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Flashcards in Cardiovascular - Pathology (2) Deck (25):
1

Dilated cardiomyopathy

  • Definition
  • Other etiologies
  • Findings
  • Treatment

  • Definition
    • Most common cardiomyopathy (90% of cases).
    • Often idiopathic or congenital.
  • Other etiologies (ABCCCD)
    • Chronic Alcohol abuse, wet Beriberi, Coxsackie B virus myocarditis, chronic Cocaine use, Chagas disease, Doxorubicin toxicity, hemochromatosis, and peripartum cardiomyopathy.
  • Findings
    • Heart failure, S3, dilated heart on echocardiogram, balloon appearance of heart on CXR.
    • Systolic dysfunction ensues.
    • Eccentric hypertrophy (sarcomeres added in series).
  • Treatment:
    • Na+ restriction, ACE inhibitors, β-blockers, diuretics, digoxin, implantable cardioverter defibrillator (ICD), heart transplant.

2

Hypertrophic cardiomyopathy

  • Definition
  • Findings
  • Treatment
  • Obstructive HCM 

  • Definition
    • 60–70% of cases are familial, autosomal dominant (commonly a β-myosin heavy-chain mutation).
    • Rarely can be associated with Friedreich ataxia.
    • Cause of sudden death in young athletes, due to ventricular arrhythmia.
  • Findings:
    • S4, systolic murmur.
    • Diastolic dysfunction ensues.
    • Marked ventricular hypertrophy [A], often septal predominance.
    • Myofibrillar disarray and fibrosis.
  • Treatment:
    • Cessation of high-intensity athletics
    • Use of β-blocker or non-dihydropyridine calcium channel blockers (e.g., verapamil). 
    • ICD if patient is high risk.
  • Obstructive HCM (subset):
    • Hypertrophied septum too close to anterior mitral leaflet
    • -->Ž outflow obstruction Ž
    • --> dyspnea, possible syncope.

3

Restrictive/infiltrative cardiomyopathy

  • Major causes
    • Sarcoidosis
    • Amyloidosis
    • Postradiation fibrosis
    • Endocardial fibroelastosis (thick fibroelastic tissue in endocardium of young children)
    • Löffler syndrome (endomyocardial fibrosis with a prominent eosinophilic infiltrate)
    • Hemochromatosis (dilated cardiomyopathy can also occur).
  • Diastolic dysfunction ensues.
  • Can have low-voltage ECG despite thick myocardium (especially amyloid).

4

CHF

  • Definition
  • Symptoms and signs
  • Systolic dysfunction
  • Diastolic dysfunction
  • RHF
  • Treatment

  • Definition
    • Clinical syndrome of cardiac pump dysfunction.
  • Symptoms and signs
    • Symptoms: dyspnea, orthopnea, and fatigue
    • Signs: rales, JVD, and pitting edema [A].
  • Systolic dysfunction
    • Low EF, poor contractility, often 2° to ischemic heart disease or DCM.
  • Diastolic dysfunction
    • Normal EF and contractility, impaired relaxation, decreased compliance.
  • Right heart failure
    • Most often results from left heart failure.
    • Isolated right heart failure is usually due to cor pulmonale.
  • Treatment
    • ACE inhibitors, β-blockers (except in acute decompensated HF), angiotensin II receptor blockers, and spironolactone decrease mortality.
    • Thiazide or loop diuretics are used mainly for symptomatic relief. 
    • Hydralazine with nitrate therapy improves both symptoms and mortality in select patients.

5

Causes of these CHF abnormalities

  • Cardiac dilation
  • Dyspnea on exertion 
  • Pulmonary edema
  • Orthopnea
  • Paroxysmal nocturnal dyspnea
  • Hepatomegaly
  • Peripheral edema
  • Jugular venous distention

  • Cardiac dilation
    • Greater ventricular end-diastolic volume.
  • Dyspnea on exertion
    • Failure of CO to increase during exercise.
  • Pulmonary edema (LHF)
    • Increased pulmonary venous pressure -->Ž pulmonary venous distention and transudation of fluid.
    • Presence of hemosiderin-laden macrophages (“heart failure” cells) in the lungs.
  • Orthopnea (LHF)
    • Shortness of breath when supine
    • Increased venous return from redistribution of blood (immediate gravity effect) exacerbates pulmonary vascular congestion.
  • Paroxysmal nocturnal dyspnea (LHF)
    • Breathless awakening from sleep
    • Increased venous return from redistribution of blood, reabsorption of edema, etc.
  • Hepatomegaly (nutmeg liver) (RHF)
    • Increased central venous pressure -->Ž increased resistance to portal flow.
    • Rarely, leads to “cardiac cirrhosis.”
  • Peripheral edema (RHF)
    • Increased venous pressure Ž--> fluid transudation.
  • Jugular venous distention (RHF)
    • Increased venous pressure.

