Nodules
• -Erythema marginatum
• -Sydenham's Chorea

• Pancarditis:
• 1. Endocarditis:
• -mitral > aortic >> tricuspid (high pressure valves affected most)

• 2. Myocarditis:
• -Aschoff bodies (granulma with giant cells)
• -Anitschkow's cells (histiocytes with wavy nuclei)
• -most common cause of death in acute phase

• 3. Pericarditis
• -friction rub and chest pain

• Findings:
• -murmur
• -elevated ESR
• -elevated ASO titres

• Chronic Rheumatic Heart Disease:
• -valve scarring as a result of rheumatic fever
• -results in mitral stenosis (fish mouth appearance)
• -complications: infectious endocarditis

• Presentation:
• -sharp pain
• -aggravated by inspiration
• -relieved by sitting up and leaning forward
• -friction rub
• -EKG: widespread ST elevation and/or PR depression

• Fibrinous
• -Dressler's syndrome
• -uremia
• -radiation
• -loud friction rub

• Serous
• -viral pericarditis (often resolves spontaneously)
• -non-infectious inflammatory diseases (RA, SLE)

• Suppurative/purulent
• -usually caused by bacterial infections (pneumococcus, streptococcus)
• -rare now with antibiotics

• Pathophysiology:
• -compression of heart by fluid (eg: blood, effusions) in pericardium
• -prevents heart from relaxing and expanding between beats
• -leads to decreased CO
• -equilibration of diastolic pressures in all 4 chambers (elevated and equal to pericardial pressure)
• -compressed chambers can't accommodate normal venous return causing an increase in systemic and pulmonary venous pressure

• Common Causes:
• -neoplasm
• -post-viral
• -uremic
• -trauma

• Presentation:
• -hypotension
• -increased venous pressure (JVD)
• -distant heart sounds
• -increase HR-pulsus paradoxus

-decrease in amplitude of systolic blood pressure by ≥ 10 mmHg during inspiration

• Seen in:
• -severe cardiac tamponade
• -asthma
• -OSA
• -pericarditis
• -croup

• Pathophysiology:
• -3° syphilis disrupts the vasa vasorum of the aorta with atrophy of the vessel wall and dilation of the aorta and valve ring

• Pathology:
• -calcifications at aortic root and ascending aortic arch
• -tree bark appearance

• Complications:
• -can result in aneurysm of the ascending aorta or aortic arch → aortic insufficiency

• Epidemiology:
• -most common primary cardiac tumor in adults (still more rare than mets)

• Pathophysiology:
• -90% occur in the atria (mostly LA)
• -benign mesenchymal tumor
• -pedunculated mass in LA can obstruct mitral valve
• 

• Presentation:
• -multiple syncopal episodes due to mitral obstruction

• Pathology:
• -gelatinous appearance
• -abundant ground substance

• Epidemiology:
• -most common primary cardiac tumor in children
• -associated with tuberous sclerosis

• Pathophysiology:
• -benign hamartoma of cardiac muscle
• -usually arises in the ventricle

• Epidemiology:
• -most common heart tumor
• -from breast carcinoma, lung carcinoma, melanoma and lymphoma

• Pathophysiology:
• -most commonly involves the pericardium → pericardial effusion

Increase in JVP on inspiration instead of a normal decrease

• Pathophysiology:
• -Inspiration → negative intrathoracic pressure not transmitted to heart → impaired filling of right ventricle → blood backs up into venae cavae → JVD

• Seen with:
• -constrictive pericarditis
• -restrictive cardiomyopathies
• -R atrial or ventricular tumors
• -cardiac tamponade

• Pathophysiology:
• -decreased blood flow to the skin due to arteriolar vasospasm in response to cold temperature or emotional stress
• -most often in fingers and toes
• -affects small vessels
• 

• Etiology:
• -Primary idiopathic (Raynaud's disease)
• -Secondary (Raynaud's syndrome: mixed CT disease, SLE or CREST)

• Temporal (Giant Cell) Arteritis
• Takayasu Arteritis

• Polyarteritis Nodosa
• Kawasaki Disease
• Buerger Disease

• Wegener Granulomatosis
• Microscopic Polyangitis
• Churg Strauss Syndrome
• Henoch-Schonlein Purpura

Granulomatous vasculitis that involves branches of the carotid artery, segmental lesions

• Epidemiology:
• -female, > 50 years

• Presentation:
• -Unilateral HA
• -Jaw claudication
• -Vision problems (Blindness)
• -associated with polymyalgia rheumatica

• Lab Findings:
• -elevated ESR (>100)
• -bx: giant cells and intimal fibrosis
• -negative bx does not rule out!!!

