B4: Path Exam 2

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aschoff body in acute rheumatic heart disease

atrial myxoma

bat's wing/butterfly opacities from LSCHF

borderline myocarditis

vulnerable plaque

calcified pericardium from constrictive pericarditis

carcinoid heart disease

cardiac rhabdomyoma

cardiomyocyte disarray w/ hyperplasia of interstitial cells (hypertrophic cardiomyopathy)

catfish mouth formation in chronic rheumatic heart disease

cavernous hemangioma

chronic rejection of cardiac transplant

concentric hypertrophy

congenital bicuspid aortic stenosis

contraction band necrosis seen in myocardial infarction

eccentric hypertrophy

epithelioid hemangioendothelioma

erythema marginatum in acute rheumatic fever

giant cell myocarditis

hard plaque

histology of a mature infantile hemangioma

histology of an early infantile hemangioma

hyperacute rejection of cardiac transplant

hypomelanotic macules (ash-leaf spots) in tuberous sclerosis complex

infantile hemangioma

janeway lesion in infective endocarditis

kaposi sarcoma

kaposi sarcoma spindle cells

kerley B lines from LSCHF

left ventricular hypertrophy

liquefactive myocytolysis seen in MI

lung tissue in LSCHF

lymphangioma circumscriptum

lymphocytic myocarditis

macrocystic lymphangioma

mitral valve leaflet with thrombotic vegetations from non-bacterial thrombotic endocarditis

mitral valve prolapse

normal heart

nutmeg liver from RSCHF

osler node in infective endocarditis

papillary fibroelastoma

pyogenic granuloma

rheumatic aortic stenosis

rheumatic endocarditis

ruptured vulnerable plaque

salmon patch (nevus simplex)

spider angioma

splinter (subungual) hemorrhage in infective endocarditis

subcutaneous nodules in acute rheumatic fever

telangiectasia

thick pericardium from chronic pericarditis

transmural infarction (STEMI) scar stained with Masson Trichrome Stain

unequivocal myocarditis

valvular vegetations in non-bacterial thrombotic endocarditis

What disorder: "Lipomatous hamartoma" -Genetics/RF: Elderly and obese -Etiology/Pathogenesis: Excess accumulations of mature adipose and brown fat mixed w residual cardiomyocytes → un-encapsulated mass Proliferation of fat cells (not hypertrophy) -Diagnosis/Prognosis: CT/MRI: dumbbell- shaped mass confined to interatrial septum -Pathology: Gross: > 2 cm mass Micro: Mature adipose w cells resembling brown fat, vacuolated cytoplasm and centrally placed nuclei -Presentation: Asymptomatic Arrhythmias (rare): Afib, APC, SVT, ectopic atrial rhythm, junctional rhythm

Lipomatous Hypertrophy of the Atrial Septum

mitral annular calcification

What are the 5 most common cardiac tumors that have no malignant potential and account for 90% of all primary heart tumors (from most common to least common)?

1. myxomas 2. fibromas 3. lipomas 4. papillary fibroelastomas 5. rhabdomyomas

What disorder: -Etiology/Pathogenesis: Discrete focus of ischemic necrosis in myocardium Most from atherosclerosis of coronary a. Other mechanisms: coronary a. Spasm, coronary vasculitis, emboli, vaso- occlusive crises of sickle cell, aortic dissection, supply-demand imbalance LAD a.: 50% of cases; proximal portion → necrosis of anterior wall of LV, anterior portion of interventricular septum, and apex RCA: 30% of cases; proximal portion → infarct in inferior/posterior wall of LV, posterior portion of interventricular septum and, in some cases, inferior/posterior wall of RV LCX a: 20% of cases; infarct of lateral wall of LV EXCEPT at the apex Hypokinesis → akinesis → dyskinesis -Diagnosis/Prognosis: Prognosis: restoration of flow w/n first 4-6 hrs assoc w salvage of ischemic myocardium, improved mortality - Better if w/n 1-2 hrs - ultimate infarct size is major predictor of long-term outcome Complications: arrhythmias, cardiogenic shock, infarct extension + expansion, myocardial rupture, RV infarct, pericarditis, ventricular aneurysm, mural thrombus + embolization Light microscopy: "wavy fibers" w/n first hour - Irrev. injured myocardial cells that have been stretched - 12-24 hrs: contraction band necrosis - 24-48 hrs: coagulative necrosis - 1-3 d: acute inflammation, PMNs - 4 d: macs at border, granulation tissue Effects of reperfusion: contraction band necrosis, hemorrhagic infarct, no-reflow phenomenon, myocardial stunning, hibernating myocardium Elevated troponin I and T, CK-MB -Pathology: "Wavefront phenomenon" = irreversible injury begins in subendocardium → after 20 min, spreads to epicardium Not identifiable w/n first 12 hrs 2-3 h w/ tetrazolium salt solutions → triphenyl tetrazolium chloride (TTC) sensitive to presence of tissue dehydrogenase enzyme activity, which is depleted in infarcted myocardium → unstained pale zone (infarct), brick red color (non-infarct) 12 h: pallor of infarcted myocardium 2-3 days: central yellow area surrounded by thin peripheral dark red rim 4-7 days: central, yellow area surrounded by depressed, thicker, dark-red rim (represents young granulation tissue) → progressively enlarges + becomes gray (young scar tissue) When healing complete: granulation tissue matures into a young (gray) and later a mature (white) scar -Presentation: Infarction > 25% of LV = CHF Infarction > 40% of LV = cardiogenic shock Sx: Sudden onset severe chest pain (constricting, crushing oppressing, or compressing) >30 min - hours in the retrosternal location frequently spreading to both sides of anterior chest with predilection for the left side, not relieved by nitroglycerin Often radiates down ulnar aspect of L arm N/v in pts w inferior infarct Weakness, dizziness, palpitations, cold sweat, sense of impending doom Cardio shock: ↓ urine output, altered mentation, hypotension, JVD

myocardial infarct

What are the 4 subtypes of IHD resulting from myocardial ischemia?

1. angina pectoris 2. myocardial infarction 3. chronic IHD with CHF 4. sudden cardiac death

Transplantation agents responsible for transplant ejection are primarily ______________ with a smaller contribution of minor histocompatibility antigens, and tissue specific antigens

ABO blood group antigens and major histocompatibility antigens

"banana-like" asymmetric configuration of hypertrophic cardiomyopathy

1 day old myocardial infarction

1 hour old myocardial infarction (wavy fibers)

3 days old myocardial infarction

Advanced bronchiolitis obliterans syndrome (chronic rejection of lung transplant) w/ complete fibrous obliteration of airway lumen w/ Masson Trichrome Stain

Anti-Schkow cell in acute rheumatic heart disease

Aschoff giant cell in acute rheumatic heart disease

Endocardial metastasis by pharyngeal squamous cell carcinoma

Epicardial metastasis by malignant mesothelioma

Fibrous plaque on endocardium w/ Trichrome stain that shows underlying elastic tissue (black) and acid Mucopoly-saccharides (blue-green) in carcinoid heart disease

LSCHF

Lipomatous Hypertrophy of the Atrial Septum

Myocardial metastasis by bladder carcinoma

Myocardial metastasis by malignant melanoma

acquired calcific aortic stenosis of tricuspid

acute Ab-mediated rejection of cardiac transplant

acute Ab-mediated rejection of cardiac transplant w/ immunoperoxidase staining for Cd4

acute Ab-mediated rejection of lung transplant with immunohistochemistry staining for Cd4

acute cell-mediated rejection of cardiac transplant

acute cell-mediated rejection of lung transplant

alveoli in LSCHF

angiofibromas in tuberous sclerosis complex

angiosarcoma

bronchiolitis obliterans syndrome (chronic rejection of lung transplant)

fibrous pericarditis

What type of acute pericarditis is this?

hemorrhagic pericarditis

What type of acute pericarditis is this?

purulent pericarditis

What type of acute pericarditis is this?

serous pericarditis

What type of acute pericarditis is this?

purulent exudate

What type of fluid is shown in the tube, drawn from a pericardial effusion?

serous exudate

What type of fluid is shown in the tube, drawn from a pericardial effusion?

chylous

What type of pericardial effusion was this fluid likely drawn from?

hydropericardium

What type of pericardial effusion was this fluid likely drawn from?

serosanguineous

What type of pericardial effusion was this fluid likely drawn from?

Which complication of an acute MI: -Early post-infarct pericarditis from transmural acute MIs → epicardial manifestation of underlying myocardial inflammation -Manifests as anterior CP and pericardial friction rub that appears 2-3 days after infarct and gradually resolves over next few days -Delayed post-infarct (Dressler syndrome) is immune mediated → Ab produced against damaged myocardium → Fibrinous pericardial effusion w/ Sx occurring 2-3 weeks following acute MI a. percarditis b. ventricular aneurysm c. infarct extension d. mural thrombus + embolization e. myocardial rupture f. RV infarct

(acquired/congenital) valvular abnormalities are common because the dec. in prevalence of rheumatic heart diseases has been accompanied by an increase in that of degenerative valve diseases a. acquired b. congenital

(hard/soft) plaques: -also known as STABLE plaques -SMALL lipid-pool & thick, collagen-rich core + may progress in severity and result in chronic angina pectoris a. hard b. soft

(pressure/volume) overload: - Refers to ventricular remodeling triggered by increased resistance to ejected blood (e.g., resistance to systolic ejection) → ventricle must generate a higher systolic pressure than opposing pressure → concentrically remodels (e.g., putting resisters in parallel for cardiomyocytes to become thicker) to accommodate for the high afterload → LV wall thickens → strengthens LV systolic contraction + increases LV systolic pressure to maintain adequate SV and CO -Sequelae from LV concentric hypertrophy can cause dec. coronary blood flow and inc. myocardial workload and O2 consumption → eventually these compensatory mechanisms will be exhausted, causing reduced contractility → dec. SV, CO, and EF → when it can no longer accommodate the residual ventricular blood volume in addition to the volume of blood pumped by the LA (e.g., inc. EDV), the LV will dilate → backward transmission of increased LV pressure to lungs can cause pulmonary venous congestion + reactive vasoconstriction of pulmonary vasculature, resulting in increase in pulmonary arterial resistance and pulmonary HTN a. pressure b. volume

(primary/secondary) cardiac tumors: -Difficult to diagnose and can be life threatening due to their position -Atrial myxomas account for 50%, 2nd most common is fibroelastoma, and then lipoma -25% are malignant, sarcomatous in nature → angiosarcoma, leiomyosarcoma, osteosarcoma, fibrosarcoma, and malignant fibrous histiocytoma (most common → least common) a. primary b. secondary

(primary/secondary) cardiomyopathies: -involve solely or predominantly the myocardium → relatively few and may be genetic or acquired Ex: Dilated cardiomyopathy, hypertrophic cardiomyopathy, restrictive cardiomyopathy, and arrhythmogenic RV cardiomyopathy Most are genetic diseases → inherited AD mostly with minority being AR, XLR, XLD, and mt a. primary b. secondary

What form of acute pericarditis: -thin, watery, straw or lemon color → due to RA, SLE (immune-mediated) a. serous b. fibrinous c. purulent d. hemorrhagic

What type of pericardial effusion: -Etiology/Pathogenesis: effusion of serous fluid into pericardial sac due to conditions w/ generalized edema (e.g., CHF, renal dz, nutritional/hepatic disorders) -Pathology: Watery clear or straw-colored serous fluid w/ low protein content and few cellular elements a. hydropericardium b. chylous c. serosanguineous d. hemopericardium

What type of rejection in CARDIAC transplantation: -Etiology/Pathogenesis: W/n min-hrs after blood flow is restored to allograft → extremely rapid destruction Preformed HLA/ABO Abs/Ags activate C' → destroy endothelial cells in microcirculation -Pathology: Secondary hemorrhage, thrombosis, necrosis Allograft looks hemorrhagic w minimal contractility a. hyperacute rejection b. cell-mediated acute rejection c. Ab-mediated acute rejection d. chronic rejection

What type of rejection in LUNG transplantation: -Etiology/Pathogenesis: W/n min-hrs after blood flow is restored to allograft → extremely rapid destruction Preformed HLA/ABO Abs/Ags activate C' → destroy endothelial cells in microcirculation -Diagnosis/Prognosis: Ab deposition on endothelial surface by immunofluorescence -Pathology: Gross: Lung heavy, dark, airless Micro: diffuse alveolar damage and fibrin/thrombi within small arterioles and capillaries of lung allograft -Presentation: Fulminant allograft dysfunction: severe hypoxemia, hemorrhagic pulmonary edema, diffuse radiographic infiltrates a. hyperacute rejection b. cell-mediated acute rejection c. Ab-mediated acute rejection d. chronic rejection

Which complication of an acute MI: -90% -25% develop in the 1st 24 h -Heart block of variable degree (including asystole), bradycardia, supraventricular tachyarrhythmias, ventricular premature contractions (PVCs), ventricular tachycardia, or ventricular fibrillation -Risk for serious (e.g., Vfib) is greatest in 1st hour and declines after a. arrhythmia b. cardiogenic shock c. infarct extension d. infarct expansion e. myocardial rupture f. RV infarct

Which effect of reperfusion after MI: -Reperfusion alters the morphology of cells already irreversible injured at the time of reflow → lethally injured cardiomyocytes develop extensive, intensely eosinophilic bands that span the short axis of the myocyte, composed of closely packed hypercontracted sarcomeres -Produced by exaggerated contraction of myofibrils at the instant perfusion is established → the blood that reperfuses the infarcted region exposes thee ischemic cells, which have lost control of their membranes, to a high concentration of calcium ions → hypercontraction of irreversibly injured myocardial cells a. contraction band necrosis b. hemorrhagic infarct c. no-reflow phenomenon d. myocardial stunning e. hibernating myocardium

Which histological type of myocarditis: -most common -viral in origin, characterized by myocardial patchy infiltration of T lymphocytes w/ damage to adjacent cardiomyocytes a. lymphocytic infiltrate b. giant cell c. granulomatous d. toxic e. eosinophilic

What disorder: -Etiology/Pathogenesis: Recurrent/relapsing pericarditis follow bacterial pericarditis, previous cardiac surgery, or irradiation to mediastinum -Pathology: Pericardial adhesions = plaque-like fibrous thickenings of pericardium ("soldier's plaque") or thin, delicate adhesions that obliterate the pericardial sac but have NO EFFECT on cardiac function Obliterated pericardial sac and adherence of parietal layer causes great strain on cardiac function → hypertrophy + dilation

adhesive mediastinopericarditis (chronic pericarditis)

Which pathophysiology of myocardial ischemia (which causes IHD): -1 - all 3 of the major coronary epicardial arteries can be involved -Stenosing plaques predominate within the 1st centimeters of the LAD and L. circumflex a., and along the entire length of RCA Presence of epicardial coronary artery stenosis, caused by these plaques, is the most frequent angiographic finding in any cardiac ischemic syndrome: -Minimal stenosis that causes symptomatic ischemia induced by exercise = 75% of cross-sectional area of coronary artery (= 50% diameter assessed via coronary angiography) -Resting blood flow is reduced only when 90% of cross-sectional area of coronary artery is occluded a. coronary atherosclerosis b. metabolic and functional consequences of ischemia c. development of collateral circulation d. acute thrombosis e. coronary artery spasm f. increase in myocardial O2 demand

Which pattern of rejection: -Minutes - hours after anastomosis takes place in recipients with preformed cytotoxic Abs to class I HLA antigens or anti-ABO blood group Abs → These Abs can occur as a result of previous exposure to antigens from blood transfusion or pregnancy -Only form of rejection that is exclusively mediated by ONLY a humoral mechanism -Pre-formed Abs to class I HLA antigens or ABO antigens bind to graft's endothelial cells and activate C' → endothelial injury → exposes subendothelial basement membrane proteins → activate platelets → thrombosis of microcirculatory bed → irreversible damage of transplanted organ -Tx = immediate graft removal a. hyperacute b. acute cell-mediated c. acute Ab-mediated d. chronic

Which type of graft: -grafts between members of different species → most disparity and elicit the maximal immune response (rapid rejection) a. xenograft b. autograft c. isograft d. allograft

Which type of myocarditis: -most cases, esp. viral -Diagnosis/Prognosis: transient ECG abnormalities in T wave, echo, cardiac cath, May cause complete recovery or sudden cardiac death a. asymptomatic b. fulminant c. acute coronary-like syndrome d. chronic

aortic valve (stenosis/regurgitation) is most commonly caused by calcification of a congenital bicuspid valve or normal tricuspid valve, rheumatic heart disease a. stenosis b. regurgitation

mitral valve (stenosis/regurgitation) is most commonly caused by rheumatic heart disease a. stenosis b. regurgitation

pulmonic valve (stenosis/regurgitation) is most commonly caused by congenital pulmonary stenosis (e.g., tetralogy of fallot), rheumatic heart disease, carcinoid heart disease a. stenosis b. regurgitation

tricuspid valve (stenosis/regurgitation) is most commonly caused by rheumatic heart disease a. stenosis b. regurgitation

What disorder: -occurs in unstable angina, subendocardial infarction, transmural infarction, and sudden cardiac death Due to disruption of atherosclerotic plaque in a main epicardial coronary a.: -Pts have mild-to-moderate disease before plaque disruption with the culprit lesion being a vulnerable plaque that is less than 70% in severity (vulnerable plaques = LARGE lipid pool, high % inflammatory cells, and THIN fibrous cap separating the lipid core from the vessel lumen (aka "soft" plaques)) which are more prone to acute rupture and exposure of the thrombogenic core (intracoronary thrombosis)

acute coronary snydrome

What are a set of S/Sx that are a result of abruptly decreased blood flow (ex: disruption of atherosclerotic plaque in a main epicardial coronary a.)? Hint: types → unstable angina, subendocardial infarction, transmural infarction, sudden cardiac death

acute coronary syndrome

What disorder: -Etiology/Pathogenesis: Inflammation of viscera or parietal pericardium Viruses, bacteria, autoimmune, neoplastic invasion, uremia, previous MI, chest trauma Enteroviruses = leading cause (coxsackie A and B most frequently implicated) Immune-mediated in pts w/ rheumatic fever, SLE, or scleroderma -Diagnosis/Prognosis: ECG: ST elevation in all leads but aVR and V1 (depressed); PR segment depression in all leads but aVR CXR: enlarged cardiac silhouette Complete recovery -Pathology: Serous: thin, watery, straw or lemon color → due to RA, SLE (immune-mediated) Fibrinous: thick shaggy fibrinous exudate → due to viral, acute MI, Dressler syndrome, uremia, chest radiation, rheumatic fever, SLE, chest wall trauma Purulent: thick creamy exudate, sometimes greenish hue - Pyogenic bacteria from nearby infection or seeding from blood Hemorrhagic: blood + fibrinous/suppurative exudate - TB, neoplasm -Presentation: Retrosternal pain that is better when sitting up, leaning forward but worse when breathing deeply or lying down Pericardial friction rub @ L sternal border w/ pt leaning forward - Myopericarditis: elevated CEs, myocardial dysfunction on echo

acute pericarditis

What disorder: -Genetics/RF: Ages 5-15 Peaks 25-40 F > M HLA-DR7 allele ↑ susceptibility -Etiology/Pathogenesis: Multi-system inflammatory disease resulting from an autoimmune reaction to infection with S. pyogenes (group A, beta-hemolytic strep) that affects the heart, joints, CNS, skin, and subq tissues S. pyogenes infection → molecular mimicry (HALLMARK): - M protein resembles myosin & other cardiac proteins → generates T cells + anti-cardiac Abs that recognize M protein and epitopes within the myocardium - PARF domain of M protein binds type IV collagen → more Abs, further damage - Leads to inflammation and valve scarring -Diagnosis/Prognosis: Streptococcal Ab testing during latent period Synovial fluid of migratory polyarthritis → neutrophils w/o bacteria Serum: +ASO, ADB, anti-hyaluronidase, ESR + CRP (+), peripheral neutrophilic leukocytosis Echo may see effusion (pericarditis) Brain MRI: increased signal in caudate nucleus (chorea) Jones criteria: 2 major sx or 1 major + 2 minor Jones Major Criteria: - murmurs, cardiomegaly, pericarditis, CHF, red/hot/swollen joints, chorea, erythema marginatum, and subq nodules Jones Minor Criteria: - joint pain w/o joint erythema and swelling, fever, increased ESR. CRP, and leukocytosis, and ECG changes -Pathology: damage, esp of mitral and sometimes aortic - Leaflet thickening, scarring, calcification, stenosis Chorea: cerebral vasculitis in basal ganglia Nodules: fibrinoid necrosis in center -Presentation: Latent period ~3 wks between precipitating S. progenies infection (onset of strep. Pharyngitis) and the appearance of the clinical features of ARF → after sore throat or subclinical inf. Migratory polyarthritis (knees, ankles, hips, and elbows); hot, swollen, red joints (polyarthralgia if minor) & asymmetrical Polyarthritis → only in the presence of obvious edema, erythema, and heat on the joint → of any joint subsides spontaneously within a week, but overall lasts ~4 weeks + resides w/o residual joint damage Polyarthralgia (pain in 1+ joints w/o inflammation) Myocardial involvement usually silent - Pericarditis: sharp, stabbing chest pain, friction rub Syndenham's chorea (F > M) Erythema marginatum: serpiginous rash mostly on trunk Subcutaneous nodules

acute rheumatic fever (ARF)

What disorder: -Genetics/RF: Peaks at 25-40 y/o F > M HLA-DR7 allele ↑ susceptibility -Etiology/Pathogenesis: Complication of ARF 3 phases of Aschoff body development: - Early (degen) phase: focal edema, fibrinoid necrosis - Intermediate (granulomatous): plump macs (Anitschkow cells) coalesce → Aschoff giant cells - Late (fibrous): granulomas regress, become fibrotic -Diagnosis/Prognosis: HF + death due to rheumatic myocarditis Prog: Approx. 50% w carditis recover, 50% develop organic valvular damage Pancarditis→ inflammation of entire heart -Pathology: Endocarditis: most commonly on mitral valve then aortic valve → verrucae (platelets, fibrin surrounding fibrinoid necrosis) on leaflet tips, along the line of closure on the atrial aspect of AV valves and on the ventricular aspect of the semilunar valves Myocarditis: scattered, perivascular granulomatous lesions (Aschoff Bodies/Nodules) within connective tissue of myocardial interstitium -Early (degen.) phase → focal edema and fibrinoid necrosis within connective tissue between cardiac myofibers -Intermediate (granulomatous) phase → pathognomonic granuloma formation, central foci of fibrinoid necrosis surrounded by inflammatory cells + plump macs called anti-schkow cells (anti-schkow cells → lots of cytoplasm and vesicular nuclei in which chromatin is disposed in a central, slender, wavy ribbon → "caterpillar or "owl-eye") that may coalesce to form multi-nucleated Aschoff giant cells (abundant basophilic cytoplasm + ragged borders and 1-4 nuclei) -Late (fibrous) phase → fibrinoid changes disappear, granulomas regress and become fibrotic in 3-6 mos or even longer Pericarditis: Acute fibrinous/serofibrinous exudates → both visceral and parietal surfaces of pericardium covered by fibrin -Presentation: New murmur Mitral valve most commonly + severely affected, then aortic Preserved systolic function but evidence of myocardial inflammation present microscopically in rheumatic myocarditis Severe mitral regurg. → LV dilation, CHF due to mitral regurg Pericarditis: friction rub

acute rheumatic heart disease

What is the cause for ~90% of acute MIs?

acute thrombus that obstructs an atherosclerotic coronary a.

Which complication of long-term post-transplant immunosuppression: -Chronic immunosuppression is associated with inc. risk of fracture due to osteoporosis and inc. prevalence of hyperlipemia and DM

metabolic disorders

What refers to pain of myocardial ischemia → typically produces a severe crushing or burning sensation in the substernal portion of chest and may radiate to L arm, jaw, or epigastrium & is the most common Sx of IHD? Hint: variety of forms → chronic stable, unstable, Prinzmetal angina, cardiac syndrome X (microvascular angina)

angina pectoris

What disorder: -malignant neoplasm -Genetics/RF: Sporadic, or d/t previous radiation or chronic lymphedema Median age: 67 yrs F > M -Etiology/Pathogenesis: 50% occur in skin of head and neck Radiation-induced develop 10-12 years after radiation for a malignancy -Diagnosis/Prognosis: Endothelial markers: von Willebrand factor, CD34, CD31, VEGF HHV8 (-) Most common site of metastasis (thru. blood) = lungs Prognosis is poor -Pathology: Gross: purple nodules/plaques with hemorrhagic or necrotic surface Micro: infiltrating vascular channels in dermis or soft tissue

angiosarcoma

What disorder: -Etiology/Pathogenesis: Caused by 1º disease of valve leaflets (e.g., bicuspid/unicuspid valves, rheumatic disease, infective endocarditis) or diseases of aortic root (e.g., Marfan Syndrome, aortic dissection, and syphilitic aortitis) -Pathology: Retrograde blood flow from aorta → LV eccentric hypertrophy -Presentation: BP 145-160/45-60 mmHg Increased PP, peripheral pulses bounding Diastolic murmur @ LSB LHF, angina pectoris

aortic regurgitation

What disorder: -Genetics/RF: Increases w age -50-70 = bicuspid valve 65%, tricuspid valve 35% ->70: tricuspid 60% and bicuspid 40% -Etiology/Pathogenesis: 3 causes: 1. Congenital bicuspid valve w calcifications 2. Calcification of normal valve (acquired) → 60% of cases, usually the consequence of age-associated "wear and tear" of anatomically normal aortic valves 3. Rheumatic disease Calcific aortic valve disease is a passive, age-related, degenerative disease → a form of dystrophic calcification induced by long-lasting mechanical stress, but bone morphogenic prot 2 (BMP2) implicated in calcification -Pathology: Narrowing of aortic ostium → restricts blood flow from LV to aorta Congenital Bicuspid: less efficient at distributing mechanical stress, function normally during early life, but may become calcified later in life due to abnormal closure patterns → only 2 functional cusps, usually of unequal size with the larger cusp having a midline raphe, resulting from incomplete commissural separation during development Acquired Calcific of Normal Tricuspid: due to presence of rigid crescent-shaped masses of calcium, deposited initially in aortic sinuses, which fix the cusps in a semi-closed position Rheumatic aortic valve disease (rarely isolation): commissural fusion (hallmark), diffuse fibrous thickening, scarring of cusps, and deposition of calcium → all reduce valve orifice and limit valve mobility -Presentation: Calcific: Concentric hypertrophy d/t pressure overload Sx don't appear til > 50 y/o - Systolic murmur (@ 2nd intercostal space in R upper sternal border), dizziness + syncope (earliest Sx due to dec. CO), angina on exertion, CHF (coughing, fatigue, progressive dyspnea on exertion orthopnea, and paroxysmal nocturnal dyspnea) - Rheumatic: almost always mitral valve disease as well

aortic stenosis

which are the most common acquired valvular heart diseases?

aortic stenosis and mitral regurgitation

What disorder: -Genetics/RF: Adolescence AD; PKP2 gene (plakophilin-2) - Other genes: DSG2 (desmoglein-2), DSP (desmoplakin), DSC-2 (desmocollin-2) -Etiology/Pathogenesis: Primarily affects RV Myocyte detachment disrupts cell-cell adhesion → apoptosis, regional fibrosis → scar → arrhythmia -Diagnosis/Prognosis: Transthoracic echo: 1. enlargement of RV outflow tract w/ reduced RV pump function 2. localized RV aneurysms -Pathology: Fibro-fatty replacement of RV myocardium, associated with RV dilation and dysfunction, paroxysmal ventricular arrhythmias (tachycardia), and sudden cardiac death -Presentation: RV arrhythmias: frequent ventricular premature beats to ventricular tachycardia (VT) -Can cause RV failure, sudden cardiac death Usually kids

arrhythmogenic RV cardiomyopathy

What are the risk factors for ischemic heart disease?

arterial HTN, tobacco use, DM, and elevated blood cholesterol

What disorder: -vascular malformation -Genetics/RF: Often Sx between age 20-40 y/o -Etiology/Pathogenesis: Most often in brain, spinal cord Resistance to blood flow lower → inc. BV -Pathology: Tangle of aa and vv, missing capillary connection Gross: tightly packed mass of enlarged vessels w/ spongy appearance on cross section -Presentation: Present from birth, sx show btw 20-40 y/o - Warm subq/submucosal swellings, palpable thrill, bruit "Blood steal phenomenon" from other tissues → ischemia, necrosis If large enough, abnormal strain on heart → HF

arteriovenous malformation

What is the disease primarily responsible for most cases of acute coronary syndrome?

atherosclerosis

Which pathophysiology of myocardial ischemia (which causes IHD): 1. Metabolic abnormalities: during ischemia, ischemic myocardial cells lose K+ and lactic acid; both accumulate in venous blood draining the ischemic area 2. Abnormalities in diastolic function: slowed ventricular relaxation 3. Abnormal systolic function: decline in regional contraction within several beats 4. ST segment depression: next phenomenon and classically it is a horizontal depression of at least 1 mm v. baseline 5. Angina pectoris: clinical manifestation of myocardial ischemia (usually last phenomenon) The ischemic cascade: Local ischemia → metabolic abnormalities → abnormal diastolic performance → abnormal systolic function → ECG abnormalities → chest pain (angina) -When restoring perfusion, the sequence is reversed with resolution of chest pain occurring before hemodynamic changes resolve, BUT regional contraction can remain depressed, reflecting the development of stunned myocardium a. coronary atherosclerosis b. metabolic and functional consequences of ischemia c. development of collateral circulation d. acute thrombosis e. coronary artery spasm f. increase in myocardial O2 demand

What disorder: -most common primary cardiac tumor -Genetics/RF: 30-60 y/o, mean age = 50 y/o F > M Inactivating mutation in PRKAR1A tumor suppressor gene on 17q22-24 → encodes regulatory subunit type 1-alpha of cAMP-dependent PKA (sporadic) -Etiology/Pathogenesis: BENIGN that originates from atrial septum and grows in the LA or RA (most commonly LA) → arises from the primitive multi-potential mesenchymal cells of subendocardium Most commonly attach to the atrial septum near the border of fossa ovalis -Diagnosis/Prognosis: Anemia, leukocytosis, elevated ESR/CRP, serum gamma globulins Dx: TTE and TEE (echo) Tx: surgical resection -Pathology: Gross: 1-10cm, sessile or pedunculated masses that protrude into chamber (can be round/hard/smooth OR soft/translucent/gelatinous) Micro: - arranged singly or in cords - elongated, fusiform, polygonal, or stellate → mimic the structure of undifferentiated mesenchyme of embryonic stage -embedded in abundant loose myxoid stroma, rich in ground substance -Presentation: Obstruction of Blood Flow: most common signs → can move into AV orifice during diastole/systole, mimicking mitral/tricuspid stenosis - mitral stenosis: dyspnea on exertion that may progress to orthopnea, paroxysmal nocturnal dyspnea, and pulmonary edema - tricuspid stenosis: fatigue, peripheral edema, hepatomegaly, ascites Auscultatory Signs: diastolic murmur, accentuated 1st heart sound Embolization: next most common sign → systemic embolization b/c most often in LA → TIAs, stroke, seizure, vision loss, pulmonary embolism or infarction General, constitutional (inflammatory) Sx: fever, weight loss, arthralgias, Raynaud phenomenon

atrial myxoma

Which effect of reperfusion after MI: -Reperfused infarct develops a confluent area of hemorrhage because the vasculature injured during a period of 1-2 h of ischemia becomes leaky on restoration of blood a. contraction band necrosis b. hemorrhagic infarct c. no-reflow phenomenon d. myocardial stunning e. hibernating myocardium

(hard/soft) plaques: -also known as VULNERABLE plaques -LARGE lipid pool, high % inflammatory cells, and THIN fibrous cap separating the lipid core from the vessel lumen a. hard b. soft

(pressure/volume) overload: -Occurs in response to mitral or aortic regurg, wherein a significant portion of blood ejected by LV in systole is NOT delivered to systemic circulation, but instead is returned to LV -adapts to volume overload by eccentric hypertrophy (e.g., putting resisters in SERIES) a. pressure b. volume

(primary/secondary) cardiac tumors: -Tumors w/ highest rate of heart metastasis → malignant melanoma, pleural mesothelioma, lung carcinoma, and breast carcinoma (most common → least common) a. primary b. secondary

(primary/secondary) cardiomyopathies: -have myocardial involvement as a component of a systemic or multi-organ disorder -Can be grouped into infiltrative, storage, toxic, inflammatory, endocrine, neuromuscular, nutritional, and autoimmune a. primary b. secondary

What form of acute pericarditis: -thick shaggy fibrinous exudate → due to viral, acute MI, Dressler syndrome, uremia, chest radiation, rheumatic fever, SLE, chest wall trauma a. serous b. fibrinous c. purulent d. hemorrhagic

What type of pericardial effusion: -Etiology/Pathogenesis: results from a communication of thoracic duct w/ pericardial space secondary to lymphatic obstruction by tumor or infection -Pathology: Turbid/milky fluid (high lipid content) a. hydropericardium b. chylous c. serosanguineous d. hemopericardium

What type of rejection in CARDIAC transplantation: -Etiology/Pathogenesis: peaks @ 1 mo. And declines rapidly over subsequent 5 mos → T cells target donor heart Ags (common, 70-85%) -Diagnosis/Prognosis: Endomyocardial biopsy -Pathology: Interstitial inflammatory (T cells, macs) infiltrate, cardiomyocyte necrosis -Presentation: Mostly asymp; can have low fever, myalgia, flu-like sx a. hyperacute rejection b. cell-mediated acute rejection c. Ab-mediated acute rejection d. chronic rejection

What type of rejection in LUNG transplantation: -Etiology/Pathogenesis: W/n 6 months perivascular cuffing by lymphocytic inflammatory cell infiltrates; severe cases demonstrate extension of inflammatory cells from perivascular zones into alveolar septa and prominent alveolar pneumocyte damage in the form of hyaline membranes (more common) -Diagnosis/Prognosis: CXR: perihilar opacities, edema CT: ground glass opacities, septal thickening, and pleural effusion -Pathology: clear lung fields or crackles -Presentation: Asymp; may have low fever, SOB, cough w/ or w/o sputum production a. hyperacute rejection b. cell-mediated acute rejection c. Ab-mediated acute rejection d. chronic rejection

Which complication of an acute MI: -5-10% -Localized area of myocardium w/ abnormal outward bulging and deformation during systole and diastole -From large, transmural infarcts that have undergone expansion and have been replaced by fibrous tissue -Give rise to formation of mural thrombi, arrhythmias, and HF -Do NOT rupture a. percarditis b. ventricular aneurysm c. infarct extension d. mural thrombus + embolization e. myocardial rupture f. RV infarct

Which complication of an acute MI: -6% -Dead myocardium doesn't contract when 40%+ myocardium is irreversibly damaged -More frequent in transmural pts -Characterized by dec. urine output, AMS, sustained hypotension, evidence of tissue hypoperfusion (cold extremities, oliguria), jugular venous distention, and pulmonary edema a. arrhythmia b. cardiogenic shock c. infarct extension d. infarct expansion e. myocardial rupture f. RV infarct

Which histological type of myocarditis: -rare, very aggressive, often fatal -characterized by intense inflammation, extensive areas of myocyte necrosis, and numerous multi-nucleated giant cells of macrophage origin, plasma cells, easily identified eosinophils, and occasional neutrophils a. lymphocytic infiltrate b. giant cell c. granulomatous d. toxic e. eosinophilic

Which histological type of myocarditis: -sarcoidosis and TB -in lungs, kidney, heart → extensive infiltration by activated macs leading to chronic inflammation and tissue damage a. lymphocytic infiltrate b. giant cell c. granulomatous d. toxic e. eosinophilic

Which pathophysiology of myocardial ischemia (which causes IHD): -The drop in post-stenotic pressure caused by advanced atherosclerotic plaques stimulate the development of [insert phenomenon] from other coronary artery beds → inc. thru progressive enlargement of pre-existing coronary anastomoses a. coronary atherosclerosis b. metabolic and functional consequences of ischemia c. development of collateral circulation d. acute thrombosis e. coronary artery spasm f. increase in myocardial O2 demand

Which pattern of rejection: -Both cellular and humoral components contribute to graft injury -Occurs to some degree in all pts in first 6 mos after transplantation (except between identical twins, unless immunosuppression is achieved) -MORE COMMON -Due to cytotoxic T-cell attack directed against mismatched HLA antigens in the transplanted organ -Cellular rejection characterized by patchy T lymphocytic infiltration (both CD4+ and CD8+ cells) of allograft interstitium → accumulate and cuff around veins in graft tissues and infiltrate around parenchymal cells -CD8+ T cells cause apoptosis to graft parenchymal cell via perforin/granzyme molecules leading to cell lysis → the identification of these molecules in allograft by immunohistochemistry allows for diagnosis of cell-mediated acute rejection Tx = combo therapy to which recipients must adhere for the rest of their lives -B-cell suppression or depletion (IV corticosteroids) and/or T-cell or B-cell depletion (anti-thymocyte globulin, alemtuzumab) OR blocking transcription of cytokine genes in activated T cells (cyclosporine, tacrolimus) a. hyperacute b. acute cell-mediated c. acute Ab-mediated d. chronic

Which type of graft: -grafts from one part of the body to another → do NOT elicit rejection a. xenograft b. autograft c. isograft d. allograft

Which type of myocarditis: -Presentation: acute severe HF at rest or cardio shock (BP 70/40, tachycardia of >120 bpm) -Diagnosis/Prognosis: echo: global hypokinesis Biopsy: myocarditis Tx supportive small # evolve to HF and death a. asymptomatic b. fulminant c. acute coronary-like syndrome d. chronic

aortic valve (stenosis/regurgitation) is most commonly caused by congenital bicuspid valve, infective endocarditis, trauma, rheumatic heart disease, dilation of aortic root due to Marfan Syndrome, Aortic Dissection, Ankylosing Spondylitis, and Syphilitic Aortitis a. stenosis b. regurgitation

mitral valve (stenosis/regurgitation) is most commonly caused by mitral valve prolapse, rheumatic heart disease, infective endocarditis, hypertrophic cardiomyopathy, annular calcification, dilated cardiomyopathy, ischemic heart disease a. stenosis b. regurgitation

pulmonic valve (stenosis/regurgitation) is most commonly caused by infective endocarditis, dilation of pulmonary root due to pulmonary HTN a. stenosis b. regurgitation

tricuspid valve (stenosis/regurgitation) is most commonly caused by functional due to RV dilatation, rheumatic heart disease, infective endocarditis, carcinoid heart disease a. stenosis b. regurgitation

What disorder: -reactive vascular hyperplasia -Genetics/RF: Immunocomp. → esp. HIV pts -Etiology/Pathogenesis: Bartonella henselae, Bartonella quintana B. henselae infects domestic cats → transmitted through bite or scratch B. quintana transmitted from human to human by body louse (Pediculus humanus humanus) After inoculation, organism taken up by regional lymph nodes → blood stream → endothelial cells -Diagnosis/Prognosis: Fastidious, G (-) rods Warthin-Starry stain: dark stained bacilli Tx: abx -Pathology: Induce host tissues to produce hypoxia-inducible factor-1alpha (HIF-1alpha) → drives VEGF production and vascular proliferation Histo: - nodules consist of proliferated capillaries w/ plump endothelial cells - lesions contain inflammatory cells (PMNs, leukocytoclastic debris) and numerous causal bacteria -Presentation: Crops of reddish-purple nodules on skin that vary in size from a pinhead to 10cm in diameter Nodules can ulcerate and bleed as they enlarge

bacillary angiomatosis

What disorder: -Pathology: Both sides of heart are affected -Presentation: Pulmonary edema + systemic edema present

biventricular (global) CHF

What is rejection of allograft mediated by?

both cellular (cell-mediated cytotoxicity and delayed-type hypersensitivity response) and humoral (B cell and Ab responses) mechanisms

What form of acute pericarditis: -thick creamy exudate, sometimes greenish hue - Pyogenic bacteria from nearby infection or seeding from blood a. serous b. fibrinous c. purulent d. hemorrhagic

What type of pericardial effusion: -Etiology/Pathogenesis: Chest trauma (accidental or caused by cardiopulmonary resuscitation) -Pathology: Blood + clear yellow blood serum; watery, pink, or pale red that does not clot a. hydropericardium b. chylous c. serosanguineous d. hemopericardium

What type of rejection in CARDIAC transplantation: -Etiology/Pathogenesis: days to weeks → preformed IgM and IgG target donor heart HLA Ags on endothelial cells of heart → classical C' pathway (C1q, MAC; C3a, C5a → C4d, C3d) -Diagnosis/Prognosis: C4d and C5d = markers of C' activation Deposits of IgG, IgM, IgA; C3d, C4d, C1q in capillary walls on immunohistochemistry or immunofluorescence -Pathology: Vasculitis: swollen endothelial cells, macs accum in capillaries -Presentation: Asymp; may have LVHF a. hyperacute rejection b. cell-mediated acute rejection c. Ab-mediated acute rejection d. chronic rejection

What type of rejection in LUNG transplantation: -Etiology/Pathogenesis: w/n 6 mos Abs specifically directed against dolor HLA Ags (less common) -Diagnosis/Prognosis: Transbronchial biopsy: capillaritis w/ necrosis of capillary walls and diffuse alveolar hemorrhage CT: diffuse pulmonary infiltrates + ground-glass opacities IgG and Cd4 on immunohistochemistry -Presentation: Respiratory failure a. hyperacute rejection b. cell-mediated acute rejection c. Ab-mediated acute rejection d. chronic rejection

Which complication of an acute MI: -Early in-hospital reinfarction after acute MI -Consists of recent areas of myocardial necrosis within the area at risk -May present as new necrosis that involves the lateral margins of the infarct OR as subendocardial infarct that becomes transmural -Occurs 2-10 days following acute MI, clinically manifested as recurrent CP or sudden deterioration of cardiac failure + re-elevation of plasma cardiac markers -Presence of areas of necrosis of different ages within same vascular territory a. arrhythmia b. cardiogenic shock c. infarct extension d. infarct expansion e. myocardial rupture f. RV infarct

Which effect of reperfusion after MI: -Coronary revascularization does not always lead to coronary reperfusion -Failure of blood to reperfuse the ischemic myocardium after the physical obstruction has been removed or bypassed --> Caused by irreversibly injured endothelial cells by the preceding ischemic episode swelling and filling the capillaries up to luminal obliteration AND leukocyte aggregation that plug capillaries, thus mechanically blocking flow a. contraction band necrosis b. hemorrhagic infarct c. no-reflow phenomenon d. myocardial stunning e. hibernating myocardium

Which pattern of rejection: -Both cellular and humoral components contribute to graft injury -Occurs to some degree in all pts in first 6 mos after transplantation (except between identical twins, unless immunosuppression is achieved) -Due to anti-donor IgM or IgG Abs that are formed de novo following implantation of the graft and attack the mismatched donor MHC antigens (HLA-I and HLA-II in the endothelium of capillaries through the classical pathway of C' cascade: C1q binds to IgM and IgG in a series of amplified steps → membrane attack complex (MAC) → lysis of endothelial cells & graft rejection -Results in capillaritis consisting in accumulation of PMNs in capillaries w/ or w/o fibrinoid necrosis of capillary wall -Inactive product, C4d, remains covalently bound in walls of graft capillaries → Identified by immunohistochemistry C4d C' within vascular wall is a marker of Ab-mediated endothelial destruction Tx = combo therapy to which recipients must adhere for the rest of their lives -elimination of circulating Abs (plasmapheresis), inhibition of C' and anti-HLA Abs (IV Ig), and suppression or depletion of B cells (corticosteroids, rituximab, or splenectomy) a. hyperacute b. acute cell-mediated c. acute Ab-mediated d. chronic

Which type of graft: -grafts between genetically identical individuals (e.g., monozygotic twins) → do NOT elicit rejection a. xenograft b. autograft c. isograft d. allograft

Which type of myocarditis: -Presentation: new chest pain at rest, unexplained HF (dyspnea on exertion), palpitations, fatigue, arrhythmias -Diagnosis/Prognosis: Elevated troponin, ST elevation on ECG, segmental wall motion abnormalities on echo a. asymptomatic b. fulminant c. acute coronary-like syndrome d. chronic

What disorder: -Genetics/RF: Pts w carcinoid tumors of small intestines that have metastasized to liver - Peak incidence 56-63 y/o -Etiology/Pathogenesis: Neuroendocrine tumors (aka carcinoid tumors), residing alongside the epithelium lining lumen of digestive tract and bronchi, contain secretory granules in cytoplasm that secrete 5-HT (serotonin), histamine, tachykinins, kallikrein, prostaglandins → leads to clinical Sx when overproduced Valvular and endocardial lesions thought to be caused by high concentrations of serotonin or other vasoactive amines and peptides produced by the tumor in the liver → these mediators metabolized in lung, so affects R heart almost exclusively involvement of L sided valves thought to be due to presence of PFO w/ R → L shunt or bronchial carcinoid -Diagnosis/Prognosis: Increased urinary levels of 5-HT in carcinoid syndrome CXR: normal or cardiomegaly Echo: RA, RV hypertrophy Advanced dz: survival of 11 months -Pathology: Affects endocardium of R heart and produces tricuspid regurg & pulmonary stenosis Thick, pearly white plaques on parietal endocardium of RA, RV, and tricuspid/pulmonic valves → composed of smooth m. cells, myofibroblasts, and elastic tissue lining the endocardial surface of cardiac valves superficial to normal valve tissue Tricuspid valve involved in 80-90% of cases -Presentation: Carcinoid syndrome: diarrhea, flushing, asthmatic attacks → due to hormones secreted by neuroendocrine tumors Sx of RHF (hepatomegaly, edema, ascites, fatigue, low CO) Murmurs of tricuspid regurg, pulm stenosis

carcinoid heart disease

What disorder: -Genetics/RF: A type of restrictive cardiomyopathy AL and ATTR present in ages 60-80 y/o -Etiology/Pathogenesis: Amyloidogenic peptides share a common beta-sheet conformation of their polypeptide backbone → confers the fibrillar, proteolytic resistant and insoluble characteristics of all amyloids → pathogenic effect → organ failure → death -Diagnosis/Prognosis: Grossly: heart is enlarged w/o ventricular dilation → ventricular walls are thick, firm, and rubbery -Pathology: Extracellular deposition of proteinic fibrils that fold into a beta-pleated sheet Accumulation of immunoglobulin light chains (AL amyloidosis) or transthyretin (ATTR amyloidosis), most prominent in myocardial interstitium Amyloid deposition increases stiffness of myocardium → diminished compliance + diastolic dysfunction

cardiac amyloidosis

What disorder: -Genetics/RF: Physiological: normal (healthy) Pathological: HTN, aortic stenosis, aortic regurgitation -Etiology/Pathogenesis: Physiological: exercise → mild increase in cardiac mass w/ normal cardiac morphology and normal and/or enhanced cardiac function → IGF-1 and TGF-B activate RTKs → activate PI3K and AKT protein kinases → activate adaptive genes → physiological hypertrophy Pathological: adaptive response to inc. hemodynamic load (either pressure/volume overload) caused by MI, HTN, valvular disease, etc.→ Ang II, ET-1 → activate GPCR → MAPK cascade → activate maladaptive genes (e.g., JNK) - switch to fetal gene expression profile (beta-MHC in lieu of adult pre-dominant alpha-MHC, alpha-SKA, and natriuretic peptide genes) → enhanced protein synthesis, increased cardiomyocyte size, ventricular hypertrophy, and greater organization of sarcomeres → maladaptive changes in cardiac function, disarray of arrangement of cardiomyocytes, cardiomyocyte apoptosis (rare, when occurs the lost cardiomyocytes are replaced by fibrous tissue), and imbalance in synthesis and degradation of extracellular matrix collagen → ventricular decompensation → chamber dilation → CHF -Diagnosis/Prognosis: Pathologic: Often progresses to HF Increased levels of natriuretic peptides (NP) → ANP (atria), BNP (ventricles), CNP -Pathology: Physiologic: increase in capillary density Pathologic: excessive increase in heart mass accompanied by myocardial dysfunction and fibrosis no change in capillary density, interstitial fibrosis - Pressure overload (hypertrophy): sarcomeres parallel to cardiomyocytes, cells get thicker → concentric hypertrophy - Vol overload (dilation): new sarcomeres in series w existing sarcomeres, cells get longer → eccentric hypertrophy -Presentation: Physiologic: enlarged heart, decreased HR and BP - beneficial Pathologic: concentric or eccentric hypertrophy

cardiac hypertrophy

What disorder: -Most common cardiac tumor in kids -Genetics/RF: rare Assoc w tuberous sclerosis (AD) -Etiology/Pathogenesis: Usually in LV or interventricular septum -Diagnosis/Prognosis: In utero US or 1st year of life Most regress spontaneously, some may require surgery -Pathology: Gross: Firm, white, well-circ myocardial nodules Micro: "spider-like" appearance d/t long eos strands of cytoplasm stretching from membrane to centrally placed nucleus Glycogen-filled -Presentation: Variable → depends on location, sie, and # of tumors If chamber or valve obstruction, arrhythmias, or failure from extensive myocardial involvement: ↓CO, RAP and vena cava P, hydrops fetalis, fetal demise

cardiac rhabdomyoma

What disorder: -Genetics/RF: 70% of pts are women Ages 40-50 -Diagnosis/Prognosis: Recommended dx test: intracoronary acetylcholine administration during coronary angiography angina w/o epicardial coronary a. spasm ECG: ST segment depression Echo: wall defects Normal metabolites Coronary arteriography: no obstructive coronary a. disease/epicardial coronary a. spasm (NORMAL) Exercise stress myocardial perfusion scintigraphy: transient regional myocardial perfusion defects -Pathology: Coronary endothelial cell dysfunction in the microcirculatory bed causing microvascular dysfunction that is scattered throughout the myocardium: microvascular dysfunction = abnormalities in vasomotor or metabolic regulators of small coronary arterioles → may be caused by hyperactivity of coronary microvasculature/ reduced vasodilator capacity → inappropriate constriction &/or impaired dilation → ischemia in small myocardial regions surrounded by regions with normal microvascular function -Presentation: Angina-like chest pain w/o limited flow stenosis on coronary angiography and exclusion of other causes of CP Typical angina-like CP (50%) Atypical CP (lasting longer and NO RESPONSE TO NITROGLYCERIN, can even be WORSE after SL nitroglycerin)

cardiac syndrome X or microvascular angina (MVA)

What disorder: -a type of pericardial effusion -Etiology/Pathogenesis: ↑ Pericardial P from rapid accumulation of pericardial fluid → restricts filling of heart by compressing all cardiac chambers → total venous return falls → CO and BP dec. -Diagnosis/Prognosis: CXR → rounded, flask-like appearance of cardiac silhouette Acute is most always fatal unless pressure relieved by removing pericardial fluid via needle pericardiocentesis or surgery -Pathology: accumulation of pericardial fluid -Presentation: ranges from minimally symptomatic → abrupt cardiovascular collapse and death, paradoxical pulse (abnormally large decline in systemic arterial pressure during inspiration w/ normal fall in BP being <10 mmHg) → low BP, dilated neck veins, muffled heart sounds, tachypnea, diaphoresis, cool extremities, and peripheral cyanosis

cardiac tamponade

What is indicated for pts w/ end-stage CHF (e.g., dilated cardiomyopathy, chronic IHD, valvular heart disease, or CHD) w/ EF <20% & prognosis <1 year w/o transplant?

cardiac transplant

What are diseases of cardiomyocytes resulting in inappropriate ventricular hypertrophy or dilation?

cardiomyopathies

What disorder: -Genetics/RF: Mean age <20, familial → inactivating germline mutations in PRKAR1A gene AD -Diagnosis/Prognosis: More aggressive myxoma; recur after surgical resection -Presentation: Cardiac myxoma, skin myxoma, spotty skin pigmentation, endocrine tumors

carney complex

What disorder: -vascular malformation -venous MALFORMATION -Genetics/RF: Most are sporadic, familial forms are rare -Etiology/Pathogenesis: Occur in skin, liver, brain, skeletal mm., soft tissues -Pathology: Large, ectatic, vascular channels arranged in diffuse pattern -Presentation: Soft, grouped, non-pulsating blue nodules without warmth that enlarge and thicken w age Can have thrombophlebitis

cavernous hemangioma

What disorder: -Etiology/Pathogenesis: Progressive CHF due to ischemic myocardial damage (e.g., accumulation of hibernating and scarred myocardium) HF due to several factors: - irreversible loss of myocardium (previous infarcts) & replacement with scar tissue - hypoperfusion of remaining myocardium → chronic ventricular contractile dysfunction (hibernating myocardium) - severe mitral regurg due to papillary m. scarring - organic or functional loss of 50% or more of LV myocardium -Pathology: Heart globally enlarged similar to a heart w/ dilated cardiomyopathy Transmural or subendocardial scars present, representing old Mis Non-infarcted regions show significant hypertrophy combined w/ inc. interstitial fibrosis Subendocardial "hibernating" myocardium reveals diffuse vaculozation -Presentation: CHF pre-dominates clinical Sx and is more incapacitating than CP

chronic ischemic heart disease

What disorder: -Etiology/Pathogenesis: All organ manifestations resolve completely except cardiac vascular damage → RHD is a chronic, progressive condition that causes cardiac disability and death many years after initial event Several recurrent episodes of ARF - May take 30-40 yrs to develop Most commonly affects mitral and aortic valves (M > A) -Diagnosis/Prognosis: Mitral stenosis in adults secondary to RHD in 99% of cases -Pathology: Valvular fibrosis → stenosis and/or insufficiency Valve neovascularization, diffuse scarring, thickening, and calcification Healing of verrucous lesions along lines of closure → formation of fibrous adhesions between leaflets, esp. at commissures (commissural fusion) Thick, short chordae = "mouth of catfish" Diffuse scarring of aortic valve w/ commissural fusion and thickening of leaflets → valve stenosis and valve regurg Mitral stenosis → LA dilation (may harbor thrombi), acute pulmonary edema → pulmonary HTN and RVH Irreg thickening of LA endocardium in mitral regurg → MacCallum patch/plaque (rough, patch-like thickening of parietal atrial endocardium) caused by regurgitant jets of blood flow due to mitral regurg. -Presentation: Asymptomatic for years - decades after initial episode of rheumatic fever (as long as 30-40 yrs between acute attacks and evolution of severe valve dysfunction) Mitral regurg or stenosis Aortic regurg or stenosis

chronic rheumatic heart disease (RHD)

What disorder: -Etiology/Pathogenesis: Excessive O2 demand caused by increased HR, LV wall stress, contractility during exertion -Diagnosis/Prognosis: Relieved in 5-15 minutes of rest or w/ nitroglycerin (-) troponins ECG: ST-segment depression -Pathology: Most commonly caused by obstruction of at least 1 large epicardial coronary a. by atherosclerotic plaque that reduces the cross-sectional area of the coronary artery by more than 70% Atherosclerotic plaque is characterized by a small lipid pool & thick fibrous cap ("hard" plaque) -Presentation: Reproducible pattern of anginal symptoms Reoccur during exertion, exposure to cold, eating, or emotional stress → exercise induced retrosternal CP Pain = constricting, squeezing or choking, tends to radiate BL into arms (L > R), neck, and lower jaw

chronic stable angina pectoris

Cardiac findings at autopsy of ________________ include: -Increased weight, enlargement of external dimensions Concentric hypertrophy due to arterial HTN undergo dilation when they fail, esp. during the decompensated stage --> It is difficult, from morphological exam, to distinguish the eccentric hypertrophy characteristic of aortic regurg from the decompensated stage of CHF → dilation of ventricular chambers + thinning of ventricular walls accentuate with onset of decompensation, but the extent of these changes is HIGHLY variable -S/Sx resulting from inability of heart to pump blood forward at a sufficient rate to meet metabolic demands of the body (forward failure) → renal under-perfusion → decreased sodium and water EXCRETION and increased plasma volume (Kidneys (macula densa) are sensing the decreased blood flow (under-perfusion), thus they excrete renin → angiotensin I → angiotensin II → ADH + aldosterone → sodium retention (aldosterone) + water retention (ADH putting in aquaporins) → reduces excretion of sodium and water → increases plasma volume) -Pump failure produces an increase in pulmonary or systemic venous pressure w/ resultant transudation of fluid from intravascular compartment → interstitial space and edema formation (backward failure) → pulmonary edema, peripheral edema (or both) -Failure of the LV produces excessive strain on RV, terminating in biventricular (or global) HF

congestive heart failure

What is a clinical syndrome in which the heart is unable to pump enough blood to meet the metabolic needs of the body because of pathological changes in the myocardium?

congestive heart failure

What disorder: -Etiology/Pathogenesis: Recurrent/relapsing pericarditis 9% of pts with acute pericarditis develop this → due to suppurative pericarditis, previous cardiac surgery, and irradiation to mediastinum -Diagnosis/Prognosis: Tx = surgical resection of shell of constricting fibrous tissue (pericardiectomy) -Pathology: Pericardial adhesions = plaque-like fibrous thickenings of pericardium ("soldier's plaque") or thin, delicate adhesions that obliterate the pericardial sac but have NO EFFECT on cardiac function heart encased in a dense, fibrous, or fibrocalcific scar that limits diastolic expansion and CO -Presentation: distant/muffled heart sounds, RSHF, elevated jugular venous pressure, hepatomegaly with marked ascites, and peripheral edema

constrictive pericarditis (chronic pericarditis)

What is the etiology of IHD in more than 80% of cases?

coronary artery obstruction by atherosclerotic plaques causing myocardial ischemia

What form of acute pericarditis: -blood + fibrinous/suppurative exudate - TB, neoplasm a. serous b. fibrinous c. purulent d. hemorrhagic

What type of pericardial effusion: -Etiology/Pathogenesis: wounds, Ventricular wall rupture (from MI), and aortic aneurysm rupture -Pathology: Frank blood in pericardium, usually clots a. hydropericardium b. chylous c. serosanguineous d. hemopericardium

What type of rejection in CARDIAC transplantation: -Etiology/Pathogenesis: Develops in the form of cardiac allograft vasculopathy (CAV), an accelerated form of coronary atherosclerosis affecting both epicardial and intramyocardial coronary aa. and vv. Ab/T cells attack donor HLA Ags and vascular endothelial cell Ags → endothelial cell damage -Diagnosis/Prognosis: Gold standard: coronary angiography NO inflammation Atherosclerosis lacks cholesterol and calcification -Pathology: CAV = progressive obliteration of coronary aa. by uniform, concentrical intimal thickening consisting of smooth m., macs, T-lymph, and abundant extracellular matrix of collagen and proteoglycans Acute ischemia due to narrowed coronary aa.: coagulation necrosis, granulation tissue, healed scar or chronic ischemia in form of myocytolysis -Presentation: Sx of CHF, ventricular arrhythmias, sudden cardiac death a. hyperacute rejection b. cell-mediated acute rejection c. Ab-mediated acute rejection d. chronic rejection

What type of rejection in LUNG transplantation: -Etiology/Pathogenesis: Bronchiolitis Obliterans Syndrome (BOS) → lymphocytic infiltration of terminal bronchioles and respiratory bronchioles + deposition of collagen in submucosa → fibrotic obliteration of airway lumen → lung disease Apoptosis of airway epithelium due to production of Abs against donor HLA Ags on bronchial epithelium -Diagnosis/Prognosis: 50% develop BOS by 5 years and 75% by 10 years BOS progressive that responds poorly to Tx Median survival after BOS onset: 3-4 yrs -Pathology: Chronic scarring process that obliterates bronchiolar lumen is an exaggerated repair response in this immunologic injury -Presentation: Progressive fall in forced expiratory volume in one second (FEV1) a. hyperacute rejection b. cell-mediated acute rejection c. Ab-mediated acute rejection d. chronic rejection

Which complication of an acute MI: -20% -Combination of attenuated myocardial contractility (causing stasis), chamber dilation, and endocardial damage (causing a thrombogenic surface) can foster mural thrombosis → may lead to systemic thromboembolism (brain, kidney, eye, spleen, bowel, legs) a. percarditis b. ventricular aneurysm c. infarct extension d. mural thrombus + embolization e. myocardial rupture f. RV infarct

Which complication of an acute MI: -40% of anterior transmural MI -Thinning of transmurally infarcted area caused by slippage between muscle bundles reducing the number of myocytes across the infarct wall and tissue loss within the necrotic zone -Usually reaches peak extent within 7-14 days a. arrhythmia b. cardiogenic shock c. infarct extension d. infarct expansion e. myocardial rupture f. RV infarct

Which effect of reperfusion after MI: -Occurs when a brief period of ischemia is relieved by reperfusion, causing a transient post-ischemic contractile dysfunction → may takes days - weeks to recover a. contraction band necrosis b. hemorrhagic infarct c. no-reflow phenomenon d. myocardial stunning e. hibernating myocardium

Which histological type of myocarditis: -characterized by necrosis of cardiomyocytes → inflammation seen as extensive intracellular TG accumulation, cardiomyocyte coagulative necrosis and myocytolysis, and a mononuclear inflammatory cell response that is mild compared to wide extent of myocyte damage a. lymphocytic infiltrate b. giant cell c. granulomatous d. toxic e. eosinophilic

What disorder: -vascular malformation -LYMPHATIC MALFORMATION -Genetics/RF: Most by age 2 -Etiology/Pathogenesis: Head, neck, axilla -Diagnosis/Prognosis: benign vesicles greatly dilated endothelial-lined lymph channels that involve upper part of dermis -Pathology: Large, soft masses of dilated, fluid-filled lymph channels that are sequestered and fail to communicate w/ rest of lymph or venous system Small, firm, clear vesicles; 2-4 mm -Presentation: Axillary folds, shoulders, neck, prox. limbs, buccal mucosa

lymphangioma circumscriptum

Which pathophysiology of myocardial ischemia (which causes IHD): -3 components of ischemic heart disease = acute coronary syndromes → unstable angina, MI, and sudden cardiac death o Frequently initiated by an abrupt conversion of a stable atherosclerotic plaque to an unstable and potentially life-threatening atherosclerotic lesion with superimposed thrombus → the coronary culprit lesion of acute coronary syndromes = rupture-prone (e.g., vulnerable) plaque complicated with thrombosis -Vulnerable plaque = LARGE lipid-rich core in ventral portion of an eccentrically thickened intima combined with THIN fibrous cap -Superimposed intracoronary thrombi composed of platelets, fibrin, and RBCs a. coronary atherosclerosis b. metabolic and functional consequences of ischemia c. development of collateral circulation d. acute thrombosis e. coronary artery spasm f. increase in myocardial O2 demand

Which pattern of rejection: -Both cellular and humoral components -Months to years after organ transplantation -Characterized by obliteration of hollow structures (coronary a. in transplanted hearts, bronchioles in transplanted lungs, bile ducts in transplanted livers) → fibrosis and organ atrophy -Incidence increases w/ time after transplantation: By 5 years → 80% for lung, 30-40% for heart + kidney, 5% of liver -Current immunosuppressive protocols have significantly decreased graft loss to acute rejection, but have had NO EFFECT ON GRAFT LOSS TO CHRONIC rejection -Etiology not fully known → appears to be mediated by de novo production of anti-donor HLA Abs after transplant combined with a cell-mediated response against mismatched donor HLA -Results in progressive fibrosus to persistent immunological tissue injury of epithelial cell-lined conduits or endothelial cell-lined blood vessels -IRREVERSIBLE AND POORLY AMENABLE TO TX -only Tx = retransplant a. hyperacute b. acute cell-mediated c. acute Ab-mediated d. chronic

Which type of graft: -grafts between members of same species that differ genetically → most common, degree of rejection depends on degree of similarity or histocompatibility between the donor and recipient a. xenograft b. autograft c. isograft d. allograft

Which type of myocarditis: -Presentation: Dilated CM → insidious onset of Sx compatible with moderate ventricular dysfunction → fatigue, dyspnea, ↑ JVD P, LE edema, rales, S3/S4 gallops, mitral/tricuspid regurg murmurs -Diagnosis/Prognosis: CXR = cardiomegaly Echo: LV dilation, reduced EF a. asymptomatic b. fulminant c. acute coronary-like syndrome d. chronic

What disorder: -Increased resistance to filling + increased filling pressures -Stiff heart that does not relax normally after contracting → impairs ability to fill with blood during diastole -Heart contracts normally → able to pump a normal proportion of blood out of ventricles, but the total amount pumped w/ each contraction may be less -LV cannot expand normally → increase in filling pressure will push back into pulmonary circulation → rapid onset pulmonary edema Reduction in ability of LV to expand normally may stem from increased thickness of LV wall (arterial HTN, hypertrophic cardiomyopathy), myocardial fibrosis (IHD), infiltrative disorders with restrictive cardiomyopathies (e.g., cardiac amyloidosis), and restrictive pericarditis

diastolic CHF

What disorder: -most common cardiomyopathy -primary cardiomyopathy -Genetics/RF: Mean age at dx: 50-59 yrs M > F AD, many genes → mostly code cytoskeletal, sarcomeric, nuclear, or surface membrane proteins with most common being TTN (titin → giant muscle protein associated with thick filaments in cardiac and skeletal muscles, connects the Z line to M line) LMNA (lamin A and C) MYH7 (β-myosin heavy chain) -Etiology/Pathogenesis: Primary: Idiopathic - TTN encodes titin = important for passive tension, systolic function Secondary: - Toxins: alcohol, cocaine, meds (esp. chemotherapy drugs), and radiation - Drugs: anthracycline-induced cardiomyopathy has (chemotherapy agent) and trastuzumab (monoclonal Ab against HER2/neu R) - Childbirth: peripartum cardiomyopathy - development of HF in last month of pregnancy or 1st 5 mos post partum - Fe overload: due to hereditary or secondary hemochromatosis - Viral myocarditis: inflammation induced myocyte loss and interstitial fibrosis through direct cytotoxic effect of intracellular virus - Catecholamine excess: causes microvascular spasm or multifocal myocardial contraction band necrosis - Takotsubo cardiomyopathy (TTC): characterized by acute onset of transient akinesia of apical and mid portions of LV, w/o artery stenosis. - Cardiomyopathy in DMD and BMD: w/o dystrophin the heart is mechanically weak → cardiomyocyte contraction causes membrane damage → cascade of inc. Ca2+ influx → eventual cell death -Diagnosis/Prognosis: Cardiomegaly → 4-chamber dilatation that is greater in the V Mortality: 20-30% at 1 yr, 50% at 5 yrs TTC: elevated cardiac enzymes -Pathology: Dec. contractility, systolic dysfunction, dec. ejection fraction, and diminished CO Contractile (systolic) dysfunction which is manifested with weakness of ventricular contraction, dec. LV ejection fraction to <40% and an enlarged ventricular chamber in the absence of abnormal loading conditions and IHD LV dilatation with normal or dec. wall thickness, LA enlargement Impaired contraction and systolic dysfunction of 1 or both ventricles associated with hypertrophy and dilation of ventricular chambers Pale, flabby myocardium, small subendocardial scars, adherent mural thrombi, marked variation in cardiomyocyte size - TCC: contraction band necrosis caused by catecholamine toxicity -Presentation: Asymptomatic for several years → presentation usually occurs late in disease with CHF (congestion and/or reduced CO), arrhythmias (Afib, PVCs, Vtach, varying degrees of AV block, or LBBB), and/or thromboembolic disease (secondary to LV mural thrombus) - TCC: chest pain, ST-T abnormalities Often preceded by emotional or physical stress

dilated cardiomyopathy

Which complication of an acute MI: -1% Laceration or tearing of ventricular wall → includes free-wall rupture, ventricular septal rupture, and papillary m. rupture · Ventricular septal rupture manifests as CHF or cardiogenic shock · Papillary m. rupture is manifested with severe mitral regurg and acute pulmonary edema -Occurs within 3-5 days when wall is weakest because of a combination of factors (coagulative necrosis, intense neutrophil infiltration and collagen matrix degradation) -Leads to hemopericardium and fatal cardiac tamponade a. arrhythmia b. cardiogenic shock c. infarct extension d. infarct expansion e. myocardial rupture f. RV infarct

Which effect of reperfusion after MI: -Chronic low blood flow due to severe coronary stenosis results in metabolic adaptations allowing the myocardial cells to survive, but these cells do not contract at rest and exhibit vacuolar degeneration (liquefactive myocytolysis) → once revascularized, function will eventually return (may be weeks - months) a. contraction band necrosis b. hemorrhagic infarct c. no-reflow phenomenon d. myocardial stunning e. hibernating myocardium

Which histological type of myocarditis: -characterized by infiltration of lymphocytes, macs, and eosinophils, due to hypersensitivity rxn to pharm agents + assoc. w/ peripheral eosinophilia a. lymphocytic infiltrate b. giant cell c. granulomatous d. toxic e. eosinophilic

Which pathophysiology of myocardial ischemia (which causes IHD): -In pts with Prinzmetal angina, myocardial ischemia is caused by occlusive epicardial [insert phenomenon] -can develop at site of subcritical or critical stenosis, as well as in angiographically normal coronary aa. -due to a hyperreactivity of local smooth m. to a wide variety of humoral constrictor stimuli (e.g., ergonovine, histamine, serotonin, ACh, dopamine) a. coronary atherosclerosis b. metabolic and functional consequences of ischemia c. development of collateral circulation d. acute thrombosis e. coronary artery spasm f. increase in myocardial O2 demand

What disorder: -Genetics/RF: A type of restrictive cardiomyopathy Equatorial Africa -Etiology/Pathogenesis: Myocardial injury by cardiotoxic components of eosinophil granules (high blood eosinophilia triggered by parasitic infestations) → intense eosinophilic infiltration of inner myocardial layers (usually both V) → fibrotic shell → decreased compliance and diastolic dysfunction

endomyocardial fibrosis

What disorder: -intermediate grade neoplasm -Etiology/Pathogenesis: Soft tissues of extremities, lungs, bone, skin, liver -Diagnosis/Prognosis: Endothelial markers: CD31, ERG, CD34 -Pathology: Gross: intravascular mass resembling an organized thrombus Micro: cords of large endothelial cells w/ abundant eosinophilic cytoplasm and round vesicular nuclei embedded in myxoid stroma -Presentation: Originates from a large vein causing lumen obstruction, edema, and thrombophlebitis of involved area

epithelioid hemangioendothelioma

Which complication of an acute MI: -very rare -Common complication of occlusion of RCA → usually lead to infarct of the posterior LV wall and posterior ventricular septum -When R ventricular myocardium affected by RCA occlusion, RSHF develops → Sx = peripheral edema, jugular v. distention, hypotension a. percarditis b. ventricular aneurysm c. infarct extension d. mural thrombus + embolization e. myocardial rupture f. RV infarct

Which pathophysiology of myocardial ischemia (which causes IHD): -Myocardial ischemia can occur if [insert phenomenon]→ common in severe LVH due to arterial HTN or aortic stenosis -Myocardial ischemia can occur if there is a reduction in oxygen-carrying capacity of RBCs (severe anemia) or in the presence of carboxyhemoglobin -2+ causes of ischemia can coexist in a pt → inc. in oxygen demand due to LVH secondary to HTN, a reduction in oxygen supply secondary to coronary atherosclerosis, and anemia -Abnormal constriction or failure of normal dilation of coronary resistance vessels can also cause ischemia, when it causes angina → microvascular angina a. coronary atherosclerosis b. metabolic and functional consequences of ischemia c. development of collateral circulation d. acute thrombosis e. coronary artery spasm f. increase in myocardial O2 demand

T/F: if there is a cardiac tumor present, it is most likely to be benign (primary)

false

What is a systolic retrograde flow from the LV into the LA due to distortion of the LV geometry & occurs when the LA or LV dilates, causing the mitral valve annulus to also dilate, thus preventing leaflets from closing properly?

functional mitral valve regurgitation

What disorder: -benign neoplasm -Genetics/RF: Ages 20-50 -Etiology/Pathogenesis: Arise from modified SM cells of glomus bodies (AV shunts) in fingertips, involved in temp. regulation -Diagnosis/Prognosis: ɑ-smooth muscle actin present 10% recur after surg. -Pathology: Histo: branching capillary-sized vessel lined by endothelial cells surrounded by collars of uniform glomus cells in a hyalinized or myxoid stroma Glomus cells = uniform, large round cells w/ epithelioid appearance & centrally placed round/ovoid nucleus -Presentation: Solitary, small bluish nodule < 1cm usually located in subungual areas of fingers or toes Triad: tenderness, severe pain, sensitivity to cold

glomus tumor (glomangioma)

What disorder: -vascular ectasia -"Osler-Weber-Rendu" -Genetics/RF: AD LoF mutation in ENG gene encoding endoglin protein on chromosome 9q34.11 -Presentation: Telangiectasias on skin and mucous membranes → severe recurrent epistaxis, GI bleeds, anemia

hereditary hemorrhagic telangiectasia (HHT)

What disorder: -Etiology/Pathogenesis: Normally functioning heart cannot keep up w/ unusual high demand for blood to 1 or more organs in body Causes = hyperthyroidism, anemia, congenital AV fistulas, paget disease, sepsis -Pathology: Elevated CO, low systemic vascular resistance (due to peripheral vasodilation) -Presentation: Lower than normal BP, warm extremities

high-output CHF

What disorder: -primary cardiomyopathy -Genetics/RF: Bimodal: Young (teens-20s) Older (50s-60s) Age-related penetrance M > F AD; MYH7 (β-myosin heavy chain) and MYBPC3 (cardiac myosin binding protein-C) -Etiology/Pathogenesis: Unexplained LV hypertrophy that carries a high incidence of sudden death Concomitant anterior displacement of papillary mm. and mitral leaflets. The mitral valve itself is normal, but distorted. During systolic contraction, there is paradoxical motion of anterior mitral valve leaflet towards the ventricular septum and mitral leaflet-septal contact. The "Systolic anterior motion" of the anterior leaflet of mitral valve is associated with failure of coaptation of anterior and posterior mitral valve leaflets & onset of mitral regurg → over time, endocardial fibrosis occurs over the septum immediately subjacent to aortic valve, leading to formation of subaortic mitral impact lesion -Diagnosis/Prognosis: Autopsy of asymmetrical: septal:free wall thickness >1.3 4 Keys for Dx microscopically: 1. Cardiomyocyte disarray → loss of normal parallel alignment of cardiomyocytes in which adjacent hypertrophied cardiomyocytes are aligned perpendicularly or obliquely to each other and around central cores of collagen = HALLMARK 2. Cardiomyocyte hypertrophy 3. Myocardial fibrosis → inc. in interstitial connective tissue, may produce grossly visible scars if extensive enough 4. Small vessel disease → severe intimal and medial smooth m. cell hyperplasia w/ narrowing of the lumen Older have better prognosis -Pathology: Diminished LV compliance, diastolic function, markedly inc. LV wall thickness, vigorous systolic function, and often asymmetric septal hypertrophy Inappropriate and exaggerated LV hypertrophy w/o apparent cause → IV septum typically more prominently involved than LV free wall, but concentric hypertrophy can occur Enhanced contractile function and diastolic dysfunction characterized by impaired diastolic relaxation with impaired diastolic filling Massive cardiac hypertrophy that can be asymmetrical or symmetrical: - Asymmetric: exaggerated thickening of basal anterior region of ventricular septum → on longitudinal sectioning, the normally round-to-ovoid LV cavity may be compressed into a "banana-like" configuration Both asymmetric + symmetric: small punctate white scars in V -Presentation: Most are asymptomatic Young: syncope and sudden death Older: SOB & angina ↑ pulm venous pressure, exertional dyspnea → orthopnea, paroxysmal nocturnal dyspnea Hypertrophied septum → sucks in anterior leaflet of mitral → LV outflow obstruction → heart failure Vent. arrhythmias, chest pain, syncope, sudden death

hypertrophic cardiomyopathy

What disorder: -benign neoplasm -most common tumor of infancy -Genetics/RF: infants -Etiology/Pathogenesis: Rapid proliferative phase first 6-10 mo, then slow involution Venous malformation Full etiology not fully known → might represent placental endothelial cells originating from chorionic villi that embolize and reach fetal skin through R → L shunts characteristic of normal fetal circulation -Diagnosis/Prognosis: GLUT-1 = diagnostic marker Spontaneous involution -Pathology: Histo: proliferation of closely packed capillaries lined by flattened endothelium and filled with blood - Early → highly cellular w/ plump endothelial cells aligned to vascular spaces with small, inconspicuous lumina - Mature → endothelium flattens, lumina enlarge and become obvious -Presentation: Raised & firm bright red papule, nodule, or plaque

infantile hemangioma

Which complication of long-term post-transplant immunosuppression: -Most pts who have had a transplant develop infection (88%) → bacterial (pseudomonas), viral (CMV, HSV, VZV), or fungal (aspergillus)

infection

What disorder: -Genetics/RF: Mean age 61 yrs More common in males Native-Valve risk factors: cardiac factors (pre-existing valvular or CHD, mitral valve prolapse—most common, degenerative calcific stenosis of aortic valve and mitral valve annular calcification, rheumatic heart disease), underlying conditions (IV drug use, indwelling IV cath, or immuno-suppression), or recent dental/surgical procedure IDU-endocarditis: Avg age: 31; mostly males, young, 7X higher risk compared to pts w/ pre-existing cardiac disease -Etiology/Pathogenesis: invasion of heart valves or mural endocardium by a microorganism (usually bacteria) → formation of vegetations composed of thrombotic debris and bacteria, often associated w/ destruction of underlying cardiac tissues Acute: valvular infection produces rapidly necrotizing, ulcerative, and destructive lesions on valves w/ most common cause being S. aureus Subacute: often due to less virulent organisms (alpha-hemolytic Strep → viridians strep, coagulase neg. staph) → less destructive and low propensity to hematogenously seed extracardiac sites + affects ABNORMAL valves Infections can occur on heart valve prosthesis, or on reconstructed native heart valves, or on patches used to bypass or obliterate cardiac defects → prosthetic valve endocarditis (PVE) and native valve endocarditis (NVE) Majority are L sided (mitral, aortic) R sided in IV drug use, pacemakers NBTE thought to be the 1st histopathological stage in pathogenesis of IE → damaged or denuded endothelium is a potent inducer of thrombogenesis and provides a site where a sterile NBTE fibrin-platelet vegetation develops, induced by venturi effect, turbulent flow, high-velocity jet effect, and direct trauma → Bacteremia converts NBTE → IE, bacteria stick to veg and proliferate, form biofilms → immune response (Osler nodes, Roth spots, glomerulonephritis from deposition of circulating immune complexes) Early onset: w/n 60 days post-op; S. aureus most common (then coag neg staph) 2-12 mo. post-op: mostly coag - staph Late onset: >12 mo. post-op; usually streptococci then S. aureus, then coag neg staph IDU-endocarditis: bacteremia (mostly S. aureus) → damage to R valves from injected particulate matter (e.g., talc) Infective Endarteritis: variant of IE characterized by infection of segments of large aa. -Diagnosis/Prognosis: Blood cultures (10% neg due to HACEK), serologic tests, PCR Labs non-specific → leukocytosis w/ neutrophilia, elevated ESR, CRP, normochromic-normocytic anemia, (+) RF Transthoracic or transesophageal echo 3 sets of blood cultures Modified Duke criteria Acute: usually requires surgery, causes metastatic infection in distant viscera, death within days - weeks despite Tx -Pathology: Vegetation = characteristic lesion → usually develops on heart valve but occasionally appears on mural endocardium Mainly L sided valves, esp. Mitral, then aortic, then tricuspid, then pulmonary R-sided valves involved in IV drug abuses → tricuspid (70%) Mural endocarditis = when occurs in mural endocardium Hallmark: infectious thrombotic vegetation Micro: Platelet/fibrin thrombi containing bacteria and PMNs Mycotic aneurysm: localized dilation of a., common w subacute → most common in cerebral aa. IDU-endocarditis: tricuspid valve most common, then mitral and aortic valves -Presentation: Heart murmurs, CHF, intracardiac abscess Emboli to CNS, spleen, kidney, lungs, etc Petechiae, splinter hemorrhages, osler nodes (subacute) → painful, red, raised lesions in pulp spaces of terminal phalanges Janeway lesions (acute) → hemorrhagic macules on feet and palms from dermal microabscesses due to septic emboli, stroke (ischemic or hemorrhagic), glomerulonephritis, arthralgias/arthritis, splenomegaly roth spots → retinal hemorrhages w/ white centers (subacute) Acute: rapid onset (hours - days) high grade fevers, night sweats, chills → fulminant course that evolves over days - weeks - complications = CHF due to valve destruction or intramyocardial suppuration, and arterial embolization Subacute: mild-moderate illness w/ subtle constitutional Sx (5 wks) → progresses slowly and is frequently not diagnosed until it has been present for mos → osler + roth spots IDU-endocarditis: acute course, pneumonia or septic pulmonary emboli, RSHF

infective endocarditis

The etiology of __________ (in more than 80% of cases) is due to coronary artery obstruction by atherosclerotic plaques, resulting in myocardial ischemia that cause a regional reduction in myocardial blood flow and inadequate perfusion of the myocardium supplied by the involved coronary artery & can be exacerbated with increased cardiac energy demand (e.g., myocardial hypertrophy or inc. HR, hypoxemia (anemia), or systemic hypotension (shock))?

ischemic heart disease

What disorder: -vascular malformation -LYMPHATIC MALFORMATION -Genetics/RF: Most by age 2 -Etiology/Pathogenesis: Head, neck, axilla -Diagnosis/Prognosis: benign massively dilated lymph spaces lined by endothelial cells & separated by intervening connective tissue stroma containing lymphoid aggregates -Pathology: Large, soft masses of dilated, fluid-filled lymph channels that are sequestered and fail to communicate w/ rest of lymph or venous system >2 cm, cystic hygroma -Presentation: Fetal neck or axilla → cystica hygroma (distended fluid-filled spaces)

macrocystic lymphangioma

What disease/disorder: -Etiology: (in more than 80% of cases) idue to coronary artery obstruction by atherosclerotic plaques, resulting in myocardial ischemia that cause a regional reduction in myocardial blood flow and inadequate perfusion of the myocardium supplied by the involved coronary artery & can be exacerbated with increased cardiac energy demand (e.g., myocardial hypertrophy or inc. HR, hypoxemia (anemia), or systemic hypotension (shock)) -There is a long period (decades) of silent, slowly progressive, coronary atherosclerosis → mean age of myocardial ischemia = 60 y/o -More common in men -Myocardial ischemia can also be caused by coronary emboli, coronary vasculitis, or vascular spasm

ischemic heart disease

What is also known as coronary artery disease (CAD) or coronary heart disease (CHD) and is a condition in which there is an inadequate supply of blood and oxygen to a portion of the myocardium and typically occurs when there is an imbalance between myocardial oxygen supply and demand?

ischemic heart disease

What is the most common cause of CHF?

ischemic heart disease

What disorder: -intermediate grade neoplasm -Genetics/RF: - Classic: Elderly Mediterranean men - Endemic African: Children in equatorial Africa - Iatrogenic: Organ transplant recipients - AIDS-Associated: AIDS patients -Etiology/Pathogenesis: HHV8 infection → transmitted via saliva (mainly), sex, blood transfusion, and organ transplantation HHV8 encoded proteins cause cell proliferation and prevent apoptosis cytokines produced by inflammatory cells recruited to sites of infection create a local proliferative millieu -Diagnosis/Prognosis: Spindle cells considered authentic neoplastic element → of endothelial origin and have HHV-8 DNA, notifiedied by PCR AIDS-Associated is the most aggressive form Tx: withdraw immunosuppress. Drug -Pathology: Histo: Patch stage: capillary proliferation in upper dermis around pre-existing blood vessels → extravasated RBCs, hemosiderin macs Plaque stage: more extensive vascular proliferation w/ slit-like vascular channels showing jagged outlines → plump, spindle cells arranged in fascicles surround the proliferating vascular channels & plaque lesions invade entire dermis including hypodermis Nodule/tumor stages: intersecting fascicles of spindle cells and numerous slit-like spaces containing RBCs w/ mitotic figures -Presentation: Classic/Sporadic: skin of lower legs → symmetrical BL bluish red macules on distal areas of LE → enlarge slowly and eventually coalesce to form large plaques → grow into nodular-like lesion → nodules enlarge into dome-shaped tumors → long-standing lesions erode and ulcerate Endemic African: lymph node involvement + lymphedema Iatrogenic: 15-30 mos after organ transplant → primarily affect the skin, Sx similar to classic KS AIDS-Associated: Mult. cutaneous nodules of face and trunk HHV8 → fever + rash

kaposi sarcoma

What disorder: -Etiology/Pathogenesis: LV does not pump enough blood → blood backs up into lungs → pulmonary edema Most often caused by IHD, HTN, aortic and mitral valvular diseases, dilated cardiomyopathy, and myocarditis Pathologic effects result from pulmonary congestion (inc. pressure in pulmonary vv. And venous capillaries) -Diagnosis/Prognosis: Most common CXR: - Kerley B lines (perivascular and interstitial edema) - perivascular and peribronchial cuffing - bat's wing or butterfly pulmonary opacities Serum NP may help differentiate acute HF from other causes of SOB -Pathology: Tends to cause RSHF Cardiac Findings: LV eccentrically hypertrophied (volume overload) + LA dilation → inc. risk for Afib Blood stasis can cause thrombus formation Pulmonary Findings: heavy, congested lungs that exude a frothy-pink edema fluid grossly, alveoli filled with pink-staining edema fluid on microscopy (pigmented macs within alveolar lumina → rupture and diffuse alveolar hemorrhages → RBCs in alveolar spaces phagocytosed and digested by macs, which store Fe as hemosiderin (rust-colored granules on H&E) → hemosiderin-laden macs hallmark for pulmonary edema + responsible for rust-colored sputum) -Presentation: Rust-colored sputum SOB, orthopnea, paroxysmal nocturnal dyspnea and/or Sx of volume overload (leg swelling, weight gain, inc. abdominal girth, R upper quadrant pain due to portal HTN) Earliest Sx = SOB (esp. after exertion) Azotemia from hypoperfusion of kidney → impaired nitrogenous product excretion

left sided CHF

What disorder: -Genetics/RF: Autoimmune: SLE (10%), antiphospholipid Ab syndrome (30%) -Etiology/Pathogenesis: A type of sterile NBTE secondary to autoimmune diseases Lesions develop due to immune complex deposition, with activation of C' and recruitment of Fc-receptor bearing cells -Diagnosis/Prognosis: Lesions heal w/o scarring -Pathology: Most frequently involves mitral valve, followed by aortic valve Vegetations found anywhere on endocardial surface, but most commonly are attached to atrial side of AV leaflet or aortic side of semilunar valves when mitral, veg may extend to chordae tendineae, papillary muscles, and adjacent mural endocardium Histo: intense valvulitis and fibrinoid necrosis of valve substance Minimal valve distortion -Presentation: Pts tend to development thromboembolic events, esp. stroke and TIA

libman-sacks endocarditis

What disorder: -Genetics/RF: Ages 40-70 -Etiology/Pathogenesis: Occur anywhere in heart but mostly RA and LV Most orig. from subendocardium, then sub-epicardium -Pathology: Gross: well-circumscribed, sessile, occasionally pedunculated masses Micro: composed of mature fat cells -Presentation: Vary based on size and location Obstruction → fatigue to syncope Compression → conduction abnormalities

lipoma

What disorder: -Genetics/RF: A type of restrictive cardiomyopathy Confined to rain forest belts of Africa, Asia, and South America -Etiology/Pathogenesis: Myeloproliferative disorder characterized by peripheral blood eosinophilia and eosinophilic infiltration of the subendocardial myocardium → eosinophil degranulation products damage subendocardial myocardium → reactive inflammatory reaction → parietal thrombosis of heart → fibrotic shell that interferes with ventricular filling (restrictive pathophysiology) -Pathology: Excessive dense fibrous thickening in endocardium and inner myocardium due to necrotic-thrombotic process → progresses toward obliteration of ventricular apex and decrease of the longitudinal diameter of ventricular cavity (tends to extend upward to the posterior leaflets of the AV valves, causing regurg) → development of a fibrotic shell

loeffler endomyocarditis

What is indicated for pts with COPD, idiopathic pulmonary fibrosis, CF, alpha1-anti-trypsin deficiency?

lung transplant

What disorder: -Genetics/RF: Increases w age More common in women HTN, diabetes, HLD -Diagnosis/Prognosis: Typically incidental finding -Pathology: Irreg. stony hard nodules behind leaflets Change mitral ring into rigid, curved bar May extend to ventricular septum -Presentation: Mild = no clinical sig. May cause functional mitral stenosis, regurg, or both Conduction deficits (AV block or BBB) Thrombi formation on calcific nodules → stroke Calcific nodules can be site for infective endocarditis

mitral annular calcification

What disorder: -most common cause of mitral regurgitation -Genetics/RF: 1º assoc w other valve abnormalities - Familial = AD 2º assoc w CT disorders (Marfan, Ehlers-Danlos, Osteogenesis imperfecta) -Etiology/Pathogenesis: Mitral valve leaflets become enlarged and redundant + chordae tendineae become thinned + elongated so that the billowed leaflets prolapse into LA during systole -Diagnosis/Prognosis: Dx w echo in 4-10% -Pathology: Excessive mitral tissue → folding and hooding = HALLMARK Gelatinous appearance and slippery texture → due to accumulation of mucopolysaccharides Long chordae, dilated annulus, normal papillary mm. Myxomatous degeneration = accum of mucopolysaccharides ↑ type III collagen -Presentation: Most are asymptomatic Mid-to-late systolic click - If significant: late systolic murmur Complications: IE, NBTE Cerebral emboli common Significant mitral regurg after 10-15 yrs: arrhythmias

mitral valve prolapse

Early biochemical consequences of acute __________ include: Sudden occlusion of major coronary a. causes a shift from aerobic metabolism → anaerobic glycolysis which is inefficient for energy 1. ATP → adenosine, which diffuses out of cardiomyocytes & causes arteriolar dilation and anginal pain 2. Ion pumps are impaired → loss of intracellular K+ and accumulation of intracellular Na+ and water 3. Lactic acid is produced → rise in tissue [H+] → dec. in pH

myocardial infarction

What disorder: -Etiology/Pathogenesis: Region of myocardial necrosis due to prolonged cessation of blood supply Abrupt disruption of coronary artery plaque → non-occlusive or occlusive thrombus formation within arterial lumen (most common) Atherosclerosis is the disease primarily responsible for most cases of acute coronary syndrome --> ~90% of acute [insert disorder] result from an acute thrombus that obstruct an atherosclerotic coronary artery - Other mechanisms of reduced coronary blood flow o Coronary arterial spasm o Coronary vasculitis of the epicardial coronary arteries (Kawasaki disease, Takayasu arteritis) o Emboli from the LA in association w/ Afib, a L sided mural thrombus, vegetations of infective endocarditis, or paradoxical emboli from the R side of the heart or the periphereal veins traversing a patent foramen ovale and into the coronary aa. - Uncommon causes of acute [insert disorder]: o Vaso-occlusive crises of sickle cell disease, aortic dissection with retrograde involvement of the coronary arteries (e.g., in marfan syndrome), and supply-demand imbalance due to marked hypertrophy inc. myocardial oxygen demands (e.g., aortic stenosis), lowered systemic BP (e.g., circulatory shock), or marked hypoxemia (e.g., severe anemia)

myocardial infarction

What develops when coronary blood flow is diminished relative to myocardial demand & is a MAJOR contributing factor to ischemic heart disease? Hint: caused by coronary atherosclerosis, acute thrombosis overlying a disrupted atherosclerotic plaque, vasospasm

myocardial ischemia

What disorder: -Genetics/RF: Mean age 42 Bimodal: young kids/teens, older adults M > F -Etiology/Pathogenesis: Inflammatory disease of the heart muscle caused by infectious or non-infectious conditions → result of exposure to either discrete external agents or internal triggers (e.g., autoimmune activation) Primary or secondary Most caused by virus (esp. adenovirus) 4 Phases: 1. Direct virus-mediated myocardial damage 2. Immune-mediated myocardial damage 3. Resolution of inflammation 4. Myocardial remodeling Mechanisms to produce dilated CM: 1. Persistent viral infection: Coxsackie → 2 specific viral proteases (2A and 3C) are encoded and produced during infection → 2A cleaves dystrophin, a protein located between the sarcolemmal protein dystroglycan and actin cytoskeleton of the cardiomyocyte 2. Immune-mediated destruction: Persistent activation of T cells and/or continued Ab-mediated cardiomyocyte destruction -Diagnosis/Prognosis: Most cases believed to be subclinical → non-specific lab results w/ no endomyocardial biopsy Gold standard: endomyocardial biopsy To confirm viral etiology → PCR analysis of biopsy sample required - Asymp: transient ECG abnormalities in T wave, echo, cardiac cath, May cause complete recovery or sudden cardiac death - Fulminant echo: global hypokinesis Biopsy: myocarditis Tx supportive - Acute: Elevated troponin, ST elevation on ECG, segmental wall motion abnormalities on echo - Chronic: CXR = cardiomegaly Echo: LV dilation, reduced EF Elevated cardiac enzymes → indicator of necrosis of myocardial cells ECG: non-specific ST changes, atrial or ventricular arrythmias OR ST-elevations simialr to pericarditis OR regional ST elevations + Q waves similar to MI CXR: cardiomegaly + Kerley B-lines - Complete resolution in most pts -Recurrence in 10-25% -Fulminant → small # evolve to HF and death - progression towards DCM → proportion evolve to this after a long latency period -Pathology: Flabby, enlarged heart with dilatation of either ventricle or all cardiac chambers (extensive) Myocardium mottled w pale foci and hyperemic- hemorrhagic areas +/- Mural thrombi +/- Pericardial effusion Late stages: focal or diffuse fibrosis Dallas criteria: inflamm infiltrate + cardiomyocyte necrosis in same slide - PMN infiltrate if necrosis is extensive Proliferation of fibroblasts → deposit collagen Histo Types: Lymphocytic infiltrate (most common) → viral in origin, characterized by myocardial patchy infiltration of T lymphocytes w/ damage to adjacent cardiomyocytes Giant cell (rare, very aggressive, often fatal) → characterized by intense inflammation, extensive areas of myocyte necrosis, and numerous multi-nucleated giant cells of macrophage origin, plasma cells, easily identified eosinophils, and occasional neutrophils Granulomatous (e.g., sarcoidosis and TB) → in lungs, kidney, heart → extensive infiltration by activated macs leading to chronic inflammation and tissue damage Eosinophilic → characterized by infiltration of lymphocytes, macs, and eosinophils, due to hypersensitivity rxn to pharm agents + assoc. w/ peripheral eosinophilia Toxic→ characterized by necrosis of cardiomyocytes → inflammation seen as extensive intracellular TG accumulation, cardiomyocyte coagulative necrosis and myocytolysis, and a mononuclear inflammatory cell response that is mild compared to wide extent of myocyte damage -Presentation: Antecedent viral-type illness: 8-10 days following a flu-like syndrome of fevers, arthralgias, and malaise or pharyngitis, tonsillitis, or URTI Cardiac involvement → Types: - Asymptomatic (most cases, esp. viral) - Fulminant: acute severe HF at rest or cardio shock (BP 70/40, tachycardia of >120 bpm) - Acute coronary-like syndromes: new chest pain at rest, unexplained HF (dyspnea on exertion), palpitations, fatigue, arrhythmias - Chronic: Dilated CM → insidious onset of Sx compatible with moderate ventricular dysfunction → fatigue, dyspnea, ↑ JVD P, LE edema, rales, S3/S4 gallops, mitral/tricuspid regurg murmurs

myocarditis

Which complication of long-term post-transplant immunosuppression: -Recipients of organ transplants are at INCREASED risk for development of malignant neoplasms → skin cancers (squamous cell and basal cell carcinoma) followed by post-transplant lymphoproliferative disorders (PTLDs)

neoplasms

What disorder: -Genetics/RF: Advanced malignancy (80%): adenocarcinoma of pancreas, lung, colon, ovary, biliary, and prostate cancers -Etiology/Pathogenesis: Tumor secretes procoagulants → hypercoag state → endothelial injury Also caused by sepsis, burns Pathogenesis in pt w/ normal valves: 1. hypercoagulable state 2. endothelial damage caused by circulating cytokines → triggers platelet deposition → disease Pathogenesis in pt w/ pre-existing valve disease: endothelial injury is consequence of jet effects (blood flow turbulences) from valve stenosis and/or regurg → triggers platelet deposition → disease Pathogenesis in pt w/ burns: Hemodynamic monitoring w/ intracardiac catheters induces acute endothelial damage → triggers platelet deposition → disease -Diagnosis/Prognosis: Transesophageal echo: valvular lesions Tx: systemic anticoagulation -Pathology: Deposition of sterile thrombotic vegetation on heart valves Vegetations: friable, white/tan masses usually on mitral or aortic valves → composed of platelets interwoven w fibrin -Presentation: Asymp until systemic embolization occurs: Cerebral most common: stroke, TIA, psych sx (anxiety, psychosis) Kidney, spleen, mesenteric bed, skin, extremities: flank pain, hematuria, rash, digital

non-bacterial endocarditis

What disorder: -2nd most common cardiac tumor + most common tumor of heart valves -Genetics/RF: Mean age = 60 y/o Point mutation in K-RAS gene -Etiology/Pathogenesis: Affects heart valves Most commonly affect aortic valve, then mitral -Diagnosis/Prognosis: echo -Pathology: Gross: "Sea anemone-like" d/t papillary fronds Small, avascular, thin stalk Micro: Central core of myxoid connective tissue covered by endothelium -Presentation: Asymptomatic Possible cause of systemic and pulm. embolism → stroke, MI, PE, syncope, sudden death

papillary fibroelastoma

What disorder: -Etiology/Pathogenesis: Usually d/t ischemia/infarction → mitral regurg Reduction in coronary blood flow (esp. terminal br. of intramyocardial coronary aa.) → interferes with papillary m. function -Presentation: Brief ischemia → stunning, temporary mitral regurg Severe ischemia → permanent dysfunction + regurg

papillary muscle dysfunction

What disorder: -Etiology/Pathogenesis: Accumulation of excess fluid within pericardial cavity, either as a transudate or exudate Hydropericardium: effusion of serous fluid into pericardial sac due to conditions w/ generalized edema (e.g., CHF, renal dz, nutritional/hepatic disorders) Chylous: results from a communication of thoracic duct w/ pericardial space secondary to lymphatic obstruction by tumor or infection Serosanguineous: Chest trauma (accidental or caused by cardiopulmonary resuscitation) Hemopericardium: wounds, Ventricular wall rupture (from MI), and aortic aneurysm rupture Cardiac Tamponade: ↑ Pericardial P from rapid accumulation of pericardial fluid → restricts filling of heart by compressing all cardiac chambers → total venous return falls → CO and BP dec. -Diagnosis/Prognosis: Cardiac Tamponade: CXR → rounded, flask-like appearance of cardiac silhouette Acute cardiac tamponade most always fatal unless pressure relieved by removing pericardial fluid via needle pericardiocentesis or surgery -Pathology: Hydropericardium: Watery clear or straw-colored serous fluid w/ low protein content and few cellular elements Chylous: Turbid/milky fluid (high lipid content) Serosanguineous: Blood + clear yellow blood serum; watery, pink, or pale red that does not clot Hemopericardium: Frank blood in pericardium, usually clots Cardiac Tamponade: Accumulation of pericardial fluid -Presentation: Cardiac Tamponade: ranges from minimally symptomatic → abrupt cardiovascular collapse and death, paradoxical pulse (abnormally large decline in systemic arterial pressure during inspiration w/ normal fall in BP being <10 mmHg) → low BP, dilated neck veins, muffled heart sounds, tachypnea, diaphoresis, cool extremities, and peripheral cyanosis

pericardial effusion

What disorder: -vascular malformation -CAPILLARY MALFORMATION -Diagnosis/Prognosis: persists through life → becomes thick and nodular over time -Pathology: Sharply demarcated red macule/patch and, microscopically, consists of a myriad of small vessels of capillary size -Presentation: Present at birth and grow commensurate with the child Unilateral face (most common) or trunk

port-wine stain (nevus flammeus)

What disorder: -Genetics/RF: smoking, often in F in 50s -Etiology/Pathogenesis: Endothelial dysfunction → deficit basal release of NO -Diagnosis/Prognosis: Responds to nitroglycerin ECG: ST-segment elevation Coronary angiography: focal epicardial coronary a. spasm @ site of atherosclerotic stenosis Gold std: coronary angiography w/ injection of provocative agents (ergonovine) -exaggerated spasm is diagnostic -Pathology: No pathological plaque/deposits -Presentation: Transient coronary a spasms w/ or w/o exertion, usually at rest w/o cause @ night 25% have generalized vasospastic diathesis (migraine HAs + Raynaud's)

prinzmetal angina

What disorder: -reactive vascular hyperplasia -Genetics/RF: Mostly early childhood, young women GoF mutation: RAS, BRAF, or GNA14 -Etiology/Pathogenesis: Primary cause = trauma Reactive connective tissue proliferation that occurs on the skin and mucous membranes, characterized by rapid growth and friable surface -Pathology: Smooth, well-circumscribed, bright red, domed nodule that bleeds easily Micro: proliferation of many capillaries disposed radially to skin surface and separated by fibrous septa → edematous stroma w/ mixed inflammatory infiltrate -Presentation: Children → nodules mostly on head, neck, and UE

pyogenic granuloma

What consist of proliferation of blood vessels in the skin or various viscera triggered by an infection?

reactive vascular hyperplasia

What disorder: -Genetics/RF: Idiopathic: MYH7 and TNNI3 genes → Loeffler endocarditis + endomyocardial fibrosis Secondary: amyloidosis, hemochromatosis, or sarcoidosis -Etiology/Pathogenesis: Characterized by restrictive filling and reduced diastolic volume of either or both ventricles Abnormal diastolic properties of the V are attributable to myocardial fibrosis, infiltration, or scarring of endomyocardial surface Idiopathic: restrictive heart disease of unknown etiology w/o specific endocardial pathological changes → dilation of both L and R atria is commonly observed due to restricted ventricular filling and pressure overloads -Pathology: Inc. stiffness of myocardium in LV and RV leading to diminished compliance and diastolic function with NORMAL systolic function and ABSENT hypertrophy, reduced diastolic volumes (of 1 or both ventricles) Fundamental abnormality = interstitial accumulation of abnormal protein (amyloid) or fibrosis and/or calcification Loeffler endocarditis + endomyocardial fibrosis characterized by formation of an endocardial fibrotic shell w/ extension into myocardium

restrictive cardiomyopathy

What disorder: -Etiology/Pathogenesis: Contraction of RV is compromised Most common cause is LSHF → increase in pulmonary pressure incidental to LSHF inevitably burdens R heart Pure RSHF infrequent, usually occurs in pts w/ cor pulmonale, which is mostly associated with parenchymal diseases of the lung (emphysema, COPD) but can also arise due to disorders that affect the pulmonary vasculature (e.g., primary pulmonary HTN, recurrent pulmonary thromboembolism) or that produce hypoxia (e.g., OSA, altitude sickness) Pulmonary HTN → hypertrophy and dilation of R heart -Pathology: Pulmonary congestion is MINIMAL Engorgement of systemic and portal venous systems may be pronounced Cardiac Findings: hypertrophy and dilation of RA + RV, associated with normal LV and LA Liver Findings: congestion of hepatic and portal vessels → inc. size + weight (congestive hepatomegaly) most prominent around central vv. Within hepatic lobules, which show red-brown centrilobular discoloration and paler, sometimes fatty, peripheral regions ("nutmeg liver") → long-standing can cause central areas to become fibrotic (cardiac cirrhosis) - portal HTN produces congestive splenomegaly + chronic congestion and edema of bowel wall, which may be so severe that it interferes w/ absorption of nutrients -Presentation: Jugular venous distention Systemic edema (leg edema, hepatomegaly) Anorexia, bloating, nausea, constipation JVD, pleural effusions, dyspnea, ankle and pretibial edema (hallmark)

right sided CHF

What disorder: -vascular malformation -CAPILLARY MALFORMATION -Diagnosis/Prognosis: Regress/fade during childhood -Pathology: Pink/red, irreg, macular patches -Presentation: Midline of face

salmon patch (nevus simplex)

What disorder: -vascular ectasia -Genetics/RF: Children and adults -Etiology/Pathogenesis: Dilation of central arteriole w radiating capillary branches Develop from the dilatation of pre-existing blood vessels Can occur from pregnancy or oral contraceptives -Diagnosis/Prognosis: Resolves after pregnancy or d/c of medication -Pathology: Compression of ventral vessel produces blanching and temporarily obliterates lesion → when released, the threadlike vessels quickly refill w/ blood from central arteriole -Presentation: Face, neck, upper trunk, arms Normal or pathologic Common in liver cirrhosis

spider angioma

What disorder: -vascular malformation -CAPILLARY MALFORMATION -Genetics/RF: Activating mutation of GNAQ gene on chromosome 9q21.2 → part of MAPK pathway that communicate signal from EGFR -Etiology/Pathogenesis: sporadic developmental disorder of vascular malformations of face, choroid, and leptomeninges due to increased signaling to epidermal growth factor receptor (EGFR) -Pathology: Vascular malformations of face, choroid, and leptomeninges -Presentation: Present at birth and grow commensurate with the child Face: port wine stain in trigeminal n. distribution Choroid: ↑IOP → glaucoma Leptomeningeal: seizures, migraines, hemiparesis, stroke-like episodes

sturge-weber syndrome

What disorder: -Etiology/Pathogenesis: Coronary flow is reduced enough to produce detectable myocardial necrosis 1st step = erosion or disruption of atheromatous plaque by endothelial injury, intraplaque hemorrhage, or mechanical forces → expose subendothelial collagen + contents to blood 2nd step = platelets adhere, aggregate, and are activated → recruit other circulating platelets via thromboxane A2, ADP, ultra-large von Willebrand factor multimers, serotonin, and thrombin 3rd step = release of tissue factor (coagulation factor III) from vascular smooth m. triggers activation of factor X → converts prothrombin to thrombin → forms thrombus -Diagnosis/Prognosis: Elevated troponin T or I and CK-MB ECG: ST-segment depression, elevation or T-wave inversion -Pathology: Culprit lesion = erosion or rupture of an atherosclerotic plaque w/ non-occlusive thrombus causing a severe narrowing of coronary a. lumen -Presentation: Stable = deep, poorly localized chest or arm discomforted reproduced by exertion or emotional stress, relieved by nitroglycerin Unstable = CP w/ 1 of 3 features 1. occurring at rest (or minimal exertion) 2. being severe and flank pain 3. occurring w/ crescendo pattern (pain that is more severe, prolonged, or frequent than previously)

subendocardial infarction (NSTEMI)

What disorder: -Etiology/Pathogenesis: Subendocardial infarct: non-occlusive thrombus that develops on a disrupted atherosclerotic plaque → normally the subendocardial zone is least perfused region, thus the most vulnerable to any reduction in coronary blood flow Circumferential: supply-demand imbalance due to massive hemorrhage or circulatory shock superimposed on chronic, otherwise non-critical, atherosclerotic coronary stenoses on 2 or 3 major coronary arteries -Diagnosis/Prognosis: ST segment depression or new T wave inversion, CK-MB or TnT/TnI elevated Complications are rare Good prognosis (30-day mortality rate 2%) -Pathology: Affects the inner 1/3 - ½ of the LV May arise within the territory of one of the major epicardial coronary arteries (regional infarct) May involve subendocardial territories of all the coronary arteries (circumferential infarct) -Presentation: Stable = deep, poorly localized chest or arm discomforted reproduced by exertion or emotional stress, relieved by nitroglycerin Unstable = CP w/ 1 of 3 features 1. occurring at rest (or minimal exertion) 2. being severe and flank pain 3. occurring w/ crescendo pattern (pain that is more severe, prolonged, or frequent than previously)

subendocardial infarction (NSTEMI)

What disorder: -Etiology/Pathogenesis: Coronary atherosclerosis underlies 80% Vfib preceded or not by ventricular tachycardia is mechanism of death in 70% of cases Bradyarrythmias and asystole in 30% -Diagnosis/Prognosis: Elevated cardiac enzymes in 50% of resuscitated pts Autopsy showing Cardiac abnormalities: - Cardiomyopathy (hypertrophic, dilated, or restrictive) - Valvular heart disease (aortic stenosis, mitral valve prolapse) - Myocarditis, CHD, or arterial HTN w/ LVH -Presentation: Unexpected death usually within 1 hour of Sx onset Prodromal Sx = palpitations, CP, dyspnea

sudden cardiac death

What disorder: -Etiology/Pathogenesis: Impaired ump function with reduced ejection fraction and an enlarged EDV LV contracts less forcefully and pumps out a lower % of blood that is returned to it → more blood remains in LV → LV enlargement, causing mass increase (hypertrophy) but cavity size is increased disproportionately → eccentric hypertrophy Most common causes → MI, myocarditis, dilated cardiomyopathy -Pathology: Enlarged LV cavity

systolic CHF

What disorder: -vascular ectasia -Genetics/RF: UV exposure, radiation, trauma, topical steroids -Etiology/Pathogenesis: Permanent dilation of capillaries/venules on skin or mucosal surfaces -Pathology: Mainly involves capillaries or venules -Presentation: Seen in SLE, scleroderma

telangiectasia

What are complications to mechanical or bioprostheses (prosthetic) valves?

thromboembolic issues like stroke, Infective endocarditis, Structural deterioration, intravascular hemolysis due to high shear forces, paravalvular leak due to inadequate healing, or obstruction due to overgrowth of fibrous tissue during healing process

What disorder: -Etiology/Pathogenesis: Associated with superimposed luminal thrombus as a result of the disruption of an atherosclerotic plaque that is not hemodynamically significant (50-60% stenosis) -Diagnosis/Prognosis: ST segment elevation, pathological Q waves as it evolves 30-day mortality rate btwn 2.5-10% Complications: cardiogenic shock, infarct expansion (anterior), pericarditis, ventircular aneurysms Masson trichrome stains collagen fibers green and myocardial cells red after healed -Pathology: Involves the full or nearly full thickness of the ventricular wall in distribution of a single coronary artery -Presentation: TRUE MEDICAL EMERGENCY

transmural infarction (STEMI)

What occurs when a recipients immune system recognizes the graft as being foreign and attacks it (immune system remains most formidable barrier to transplantation)?

transplant rejection

T/F: if there is a cardiac tumor present, it is most likely to be MALIGNANT (100x more common to be metastatic than primary)

true

What disorder: -Genetics/RF: AD, TSC1 (encodes hamartin on chromosome 9) or TSC2 (encodes tuberin on chromosome 16) tumor suppressor genes -Etiology/Pathogenesis: "Two hit" hypoth. → loss of TSC1 and 2 expression → overgrowth of tumors (hamartomas) in multiple organs -Diagnosis/Prognosis: Higher risk for malignant tumors → renal cell carcinoma (kidney), glioblastoma (brain), and malignant angiomyolipoma (soft tissues) -Pathology: Glioneuronal hamartoma, sub-ependymal glial nodules, sub-ependymal giant cell astrocytomas → CNS tumors Angiomyolipomas and renal cell carcinomas → renal tumors Lymphangioleiomyomatosis → alveolar smooth m. proliferation and cystic destruction of parenchyma -Presentation: Most often due to TSC2 gene mutations Severe: 1st year of life → seizures, delayed mental development, neuropsychiatric disorders Mild: subtle predominantly dermatological features in adulthood CNS sx due to brain tumors Cutaneous: "ash leaf spots", facial angiofibromas Cardiac rhabdomyomas, kidney tumors, lymphangioleiomyomatosis (F only → dyspnea + pneumothorax)

tuberous sclerosis complex

What disorder: -Genetics/RF: Malignant melanoma, pleural mesothelioma, lung and breast cancers, malignant lymphoma -Etiology/Pathogenesis: x100 more common than 1º tumors of heart Spread via lymph or blood, or direct or transvenous extension - lymph → usually pericardium - blood → usually myocardium -Pathology: Small and multiple cardiac metastases in (descending order of frequency) pericardium → myocardium → endocardium -Presentation: Clinically silent, not usually found until after death Pericardial may cause pericarditis w effusion Myocardial may cause HF

tumors metastatic to the heart

What disorder: -Genetics/RF: Mean age 62 y/o -Etiology/Pathogenesis: NO detectable myocardial necrosis 1st step = erosion or disruption of atheromatous plaque by endothelial injury, intraplaque hemorrhage, or mechanical forces → expose subendothelial collagen + contents to blood 2nd step = platelets adhere, aggregate, and are activated → recruit other circulating platelets via thromboxane A2, ADP, ultra-large von Willebrand factor multimers, serotonin, and thrombin 3rd step = release of tissue factor (coagulation factor III) from vascular smooth m. triggers activation of factor X → converts prothrombin to thrombin → forms thrombus -Diagnosis/Prognosis: NORMAL metabolites ECG: ST-segment depression, elevation or T-wave inversion -Pathology: Culprit lesion = erosion or rupture of an atherosclerotic plaque w/ non-occlusive thrombus causing a severe narrowing of coronary a. lumen -Presentation: Stable = deep, poorly localized chest or arm discomforted reproduced by exertion or emotional stress, relieved by nitroglycerin Unstable = CP w/ 1 of 3 features 1. occurring at rest (or minimal exertion) 2. being severe and flank pain 3. occurring w/ crescendo pattern (pain that is more severe, prolonged, or frequent than previously)

unstable angina

What is also known as incompetence or insufficiency and is the failure of a valve to close completely, thereby allowing reversed flow?

valve regurgitation

What is the failure of a valve to open completely, impeding forward flow?

valve stenosis

What are characterized by anomalous dilation of small venules, arterioles, or capillaries → can be localized on skin only or be generalized and affect the skin, mucous membranes, GI tract, and other organs? hint: abnormal, fragile, and bleed more easily

vascular ectasias

What are congenital blood vessel anomalies that do not undergo true proliferation → grow commensurately with the child, do not involute, but may expand hemodynamically forming a soft tissue mass and result from developmental errors during embryogenesis?

vascular malformations

What are neoplasms characterized by increased proliferation rates of endothelial and other vascular cells?

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