Cardiovascular Flashcards
Modifiable and non-modifiable risk factors for atherosclerosis
Modifiable: diet, exercise, smoking, dental hygiene, homocystinemia, T2DM
Nonmodifiable: age, sex, FHx, genetic predisposition, T1DM, coagulation anomalies, primary HTN
Development of atherosclerosis
Response to injury hypothesis:
-Endothelial injury causing increased vascular permeability, leukocyte adhesion, thrombosis
- accumulation of lipoprotein in vessel wall
- monocyte adhesion to the endothelium followed by migration into intima, and transformation into macrophages and foam cells
- platelet adhesion
- factors released for smooth muscle cell and macrophage recruitment
- smooth muscle cell proliferation and extracellular matric production, lipid accumulation extracellularly and intracellularly
- inflammatory cell infiltration
- metalloproteinase release
- plaque complication - cap rupture, thrombus adherence, possible stenosis or embolism
List at least 5 benign vascular neoplasms
Hemangioma
Angiofibroma
Lobular capillary hemangioma
Glomus tumor
Lymphangioma
List examples of immune complex vasculitis
SLE vasculitis
IgA HSP
Cryoglobulin vasculitis
Drug hypersensitivity
Rheumatoid vasculitis
Classification of vasculitidies and examples in each category
Chapel-Hill classification
Large vessel: GCA, takayasu arteritis
Medium: polyarteritis nodosa, kawasaki disease
Small: microscopic polyangiitis, GPA, EGPA, HSP
Etiology, cardiac sequelae, gross characteristics, microscopic features of: Mitral stenosis
Rheumatic heart disease
Left atrial enlargement and atrial fib
Leaflet thickening and commisural fusion. shortening, thickening and fusion of chordae tendonae
Diffuse fibrosis and neovascularization
Etiology, cardiac sequalae, gross characteristics, microscopic features of: Mitral regurg
Mitral valve prolapse
Left atrial enlargment, LVH, atrial fib
Hooding of mitral leaflets, enlarged thickened and redundant leaflets, chordae elongated thinned and occasionally ruptured
Attenuation of fibrosa, marked thickening of spongiosa with myxomatous material
Etiology, cardiac sequalae, gross characteristics, microscopic features of: Aortic stenosis
Senile calification
LVH
Calcification within cusps
Fibrosis and calcs
Etiology, cardiac sequalae, gross characteristics, microscopic features of: Aortic regurg
Aortic dilatation
LVH, dilated aorta
Cusps from near normal to thickened and calcified with commissural fusion
Near normal to fibrosis, calcification
Common causes of failure of cardiac valve prostheses
Thrombosis/thromboembolism
Prosthetic valve endocarditis
Structural deterioration: wear, frature, cuspal tear, calcs
Nonstructural dysfunction: granulation tissue, suture or tissue entrapment, paravalvular leak
Characteristic features of cardiac lesions of rheumatic fever
Acute rheumatic fever:
- diffuse pancarditis and aschoff bodies
- verrucae : vegetations due to fibrinoid necrosis within cusps or tendinous cords
-MacCallum plaques: subendocardial lesions, exacerbated by regurgitant jets, can induce irregular thickenings in left atrium
Chronic rheumatic fever:
-valvular leaflet or cusp thickening
- commissural fusion
- shorted, fused, thickened chordae tendonae, fish mouth mitral valve orifice
Borderline myocarditis vs active myocarditis
Borderline: inflammation but no myocyte necrosis
Active: inflammation and myocyte necrosis
What to do and what to recommend with a diagnosis of borderline myocarditis
Do: cute through block to look for missed myocyte necrosis, stain adjacent sections
Recommend: repeat or follow up biopsy
Features of endomyocardial biopsies to differentiate dilated, hypertrophic, restrictive cardiomyopathies
None: all show fibrosis and hypertrophy
Disarray can be a normal finding for endomyocardial right ventricular biopsies and thus not specific for hypertrophic cardiomyopathy
Man causes of aortic valve insufficiency
Valve: infective endocarditis, rheumatic valvular disease, congenital bicuspid aortic valce