• Treatment:
• -Corticosteroids (empiric)

Granulomatous vasculitis that involves aortic arch and branch points

• Epidemiology:
• -Young Asian females

• Presentation:
• -pulseless disease
• -fever
• -night sweats
• -arthritis
• -myalgias
• -skin nodules
• -visual and neurologic symptoms

• Lab Findings:
• -elevated ESR

• Treatment:
• -corticosteroids

Necrotizing vasculitis involving multiple organs, sparing the lungs

• Epidemiology:
• -young adults
• -associated with Hepatitis B seropositivity

• Presentation:
• -hypertension (renal artery involvement)
• -abdominal pain with melena
• -neurologic dysfunction
• -cutaneous eruptions

• Lab Findings:
• -transmural fibrinoid necrosis
• -lesions of different ages
• -string of pearls (aneurysms and constrictions)

• Treatment:
• -Corticosteroids
• -Cyclophosphamide

• Epidemiology:
• -Asian children, < 4 years

• Presentation:
• -fever
• -conjunctivitis
• -enlarged cervical LNs
• -Strawberry tongue
• -hand-foot erythema
• "Kid on a Kawasaki motorcycle: palm and sole rash, heart racing"

• Complications:
• -coronary aneurysms, thrombosis (MI)

• Treatment:
• -IVIg
• -ASA

Necrotizing vasculitis involving digits

• Epidemiology:
• -heavy smokers
• -males, <40

• Presentation:
• -gangrene
• -autoamputation of digits
• -superficial nodular phlebitis
• -Raynaud's (neural involvement)

• Treatment:
• -smoking cessation

Necrotizing granulomatous vasculitis involving nasopharynx, lungs and kidneys

• Epidemiology:
• -middle aged men

• Presentation:
• -nasopharyngeal ulceration, otitis media, chronic sinusitis, mastoiditis
• -hemoptysis, cough, dyspnea
• -hematuria

• Lab Findings:
• -c-ANCA
• -CXR: large nodular densities

• Treatment:
• -Cyclophosphamide
• -Corticosteroids

Necrotizing vasculitis involving especially lung and kidney

• Presentation:
• -palpable purpura
• -similar to Wegener
• -but NO nasopharyngeal involvement

• Lab Findings:
• -NO granulomas
• -p-ANCA

• Treatment:
• -corticosteroids
• -cyclophosphamide

Necrotizing granulomatous inflammation with eosinophils affecting especially lung and heart

• Presentation:
• -asthma
• -sinusitis
• -palpable purpura
• -peripheral neuropathy

• Lab Findings:
• -granulomatous vasculitis with eosinophilia
• -p-ANCA

***distinguish from Microscopic Polyangiitis (asthma, granulomas, eosinophilia)

Epidemiology: most common form of childhood systemic vasculitis

• Pathophysiology:
• -IgA immune complex deposition usually following URI

• Presentation (Triad):
• -palpable purpura on buttocks and legs
• -GI pain and melena
• -Arthralgia

-often associated with IgA nephropathy

• Treatment:
• -often self limited-Steroids

• Pathophysiology:
• -congenital disorder that affects capillaries

• Presentation:
• -port-wine stain (nevus flemmeus) on face
• -ipsilaterial leptomeningeal angiomatosis
• -seizures
• -early onset glaucoma

• Strawberry hemangioma
• Cherry hemangioma
• Pyogenic granuloma
• Cystic hygroma
• Glomus tumor
• Bacillary angiomatosis
• Angiosarcoma
• Lymphangiosarcoma
• Kaposi's Sarcoma

Benign capillary hemangioma of infancy

Appears in first few weeks of lifegrows rapidly and regresses spontaneously around 5-8yrs

blanchable

Benign capillary hematoma of the elderly

Does NOT regress

Increased frequency with age

Polypoid capillary hemangioma

May ulcerate and bleed

Associated with trauma and pregnancy

Cavernous lymphangioma of the neck

Associated with Turner Syndrome

Benign, painful, red-blue tumor under fingernails

Arises from modified smooth muscle cells of glomus body

Benign capillary skin papules found in AIDS patients

Caused by Bartonella henselae

Don't confuse with Kaposi Sarcoma

Malignant proliferation of endothelial cells

Highly lethal malignancy of liver

Associated with Polyvinyl chloride (PVC), arsenic and thorotrast exposure

Lymphatic malignancy associated with persistent lymphedema

ie: post radical mastectomy

Low grade malignant proliferation of endothelial cells

Associated with HHV-8 infection

• Seen in:
• -old, eastern european males
• -AIDS
• -transplant recipients

NOT blanchable

Don't mistake for bacillary angiomatosis