6

Bacterial endocarditis

  • Symptoms
  • Valves
  • Complications

  • Symptoms:
    • Fever (most common symptom), new murmur, Roth spots (round white spots on retina surrounded by hemorrhage), Osler nodes (tender raised lesions on finger or toe pads), Janeway lesions (small, painless, erythematous lesions on palm or sole), anemia, splinter hemorrhages [A] on nail bed.
    • ♥ Bacteria FROM JANE ♥:
      • Fever
      • Roth spots
      • Osler nodes
      • Murmur
      • Janeway lesions
      • Anemia
      • Nail-bed hemorrhage
      • Emboli
  • Valves
    • Mitral valve is most frequently involved.
    • Tricuspid valve endocarditis is associated with IV drug abuse (don’t “tridrugs): associated with S. aureus, Pseudomonas, and Candida.
  • Complications
    • Chordae rupture, glomerulonephritis, suppurative pericarditis, emboli.

7

Bacterial endocarditis

  • Diagnosis
  • Acute
  • Subacute
  • Culture-negative
  • May also be...

  • Diagnosis
    • Multiple blood cultures necessary for diagnosis.
  • Acute
    • S. aureus (high virulence). 
    • Large vegetations on previously normal valves [B].
    • Rapid onset.
  • Subacute
    • Viridans streptococci (low virulence).
    • Smaller vegetations on congenitally abnormal or diseased valves. 
    • Sequela of dental procedures.
    • Gradual onset.
  • Culture-negative
    • Most likely Coxiella burnetii and Bartonella spp.
  • Endocarditis may also be nonbacterial 2° to malignancy, hypercoagulable state, or lupus (marantic/thrombotic endocarditis).
    • S. bovis is present in colon cancer, S. epidermidis on prosthetic valves.

8

Rheumatic fever

  • A consequence of...
  • Late sequelae
  • Lesions
  • Associated with...
  • Immunology
  • Symptoms

  • A consequence of pharyngeal infection with group A β-hemolytic streptococci.
    • Early deaths due to myocarditis.
  • Late sequelae 
    • Include rheumatic heart disease, which affects heart valves
    • Mitral > aortic >> tricuspid (high-pressure valves affected most).
  • Lesions
    • Early lesion is mitral valve regurgitation
    • Late lesion is mitral stenosis.
  • Associated with...
    • Aschoff bodies (granuloma with giant cells (blue arrows in [A])
    • Anitschkow cells (enlarged macrophages with ovoid, wavy, rod-like nucleus (red arrow in [A]))
    • Increased ASO titers. 
  • Immunology
    • Immune mediated (type II hypersensitivity)
      • Not a direct effect of bacteria.
    • Antibodies to M protein cross-react with self antigens.
  • Symptoms (FEVERSS)
    • Fever
    • Erythema marginatum
    • Valvular damage (vegetation and fibrosis)
    • ESR increased
    • Red-hot joints (migratory polyarthritis)
    • Subcutaneous nodules
    • St. Vitus’ dance (Sydenham chorea)

9

Acute pericarditis

  • Definition
  • Fibrinous
  • Serous
  • Suppurative/purulent

  • Definition
    • Commonly presents with sharp pain, aggravated by inspiration, and relieved by sitting up and leaning forward.
    • Presents with friction rub.
    • ECG changes include widespread ST-segment elevation and/or PR depression.
  • Fibrinous
    • Caused by Dressler syndrome, uremia, radiation.
    • Presents with loud friction rub.
  • Serous
    • Viral pericarditis (often resolves spontaneously)
    • Noninfectious inflammatory diseases (e.g., rheumatoid arthritis, SLE).
  • Suppurative/purulent
    • Usually caused by bacterial infections (e.g., Pneumococcus, Streptococcus).
    • Rare now with antibiotics.

10

Cardiac tamponade

  • Definition
  • Findings
  • Pulsus paradoxus

  • Definition
    • Compression of heart by fluid (e.g., blood, effusions) in pericardium [A], leading to decreased CO.
    • Equilibration of diastolic pressures in all 4 chambers.
  • Findings:
    • Beck triad (hypotension, distended neck veins, distant heart sounds), increased HR, pulsus paradoxus, Kussmaul sign. ECG shows low-voltage QRS and electrical alternans (due to “swinging” movement of heart in large effusion).
  • Pulsus paradoxus
    • Decrease in amplitude of systolic blood pressure by ≥ 10 mmHg during inspiration. 
    • Seen in cardiac tamponade, asthma, obstructive sleep apnea, pericarditis, and croup.

11

Syphilitic heart disease

  • 3° syphilis disrupts the vasa vasorum of the aorta with consequent atrophy of the vessel wall and dilation of the aorta and valve ring.
  • May see calcification of the aortic root and ascending aortic arch.
    • Leads to “tree bark” appearance of the aorta.
  • Can result in aneurysm of the ascending aorta or aortic arch and aortic insufficiency.

12

Cardiac tumors

  • Definition
  • Myxomas
  • Rhabdomyomas

  • Definition
    • Most common heart tumor is a metastasis (e.g., from melanoma, lymphoma).
  • Myxomas
    • Most common 1° cardiac tumor in adults [A].
    • 90% occur in the atria (mostly left atrium).
    • Myxomas are usually described as a “ball valve” obstruction in the left atrium (associated with multiple syncopal episodes).
  • Rhabdomyomas
    • Most frequent 1° cardiac tumor in children (associated with tuberous sclerosis).

13

Kussmaul sign

  • Increase in JVP on inspiration instead of a normal decrease.
  • Inspiration Ž
    • --> negative intrathoracic pressure not transmitted to heart Ž
    • --> impaired filling of right ventricle Ž
    • --> blood backs up into venae cavae Ž
    • --> JVD.
  • May be seen with constrictive pericarditis, restrictive cardiomyopathies, right atrial or ventricular tumors.

14

Raynaud phenomenon

  • Definition
  • Called...
    • Primary
    • Secondary

  • Definition
    • Decreased blood flow to the skin due to arteriolar vasospasm in response to cold temperature or emotional stress.
    • Most often in the fingers and toes.
    • Affects small vessels.
  • Called:
    • Raynaud disease when 1° (idiopathic)
    • Raynaud syndrome when 2° to a disease process such as mixed connective tissue disease, SLE, or CREST (limited form of systemic sclerosis) syndrome.

15

Vascular tumors

  • Strawberry hemangioma
  • Cherry hemangioma
  • Pyogenic granuloma 
  • Cystic hygroma 
  • Glomus tumor 

  • Strawberry hemangioma
    • Benign capillary hemangioma of infancy [A].
    • Appears in first few weeks of life (1/200 births)
    • Grows rapidly and regresses spontaneously at 5–8 years old.
  • Cherry hemangioma
    • Benign capillary hemangioma of the elderly [B].
    • Does not regress.
    • Frequency increases with age.
  • Pyogenic granuloma
    • Polypoid capillary hemangioma that can ulcerate and bleed.
    • Associated with trauma and pregnancy.
  • Cystic hygroma
    • Cavernous lymphangioma of the neck.
    • Associated with Turner syndrome.
  • Glomus tumor
    • Benign, painful, red-blue tumor under fingernails.
    • Arises from modified smooth muscle cells of glomus body.

16

Vascular tumors

  • Bacillary angiomatosis 
  • Angiosarcoma
  • Lymphangiosarcoma
  • Kaposi sarcoma

  • Bacillary angiomatosis
    • Benign capillary skin papules found in AIDS patients.
    • Caused by Bartonella henselae infections. 
    • Frequently mistaken for Kaposi sarcoma.
  • Angiosarcoma
    • Rare blood vessel malignancy typically occurring in the head, neck, and breast areas.
    • Usually in elderly, on sun-exposed areas.
    • Associated with radiation therapy and arsenic exposure.
    • Very aggressive and difficult to resect due to delay in diagnosis.
  • Lymphangiosarcoma
    • Lymphatic malignancy associated with persistent lymphedema (e.g., post–radical mastectomy).
  • Kaposi sarcoma
    • Endothelial malignancy most commonly of the skin, but also mouth, GI tract, and respiratory tract.
    • Associated with HHV-8 and HIV.
    • Frequently mistaken for bacillary angiomatosis.

17

Temporal (giant cell) arteritis

  • Type of condition
  • Epidemiology / Presentation
  • Pathology / Labs
  • Treatment

  • Type of condition
    • Large-vessel vasculitis
  • Epidemiology / Presentation
    • Generally elderly females.
    • Unilateral headache (temporal artery), jaw claudication.
    • May lead to irreversible blindness due to ophthalmic artery occlusion.
    • Associated with polymyalgia rheumatica.
  • Pathology / Labs
    • Most commonly affects branches of carotid artery.
    • Focal granulomatous inflammation [A].
    • Increased ESR.
  • Treatment
    • Treat with high-dose corticosteroids prior to temporal artery biopsy to prevent vision loss.

18

Takayasu arteritis

  • Type of condition
  • Epidemiology / Presentation
  • Pathology / Labs
  • Treatment

  • Type of condition
    • Large-vessel vasculitis
  • Epidemiology / Presentation
    • Asian females < 40 years old.
    • “Pulseless disease” (weak upper extremity pulses), fever, night sweats, arthritis, myalgias, skin nodules, ocular disturbances.
  • Pathology / Labs
    • Granulomatous thickening and narrowing of aortic arch [B] and proximal great vessels.
    • Increased ESR.
  • Treatment
    • Treat with corticosteroids.

19

Polyarteritis nodosa

  • Type of condition
  • Epidemiology / Presentation
  • Pathology / Labs
  • Treatment

  • Type of condition
    • Medium-vessel vasculitis
  • Epidemiology / Presentation
    • Young adults.
    • Hepatitis B seropositivity in 30% of patients.
    • Fever, weight loss, malaise, headache.
    • GI: abdominal pain, melena.
    • Hypertension, neurologic dysfunction, cutaneous eruptions, renal damage.
  • Pathology / Labs
    • Typically involves renal and visceral vessels, not pulmonary arteries.
    • Immune complex mediated.
    • Transmural inflammation of the arterial wall with fibrinoid necrosis.
    • Innumerable microaneurysms [C] and spasm on arteriogram.
  • Treatment
    • Treat with corticosteroids, cyclophosphamide.

20

Kawasaki disease

  • Type of condition
  • Epidemiology / Presentation
  • Pathology / Labs
  • Treatment

  • Type of condition
    • Medium-vessel vasculitis
  • Epidemiology / Presentation
    • Asian children < 4 years old.
    • Fever, cervical lymphadenitis, conjunctival injection, changes in lips/oral mucosa (“strawberry tongue” [D]), hand-foot erythema, desquamating rash.
  • Pathology / Labs
    • May develop coronary artery aneurysms [E], thrombosis Ž--> MI, rupture.
  • Treatment
    • Treat with IV immunoglobulin and aspirin.

21

Buerger disease (thromboangiitis obliterans)

  • Type of condition
  • Epidemiology / Presentation
  • Pathology / Labs
  • Treatment

  • Type of condition
    • Medium-vessel vasculitis
  • Epidemiology / Presentation
    • Heavy smokers, males < 40 years old.
    • Intermittent claudication may lead to gangrene, autoamputation of digits, superficial nodular phlebitis.
    • Raynaud phenomenon is often present.
  • Pathology / Labs
    • Segmental thrombosing vasculitis.
  • Treatment
    • Treat with smoking cessation.

22

Granulomatosis with polyangiitis (Wegener)

  • Type of condition
  • Epidemiology / Presentation
  • Pathology / Labs
  • Treatment

  • Type of condition
    • Small-vessel vasculitis
  • Epidemiology / Presentation
    • Upper respiratory tract: perforation of nasal septum, chronic sinusitis, otitis media, mastoiditis.
    • Lower respiratory tract: hemoptysis, cough, dyspnea.
    • Renal: hematuria, red cell casts.
  • Pathology / Labs
    • Triad:
      • Focal necrotizing vasculitis
      • Necrotizing granulomas in the lung and upper airway
      • ƒƒNecrotizing glomerulonephritis.
    • PR3-ANCA/c-ANCA [F] (anti-proteinase 3).
    • CXR: large nodular densities.
  • Treatment
    • Treat with cyclophosphamide, corticosteroids.

23

Microscopic polyangiitis

  • Type of condition
  • Epidemiology / Presentation
  • Pathology / Labs
  • Treatment

  • Type of condition
    • Small-vessel vasculitis
  • Epidemiology / Presentation
    • Necrotizing vasculitis commonly involving lung, kidneys, and skin with pauci-immune glomerulonephritis and palpable purpura.
    • Presentation similar to granulomatosis with polyangiitis but without nasopharyngeal involvement.
  • Pathology / Labs
    • No granulomas.
    • MPO-ANCA/p-ANCA [G] (anti-myeloperoxidase).
  • Treatment
    • Treat with cyclophosphamide and corticosteroids.

24

Churg-Strauss syndrome

  • Type of condition
  • Epidemiology / Presentation
  • Pathology / Labs

  • Type of condition
    • Small-vessel vasculitis
  • Epidemiology / Presentation
    • Asthma, sinusitis, palpable purpura, peripheral neuropathy (e.g., wrist/foot drop).
    • Can also involve heart, GI, kidneys (pauciimmune glomerulonephritis).
  • Pathology / Labs
    • Granulomatous, necrotizing vasculitis with eosinophilia [H].
    • MPO-ANCA/p-ANCA, increased IgE level.

25

Henoch-Schönlein purpura

  • Type of condition
  • Epidemiology / Presentation
  • Pathology / Labs

  • Type of condition
    • Small-vessel vasculitis
  • Epidemiology / Presentation
    • Most common childhood systemic vasculitis.
    • Often follows URI.
    • Classic triad:
      • Skin: palpable purpura on buttocks/legs [I]
      • Arthralgias
      • GI: abdominal pain, melena, multiple lesions of same age
  • Pathology / Labs
    • Vasculitis 2° to IgA complex deposition.
    • Associated with IgA nephropathy.

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