Aorta: aortic aneurysm, annular dilation, aortic dissection, aortitis
Main causes of aortic valve stenosis
Age related degeneration: calcific degeneration
Congenital bicuspid valve
Postinflammatory disease: rheumatic valve disease
Components of mitral valve apparatus necessary for valve competence
Annulus
Leaflets
Chordae
Left venticular papillary muscles
Left ventricle myocardium
How to distinguish tricuspid and mitral valves
Septal attachments present on tricuspid valve
Tricuspid and pulmonary valves due to infuldibular spetum
Mitral valve has higher point of insertion on ventricular septum
Mitral as 2 leaflets, tricuspid has 3
Components of tetrology of fallot
VSD
Pulmonary stenosis
Overriding aorta
RVH
Systemic or cardiac conditions associated with aortic dissection
Bicuspid aortic valve
Systemic arterial hypertension
Trauma - iatrogenic at surgery, iatrogenic at cath, nonpenetrating blunt chest injury
Pregnancy
Connective tissue disease
Giant cell aortitis (really any aortitis)
Complications of acute aortic dissection
MI
Coronary artery dissection
Aortic rupture
Aortic valve insufficiency
Stroke
Visceral ischemia
Hemopericardium and cardiac tamponade
Complications of atherosclerotic plaques
Thrombosis
Plaque rupture
Plaque hemorrhage
Aneurysm
Embolism (thrombus and athromatous debris)
Plaque erosion
Vessel rupture
Workup of unexpected giant cell inflammation in aortic aneurysm resection
History - check for infections (TB, syphilis), cultures, serology etc, any systemic inflammatory disease
Call clinician to report critical value
Stunned myocardium vs hibernating myocardium
Stunned: post MI, non-contractile, reversible with time
Hibernating: chronic ischemia related (myocytolysis), noncontractile, reversible with revascularization
Molecular testing at autopsy includes testing for which conditions
Hypertrophic cardiomyopathy
Arrhythmogenic cardiomyopathy
QT segment - long or short
Brugada syndrome
Catecholaminergic polymorphic ventricular tachycardia (CPVT)
Causes of constrictive pericarditis
Radiation
TB
Cardiac surgery
Tumor involvement - primary or secondary
Clinical signs/symptoms of pericarditis
Chest pain, dyspnea, fever/chills, friction rub, pericardial effusion, ST elevation, decreased QRS amplitude
Syndrome of pericarditis presenting late after an MI
Dressler syndrome
Causes of fibrinous pericarditis
Viral
Drugs
Uremia
Collagen vascular disease
Trauma
Tumor
MI
Clinical manifestations of carcinoid heart disease
Valvular heart disease: tricuspid regurg, pulmonary regurg, pulmonary stenosis
Coronary artery vasospasm
Arrhythmias
Carcinoid tumor mets directly in myocardium
MI
Why is right sided heart failure more common in carcinoid heart diease
Left side is spared because lungs metabolize vasoactive substances
Characteristic pathological findings of carcinoid heart disease
Endocardial plaques - RV, tricuspid valve, pulmonary valve, occasionally vena cava, pulmonary artery, coronary sinus
Complications of bioprosthetic and mechanical prosthetic valves
Both:
- Size mismatch
- incorrect positioning
- Suture impingement on disc
- paravalvular leak
- coronary artery ostial occlusion by valve ring
- infective endocarditis
- fibrous pannus
Bioprostheses:
- leaflet degenerative changes with fibrosis and calcification
- leaflet tears and perforation
Mechanical:
- cloth wear
- ball or disc emoboli or erosion
- ball degeneration and cracking
- stent creep
- thrombosis
Regions requiring thorough examination in assessing a coronary artery bypass graft
Bypass graft ostium anastomosis at aorta
Bypass graft body
Distal anastomosis
Distal coronary artery
proximal native coronary artery
Coronary artery ostium
Key features of giant cell arteritis
Vasculitis involving the temporal artery and aorta
FOund in older patients with headaches, fatigues, fever, vision loss, etc
Associated with PMR
Ophthalmic artery involve may result in irreversible blindness
Histologic features of giant cell arteritis
Granulomatous inflammation with destruction of internal elastic lamina
Mixed inflammatory infiltrate consisting of lymphocytes, eos and histiocytes
Intimal proliferation
Medial fibrosis
Healed stage: collagenous scar and neovascularization of vessel wall
Processing of temporal artery biopsy specimens
Submit entire segment for processing and sectioned at the time of embedding
Serially section at 2-3mm, submitted in toto
Multiple levels +/- elastin stains
Clinical significance of negative temporal artery biopsy
Negative biopsies do not rule out this disease as it is patchy
Treatment decisions should be made knowing this
Clinical features of mitral valve prolapse
Most commonly in young women
Often incidental finding of midsystolic click
Associated with marfan syndrome
Complications include infective endocarditis, mitral insufficiency, ventricular arrhythmia
Pathologic features of mitral valve prolapse
Interchordal ballooning (hooding) of mitral leaflets
enlarged redundant, thick, and rubbery leaflets
Elongated and thinned tendinous cords
Annular dilatation
Microscopic features - attenuated fibrosa layer of valve and thickened sponsiosa layer with myxomatous degeneration
Clinical complications of mitral valve prolapse
Mitral valve regurg
Arrhythmias
Endocarditis
Chordae tendinae rupture
Embolism
Pathogenesis of mitral valve prolapse
Degenerative/myxomatous disease
Clinical features of angiosarcoma
Often in older adults, may be secondary to radiation therapy
Most commonly in skin, soft tissue, breast, liver
Characterized by rapid growth, leading to ulcers and hemorrhage
Histologic features of angiosarcoma
Anastomosing vascular channels lined by atypical endothelial cells with frequent mitotic figures and necrosis
Degrees of differentiation from obviously vascular to undifferentiated
3 causes of angiosarcoma
Vinyl chloride
Radiation
Lymphedema
IHC profile of angiosarcoma
CD31+ CD34+ FXIII+
Classification of endocarditis
Infective endocarditis:
- native valve endocarditis
- prosthetic valve endocarditis, early and late
- Intravenous drug abuse endocarditis
Noninfective:
- nonbacterial thrombotic endocarditis
- endocarditis of SLE (Libman-Sacks)
-verrucous endocarditis
Gross and microscopic features of Acute infective endocarditis
Gross - bulky, irregular, friable vegetation on valve cusps; valve destruction; abscess
Micro - fibrin, inflammatory cells, bacteria or other organisms
Gross and microscopic features of Healing infective endocarditis
Gross - smaller, friable vegetations
Micro - granulation tissue at base of vegetations, chronic inflammatory cells
Gross and microscopic features of acute rheumatic valvular disease
Gross - row of small, warty vegetations along the lines of closure of valve
Micro - Aschoff bodies, Anitschkow cells, fibrin, inflammatory cells
Gross and microscopic features of NBTE
Gross - small, bland vegetations, usually attached at the line of valve closure
Micro - bland thrombus with no inflammatory reaction or valve damage
Gross and microscopic features of Libman-Sacks endocarditis
Gross - small or medium-size vegetations of either or both sides of valve
Micro - Fibrin, cellular debris, inflammatory cells without PMNs
Predisposing conditions to infective endocarditis
Rheumatic heart disease
Myxomatous mitral valve
Bicuspid aortic valve
Prosthetic valve
Gross evaluation of aortic valve
Assess number of cusps, size, and consistency
Describe any abnormalities - distribution, surface, location
Describe any vegetations
Submit representation sections taken from free edge to annulus
Pertinent findings in an autopsy patient with infective endocarditis
Vegetations on aortic and mitral valve
Vegetations on pulmonary and tricuspid valve for IVDU
Any valve abnormalities eg perforation, indentation, rupture chordae
Ring abscess
Septic emboli
Risk factors for SLE
Young women
Ethnicity: African-Americans, Hispanics, