cardiology pathology

Question 1

congenital heart diseases

what are the 5 Ts?

Early cyanosis, blue babies become evident immediately after birth

need urgent surgical tx and/or maintenance of PDA

Answer 1

5 Ts

1. Truncus arteriosus
2. transposition
3. tricupid atresia
4. tetralogy of Fallot
5. TAPVR-Total anomalous pulmonary venous return (TAPVR) is a birth defect of the heart. In a baby with TAPVR, oxygen-rich blood does not return from the lungs to the left atrium. Instead, the oxygen-rich blood returns to the right side of the heart.

Question 2

congenital heart disease:

failure of aorticopulmonary septum formation. most patients have accompanying VSD

• heart that does not have pulmonary trunk and aorta

R-L shunt

Answer 2

persistent truncus arteriosus

Question 3

aorta leaves RV (anterior) and pulmonary trunk leaves LV (posterior)–> separation of systemic and pulmonary circulation

this condition is not compatible with life unless there is a shunt to allow mixing of blood (VSD, PDA, patent foramen ovale)

due to failure of aorticopulmonary septum to spiral

R-L shunt

Answer 3

D-transposition of great vessels

Question 4

absence of tricuspid valve and hypoplastic RV

pt needs ASD and VSD for viability

R-L shunt

Answer 4

tricuspid atresia

Question 5

anterosuperior displacement of infundibular septum and most common cause of childhood cyanosis

1. pulmonary infundibular stenosis (most important determinant for prognosis)
2. Right ventricular hypertrophy (boot-shaped heart on CXR)
3. overriding aorta- aorta is positioned directly over a ventricular septal defect (VSD), instead of over the left ventricle.
4. VSD

pulmonary stenosis forces R-L shunt across VSD–> RVH (often caused by crying, fever, exercise due to exacerbation of RV outflow obstruction)

squatting increases afterload, decrease shunting, improves cyanosis

associated with DiGeorge and need early surgical correction

DiGeorge syndrome- 22q11.2 deletion

R-L shunt

Answer 5

Tetralogy of Fallot

Question 6

congenital heart disease

pulmonary veins drain into right heart circulation (SVC, coronary sinus, etc) associated with ASD and sometimes PDA to allow R-L shunting to maintain CO

R-L shunt

Answer 6

total anomalous pulmonary venous return

Question 7

congenital heart disease

characterized by displacement of tricuspid valve leaflet downward into RV, “atrializing” the ventricle

associated with TR, accessory condution pathway, right HF

caused by lithium exposure in utero

R-L shunt

Answer 7

Ebstein anomaly

Question 8

Left-Right shunts: acyanotic at presentation; cyanosis may occur years later

Answer 8

R-L: early cyanosis

L-R: later cyanosis

Question 9

most common congenital cardiac defect

asymptomatic at birth and manifest weeks or later or remain asymptomatic throughout life

most are self-resolving; larger lesion may lead to LV overload and HF

L-R shunt

Answer 9

ventricular septal defect

O2 saturation increase in RV and pulmonary artery.

frequency: VSD> ASD> PDA

Question 10

congenital heart disease

defect in interatrial septum

-wide, fixed split S2

Ostium secundum defects most common

O2 saturation increased in RA, RV and pulmonary artery. may lead to paradoxical emboli

associated with Down syndrome

distinct from foramen ovale in that septa are missing tissue rather than unfused

L-R shunt

Answer 10

atrial septal defect

Question 11

PDA

fetal period, shunt is right to left (normal)

in neonatal period, decreased pulmonary vascular ressitance–> shunt becomes left to right–> progressive RVH and/or LVH and HF

associated with continuous, machine-like murmur

patency is maintained by PGE synthesis and low O2 tension.

uncorrected PDA can eventually result in late cyanosis in the lower extremities (differential cyanosis)

Answer 11

Endomethacin (indomethacin) ends patency,

PGE keeps it open- could be needed to sustain life such as in transposition of the great vessels

Question 12

uncorrected Left-Right shunt (VSD, ASD, PDA) –> increase pulmonary blood flow–> pathologic remodeling of vasculature–> pulmonary arterial hypertension.

RVH occurs compensate–> shunt becomes right to left shunt

cause late cyanosis, clubbing and polycythemia

Answer 12

Eisenmenger syndrome

Question 13

aortic narrowing near insertion of ductus arteriosus (juxtaductal) associated with bicuspid aortic valve and turner syndrome.

HTN of upper extermities and weak, delayed pulse in lower extremities (brachial-femoral delay)

with age, the intercostal arteries enlarge due to collateral circulation. arteries erode ribs--> notched appearance on CXR.

complication include HF, increase cerebral hemorrhage (berry aneurysm), aortic rupture and possible endocarditis

Answer 13

coarctation of aorta

Question 14

1. alcohol exposure in utero (fetal alcohol syndrome)
2. congenital rubella
3. down syndrome
4. infant of diabetic mother
5. marfan syndrome
6. prenatal lithium exposure
7. Turner syndrome
8. William syndrome
9. 22q11 syndrome

Answer 14

1. VSD, PDA, ASD, teterlogy of Fallot
2. PDA, pulmonary artery stenosis, septal defects
3. AV septal defect (endocardial cushion defect), VSD, ASD
4. Transposition of great vessel, VSD
5. MVP, thoracic aotic aneurysm and dissection, aortic regurgitation
6. Ebstein anomaly
7. biscupsid aortic valve, coarctation of aorta
8. supravalvular aortic stenosis
9. Truncus arteriosus, tetralogy of Fallot

Question 15

HTN

persistent systolic BP > 130 and or diastolic > 80

RF: advancing age, diabetes, inactivity, excess salt, excess alcohol, cigarette, FH, African> causcasian> Asian

90% of HTN are essential- related to increase CO and TPR

remaining are seconday- renal/renovascular diseases like fibromuscular dysplasia (string of beads appearance of renal arteries in women of child-bearing age) and atherosclerotic renal artery stenosis or primary hyperaldoseronism

Answer 15

Hypertensive urgency- severe (>180/>120) HTN without acute end-organ damage

Hypertensive emergency- severe HTN with evidence of acute-end organ damage (encephalopathy, stroke, retinal hemorrhages and exudates, papilledema, MI, HF, aortic dissection, kidey injury, microangiopathic hemolytic anemia, eclampsia)

Question 16

hyperlipidemia signs

xanthomas-plaques composed of lipid-laden histiocytes in skin and especially the eyelids (xanthelasma)

Tendinous xanthoma-lipid deposit in tendon esp, achilles

corneal arcus-lipid deposit in corea. common in elderly (arcus senilis) but earlier in life with hypercholesterolemia

Answer 16

arteriosclerosis-hardening of arteries, with arterial wall thickening and loss of elasticity

arteriolosclerosis-commonly affects small arteries and arterioles.

2 types of arteriolosclerosis:

1. hyaline (thickening of vessel walls in essential HTN or diabetes mellitus) and

2. hyperplastic (onion skinning in severe HTN with proliferation of smooth muscle cells)

Monckeberg sclerosis (medial calcific sclerosis)- uncommon. affects medium arteries, calcification of the internal elastic lamina and media of arteries–> vascular stiffening without obstruction.

pepestern appearance on xray and does not obstruct blood flow. intima is not involved

Question 17

Atherosclerosis

disease of elastic arteries and large-medium sized muscular arteries; a form of arterosclerosis caused by buildup of cholesterol plaques

abdominal aorta> coronary artery> popliteal artery> carotid artery

sx: angina, claudication-(Pain, commonly in the legs, caused by too little blood flow, usually during exercise. Often indicates peripheral artery disease.), but can be asymptomatic

progression: endothelial cell dysfunction–> marcophage and LDL accumulation–> foam cell formation–> fatty streak–> smooth muscle cell migration (involves acculumation PDGF and FGF), proliferation, and extracellular matrix deposition–> fibrous plaque–> complex atheroma

complication: aneurysm, ischemia, infarcts, peripheral vascular disease, thrombus, emboli

Answer 17

aortic aneurysm

localized dilation of the aorta causing abdominal and or back pain, which is a sign of leaking, dissection or imminent rupture

abdominal aortic aneurysm- associated with atherosclerosis. palpable pulsatile abdominal mass

most are infrarenal (distal to origin of renal arteries)

thoracic aortic aneurysm-associated with c_ystic medial degeneration_. risk factor include HTN, Bicuspid aortic valve, connective tissue disease (marfan syndrome)

also associated with 3’ syphilis (obliterative endarteritis of vasa vasorum) aortic root dilation may lead to aortic valve regurgitation

Traumatic aortic rupture-due to trauma and or deacceleration injury, most commonly at aortic isthmus (proximal descending aorta just distal to origin of left subclavian artery)

xray may should widened mediastinum.

Question 18

longitudinal intimal tear forming a false lumen associated with HTN, bicuspid, Marfans,

can present with tearing, sudden onset of chest pain that radiates to the back .

CXR shows mediastinal widening.

stanford type A: (proximal)- involves ascending aorta. may extend to aortic arch or desencing aorta. may result in acute aortic regurg or cardiac tamponade

type B: (distal)- involves only descending aorta (below left subclavian artery) treated medically with beta-blockers, then vasodilators

Answer 18

ischemic heart disease manifestation

angina- chest pain due to ischemic myocardium secondary to coronary artery narrowing or spasm; no myocyte necrosis

1. stable: usually secondary atherosclerosis (>70% occulsion)- exertional chest pain in classic distribution (usually with ST depression on EKG) resolving with rest or nitroglycerin

2. vasospastic (also known as prinzmetal or variant)- occurs at rest secondary to coronary artery spasm; transient ST elevation on EKG, smoking is risk factor; HTN and hypercholesterolemia are not. trigger include cocaine, alcohol, triptans. treat with Ca2+ channel blockers, nitrates and smoking cessation

unstable: thrombosis with incomplete coronary artery occlusion, +/- ST depression and T wave inversion on EKG but no cardiac biomarker elevation (unlike NSTEMI); infrequency or intensity of chest pain or any chest pain at rest

coronary steal syndrome- distal to coronary stenosis, vessels are maximally dilated at baseline. adminsteration of of vasodilators (dipyramidole, regadenoson) dilates normal vessels–> blood is shunted toward well-perfused areas--> ischemia myocardium perfused by stenosed vessels.

principle behind pharmacologic stress tests with coronary vasodilators

Question 19

ischemic heart disease manifestations

sudden cardiac death- death from cardiac within 1 hour onset on sx. most commonly due to lethal arrhythmia (VF). associated with CAD, cardiomyopathy (hypertrophic, dilated) and hereditary ion channelopathies (long QT syndrome, Brugada syndrome). prevent with ICD

Chronic ischemic heart disease- progressive onset of HF over many years due to chronic ischemic myocardial damage

myocardial infarction- most often rupture of coronary artery atherosclerotic plaque--> acute thrombosis. increase cardiac biomarkers (CK-MB, troponins) are diagnostic.

non-STEMI-subendocardial infarcts- subendocardium (inner 1/3) especially vulnerable to ischemia. ST depression on EKG

ST- segment elevation MI (STEMI)- transmural infarcts, full thickness of myocardial wall involved. ST elevation, Q waves on EKG

Answer 19

evolution of myocardial infarction

occluded LAD>RCA>LCX

sx: diaphoresis, nausea, vomiting, severe retrosternal pain, pain in left arm and or jaw, SOB, fatigue

0-24hr- Gross: none. microscopically: early coagulative necrosis, wavy fibers, reprofusion injury, complication: Venticular arrhythmia, HF, cardiogenic shock

Pathoma: <4 hours: gross changes: none, microscopic: none. complication: cardiogenic shock (massive infarction) congestive heart failure, arrhythmia

4-24 hours: gross changes: dark discoloration, microscopic: coagulative necrosis. complication arrhythmia

pathoma: 1-3 days: gross changes: yellow pallor, microscopic changes: neutrophils, complication: fibrinous pericarditis represented as chest pain with friction rub
pathoma: 4-7 days: gross: yellow pallor, microscopic: marcophages, complication: rupture of ventricular free wall (lead to cardiac tamponade), interventicular septum (leads to shunt) or papillary muscle (leads to mitral insufficiency)

1-3 weeks gross: red border emerges as granulation tissue enters from edge of infarct, microscopic: granulation tissue

months: gross changes: white scar, microscopic changes: fibrosis, complication: Aneurysm, mural thrombus or dressler syndrome

Question 20

in first 6 hours: EKG is gold standard

cardiac troponin I rise after 4 hours (peaks 24hrs) last form 7-10 days

CK-MB- rise 6-12 hours (peak 16-24 hr) good for diagnosing reinfarction following MI because level return to normal in 48 hours

EKG localization of STEMI

location of infacrion and where to see ST elevation or Q waves:

anteroseptal (LAD): V1-2

anteroapical (distal LAD): V3-4

anterolateral (LAD or LCX): V5-6

Lateral (LCX): I, aVL

inferior (RCA) II, III, aVF

posterior (PDA): V7-9, ST depression in V1-3 with tall R waves

Answer 20

myocardial infarction complication

cardiac arrhythmia- within first few days after MI, important cause of death before reaching the hospital and within 24 hours post-MI

Post-infarction fibrinous pericarditis: 1-3 days friction rub

papillary muscle rutpure: 2-7 days: posteriomedial papillary muscle rupture. increase risk due to single blood supply from posterior descending artery. can result in severe mitral regurg

interventricular septal rupture: 3-5 days: macrophage-mediated degradation–> VSD–> increase O2 sat and pressure in RV

ventricular pseudoaneurysm formation: 3-14 days: free wall rupture contained by adherent pericardium or scar tissue: decrease CO, increase risk of arrhythmia, embolus from mural thrombus

ventricular free wall rupture: 5-14 days: free wall rupture–> cardiac tamponade. LV hypertrophy and previous MI protect against free wall rupture. Acute form usually leads to sudden death

true ventricular aneurysm: 2wks to several months: outward bulge with contraction (dyskinesia) associated with fibrosis

Dressler syndrome: several weeks: autoimmune phenomenon resulting in fibrinous pericarditis

LV failure and pulmonary edema: can occur secondary to LV infarction, VSD, free wall rupture, papillary muscle rupture with mitral regurg.

Question 21

unstable angina/NSTEMI- anticoagulation (heparin), antiplatelet therapy (aspirin) + ADP (adenosine diphosphate) receptor inhibitor (clopidogrel), B-blocker, ACE inhibitors, statins. symptom control with nitroglycerin and morphine

STEMI- in addition to above, reperfusion therapy most important (percutaneous coronary intervention preferred over fibrinolysis)

Question 22

Most common cardiomyopathy (90% of cases). Often idiopathic or familial. Other etiologies include chronic Alcohol abuse, wet Beriberi, Coxsackie B viral myocarditis, chronic Cocaine use, Chagas disease, Doxorubicin toxicity (ABCCCD), hemochromatosis, sarcoidosis, thyrotoxicosis, peripartum cardiomyopathy.

Findings: HF, S3, systolic regurgitant murmur, dilated heart on echocardiogram, balloon appearance of heart on CXR.

Leads to systolic dysfunction. Dilated cardiomyopathy displays eccentric hypertrophy (sarcomeres added in series). ABCCCD.

Takotsubo cardiomyopathy: broken heart syndrome—ventricular apical ballooning likely due to increased sympathetic stimulation (eg, stressful situations).

Treatment: Na+ restriction, ACE inhibitors, β-blockers, diuretics, digoxin, ICD, heart transplant.

Answer 22

dilated cardiomyopathies

Question 23

60–70% of cases are familial, autosomal dominant (most commonly due to mutations in genes encoding sarcomeric proteins, such as myosin binding protein C and β-myosin heavy chain).

Causes syncope during exercise and may lead to sudden death (eg, in young athletes) due to ventricular arrhythmia.

Findings: S4, systolic murmur. May see mitral regurgitation due to impaired mitral valve closure.

Diastolic dysfunction ensues. Marked ventricular concentric hypertrophy (sarcomeres added in parallel), often septal predominance. Myofibrillar disarray and fibrosis.

Physiology of HOCM—asymmetric septal hypertrophy and systolic anterior motion of mitral valve –> outflow obstruction –> dyspnea, possible syncope.

Other causes of concentric LV hypertrophy: chronic HTN, Friedreich ataxia.

Treatment: cessation of high-intensity athletics, use of β-blocker or non-dihydropyridine Ca2+ channel blockers (eg, verapamil). ICD if patient is high risk

Answer 23

hypertrophic obstructive cardiomyopathy

Question 24

Postradiation fibrosis, Löffler endocarditis-(abnormal endomyocardial infiltration of eosinophils, with subsequent tissue damage from degranulation, eventually leading to fibrosis,) Endocardial fibroelastosis (thick fibroelastic tissue in endocardium of young children), Amyloidosis, Sarcoidosis, Hemochromatosis (although dilated cardiomyopathy is more common)

(Puppy LEASH).

Diastolic dysfunction ensues. Can have low-voltage ECG despite thick myocardium (especially in amyloidosis).

Löffler endocarditis—associated with hypereosinophilic syndrome; histology shows eosinophilic infiltrates in myocardium.

Answer 24

restrictive/infiltrative cardiomyopathy

Question 25

Clinical syndrome of cardiac pump dysfunction–> congestion and low perfusion. Symptoms include dyspnea, orthopnea, fatigue; signs include S3 heart sound, rales (crackles), jugular venous distention (JVD), pitting edema.

Systolic dysfunction—reduced EF, increase EDV; decrease contractility often 2° to ischemia/MI or dilated cardiomyopathy.

Diastolic dysfunction—preserved EF, normal EDV; decrease compliance (increase EDP) often 2° to myocardial hypertrophy.

Right HF most often results from left HF. Cor pulmonale refers to isolated right HF due to pulmonary cause.

ACE inhibitors or angiotensin II receptor blockers, β-blockers (except in acute decompensated HF), and spironolactone decrease mortality.

Thiazide or loop diuretics are used mainly for symptomatic relief.

Hydralazine with nitrate therapy improves both symptoms and mortality in select patients

Answer 25

heart failure

Question 26

Left heart failure

Orthopnea: Shortness of breath when supine: increase venous return from redistribution of blood (immediate gravity effect) exacerbates pulmonary vascular congestion.

Paroxysmal nocturnal dyspnea: Breathless awakening from sleep: increase venous return from redistribution of blood, reabsorption of peripheral edema, etc.

Pulmonary edema: increase pulmonary venous pressure –> pulmonary venous distention and transudation of fluid. Presence of hemosiderin-laden macrophages (“HF” cells) in lungs

Answer 26

Right heart failure

Hepatomegaly (nutmeg liver) increase central venous pressure –> increase resistance to portal flow. Rarely, leads to “cardiac cirrhosis.”

Jugular venous distention increase venous pressure.

Peripheral edema increase venous pressure –> fluid transudation

Question 27

hypovolemic shock-caused by hemorrhage, dehydration, burns, skin: cold, clammy. decrease PCWP (preload) and CO. incresae SVR (afterload), treatment: IV fluid

cardiogenic shock: caused by acute MI, HF, valvular dysfunction, arrhythmia. treatment: inotropes, diuresis

obstructive shock: cardiac tamponade, pulmonary embolism, tension pneumothroax, treatment: relieve obstruction

both cardiogenic and obstructive shock have cold clammy skin, decrease CO, increased afterload

distribution shock caused by sepsis, anaphylaxis, CNS injury, skin: warm, dry, decreased PCWP and SVR (afterload) decreased, treatment: IV fluid, pressors, epinephrine (anaphylaxis)

Question 28

Acute—S aureus (high virulence). Large vegetations on previously normal valves A . Rapid onset.

Subacute—viridans streptococci (low virulence). Smaller vegetations on c_ongenitally abnormal or diseased valves_. Sequela of dental procedures. Gradual onset.

Symptoms: fever (most common), new murmur, Roth spots (round white spots on retina surrounded by hemorrhage (B) ), Osler nodes (tender raised lesions on finger or toe pads (C) due to immune complex deposition), Janeway lesions (small, painless, erythematous lesions on palm or sole) (D), splinter hemorrhages (E) on nail bed.

Associated with glomerulonephritis, septic arterial or pulmonary emboli. May be nonbacterial (marantic/thrombotic) 2° to malignancy, hypercoagulable state, or lupus.

Fever

Roth spots

Osler nodes

Murmur

Janeway lesions

Anemia

Nail-bed hemorrhage

Emboli

Requires multiple blood cultures for diagnosis. If culture ⊝, most likely Coxiella burnetti, Bartonella spp, HACEK (Haemophilus, Aggregatibacter [formerly Actinobacillus], Cardiobacterium, Eikenella, Kingella). Mitral valve is most frequently involved. Tricuspid valve endocarditis is associated with IV drug abuse (don’t “tri” drugs). Associated with S aureus, Pseudomonas, and Candida.

S bovis (gallolyticus) is present in colon cancer

S epidermidis on prosthetic valves

Answer 28

bacterial endocarditis

Question 29

A consequence of pharyngeal infection with group A β-hemolytic streptococci. Late sequelae include rheumatic heart disease, which affects heart valves—mitral > aortic >> tricuspid (high-pressure valves affected most). Early lesion is mitral valve regurgitation; late lesion is mitral stenosis.

Associated with Aschoff bodies (granuloma with giant cells [blue arrows in A]), Anitschkow cells (enlarged macrophages with ovoid, wavy, rod-like nucleus [red arrow in A]), increase antistreptolysin O (ASO) titers.

Immune mediated (type II hypersensitivity); not a direct effect of bacteria. Antibodies to M protein cross-react with self antigens (molecular mimicry).

J♥NES (major criteria):

Joint (migratory polyarthritis)

♥ (carditis)

Nodules in skin (subcutaneous)

Erythema marginatum (evanescent rash with ring margin)

Sydenham chorea

Treatment/prophylaxis: penicillin

Answer 29

rheumatoid arthritis

Question 30

Inflammation of the pericardium [A , red arrows].

Commonly presents with sharp pain, aggravated by inspiration, and relieved by sitting up and leaning forward.

Often complicated by pericardial effusion [between yellow arrows in A]. Presents with friction rub. ECG changes include widespread ST-segment elevation and/or PR depression.

Causes include idiopathic (most common; presumed viral), confirmed infection (eg, coxsackievirus B), neoplasia, autoimmune (eg, SLE, rheumatoid arthritis), uremia, cardiovascular (acute STEMI or Dressler syndrome), radiation therapy

Answer 30

acute pericarditis

Question 31

Inflammation of myocardium –> global enlargement of heart and dilation of all chambers.

Major cause of SCD in adults < 40 years old. Presentation highly variable, can include dyspnea, chest pain, fever, arrhythmias (persistent tachycardia out of proportion to fever is characteristic).

Multiple causes:

ƒ Viral (eg, adenovirus, coxsackie B, parvovirus B19, HIV, HHV-6); lymphocytic infiltrate with focal necrosis highly indicative of viral myocarditis.

ƒ Parasitic (eg, Trypanosoma cruzi, Toxoplasma gondii) ƒ

Bacterial (eg, Borrelia burgdorferi, Mycoplasma pneumoniae)

ƒ Toxins (eg, carbon monoxide, black widow venom)

ƒ Rheumatic fever

ƒ Drugs (eg, doxorubicin, cocaine)

ƒ Autoimmune (eg, Kawasaki disease, sarcoidosis, SLE, polymyositis/dermatomyositis)

Complications of myocarditis: include sudden death, arrhythmias, heart block, dilated cardiomyopathy, HF, mural thrombus with systemic emboli.

Answer 31

myocarditis

Question 32

Compression of the heart by fluid (eg, blood, effusions [arrows in A] in pericardial space) –> decrease CO. Equilibration of diastolic pressures in all 4 chambers.

Findings: Beck triad (hypotension, distended neck veins, distant heart sounds), increased HR, pulsus paradoxus. ECG shows low-voltage QRS and electrical alternans (due to “swinging” movement of heart in large effusion).

Pulsus paradoxus—decrease in amplitude of systolic BP by > 10 mm Hg during inspiration. Seen in cardiac tamponade, asthma, obstructive sleep apnea, pericarditis, croup.

Answer 32

cardiac tamponade

Question 33

3° syphilis disrupts the vasa vasorum of the aorta with consequent atrophy of vessel wall and dilatation of aorta and valve ring.

May see calcification of aortic root, ascending aortic arch, and thoracic aorta. Leads to “tree bark” appearance of aorta.

Can result in aneurysm of ascending aorta or aortic arch, aortic insufficiency.

Answer 33

syphilitic heart disease

Question 34

Usually elderly females. Unilateral headache (temporal artery), jaw claudication. May lead to irreversible blindness due to ophthalmic artery occlusion.

Associated with polymyalgia rheumatica.-inflammatory disorder that causes muscle pain and stiffness, especially in the shoulders, quick onset and worse in the morning

Most commonly affects branches of carotid artery.

Focal granulomatous inflammation (A).

increase ESR.

Treat with high-dose corticosteroids prior to temporal artery biopsy to prevent blindness.

Answer 34

giant cell (temporal) arteritis (large-vessel vasculitis)

Question 35

Usually Asian females < 40 years old. “Pulseless disease” (weak upper extremity pulses), fever, night sweats, arthritis, myalgias, skin nodules, ocular disturbances.

Granulomatous thickening and narrowing of aortic arch and proximal great vessels (B).

increase ESR.

Treat with corticosteroids.

Answer 35

Takayasu arteritis

Question 36

Usually middle-aged men.

Hepatitis B seropositivity in 30% of patients. Fever, weight loss, malaise, headache. GI: abdominal pain, melena.

Hypertension, neurologic dysfunction, cutaneous eruptions, renal damage.

Typically involves renal and visceral vessels, not pulmonary arteries.

Transmural inflammation of the arterial wall with fibrinoid necrosis.

Different stages of inflammation may coexist in different vessels.

Innumerable renal microaneurysms (F) and spasms on arteriogram.

Treat with corticosteroids, cyclophosphamide

Answer 36

polyarteritis nodosa- (medium-vessel vasculitis)

Question 37

Asian children < 4 years old.

Conjunctival injection, Rash (polymorphous –> desquamating), Adenopathy (cervical), Strawberry tongue (oral mucositis) (D), Hand-foot changes (edema, erythema), fever.

CRASH and burn
May develop coronary artery aneurysms (E) ; thrombosis or rupture can cause death.

Treat with IV immunoglobulin and aspirin.

Answer 37

Kawasaki disease (mucocutaneous lymph node syndrome) (medium-vessel vassculitis)

Question 38

Heavy smokers, males < 40 years old.

Intermittent claudication may lead to gangrene (C) , autoamputation of digits, superficial nodular phlebitis. Raynaud phenomenon is often present.

Segmental thrombosing vasculitis with vein and nerve involvement.

Treat with smoking cessation.

Answer 38

Buerger disease (thromboangiitis obliterans) (medium-vessel vasculitis)

Question 39

Upper respiratory tract: perforation of nasal septum, chronic sinusitis, otitis media, mastoiditis.

Lower respiratory tract: hemoptysis, cough, dyspnea.

Renal: hematuria, red cell casts

Triad:

ƒ Focal necrotizing vasculitis

ƒ Necrotizing granulomas in the lung and upper airway

ƒ Necrotizing glomerulonephritis

PR3-ANCA/c-ANCA, (anti-proteinase 3).

CXR: large nodular densities.

Treat with cyclophosphamide, corticosteroids

Answer 39

Granulomatosis with polyangiitis (Wegener) (small-vessel vasculitis)

Question 40

Necrotizing vasculitis commonly involving lung, kidneys, and skin with pauci-immune-(minimal evidence of hypersensitivity upon immunofluorescent staining for IgG) glomerulonephritis and palpable purpura.

Presentation similar to granulomatosis with polyangiitis but without nasopharyngeal involvement.

No granulomas.

MPO-ANCA/p-ANCA (antimyeloperoxidase).

Treat with cyclophosphamide, corticosteroids.

Answer 40

Microscopic polyangiitis

Question 41

High incidence in Turkish and eastern Mediterranean descent.

Recurrent aphthous ulcers, genital ulcerations, uveitis, erythema nodosum. Can be precipitated by HSV or parvovirus. Flares last 1–4 weeks.

Immune complex vasculitis. Associated with HLA-B51.

Answer 41

Behçet syndrome (small-vessel vasculitis)

Question 42

Asthma, sinusitis, skin nodules or purpura, peripheral neuropathy (eg, wrist/foot drop).

Can also involve heart, GI, kidneys (pauci immune glomerulonephritis).

Granulomatous, necrotizing vasculitis with eosinophilia

MPO-ANCA/p-ANCA, increased IgE level.

Answer 42

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (small-vessel vasculitis)

Question 43

Also known as Henoch-Schönlein purpura.

Most common childhood systemic vasculitis.

Often follows URI.

Classic triad:

ƒ 1. Skin: -palpable purpura on buttocks/legs (I)

ƒ 2. Arthralgias,

3. GI: abdominal (associated with intissusception)

Vasculitis 2° to IgA immune complex deposition. Associated with IgA nephropathy (Buerger disease).

Answer 43

Immunoglobulin A vasculitis (small-vessel vasculitis)

Question 44

often due to viral infection, especially HCV

triad

1. palpable purpura
2. weakness
3. arthralgia

may also have peripheral neuropathy and renal disease (glomerulonephritis)

cryoglobulin are immunoglobulin that precipate in the cold

vasculitis due to mixed IgG and IgA immune complex deposition

Answer 44

mixed cryoglobulinemia (small-vessel vasculitis)

Question 45

Most common 1° cardiac tumor in adults

90% occur in the atria (mostly left atrium).

Myxomas are usually described as a “ball valve” obstruction in the left atrium (associated with multiple syncopal episodes). May auscultate early diastolic “tumor plop” sound.

Histology: gelatinous material, myxoma cells immersed in glycosaminoglycans.

Most common heart tumor is a metastasis (eg, melanoma).

Answer 45

Myxomas (cardiac tumors)

Question 46

Most frequent 1° cardiac tumor in children (associated with tuberous sclerosis). Histology: hamartomatous growths

Answer 46

Rhabdomyomas (cardiac tumors)

Question 47

increased in JVP on inspiration instead of a normal decrease.

Inspiration –> negative intrathoracic pressure not transmitted to heart –> impaired filling of right ventricle –> blood backs up into vena cava –> JVD.

May be seen with constrictive pericarditis, restrictive cardiomyopathies, right atrial or ventricular tumors.

Answer 47

Kussmaul sign

Question 48

Also known as Osler-Weber-Rendu syndrome. Inherited disorder of blood vessels.

Findings: blanching lesions (telangiectasias) on skin and mucous membranes, recurrent epistaxis, skin discolorations

arteriovenous malformations (AVMs),

GI bleeding, hematuria.

Answer 48

Hereditary hemorrhagic telangiectasia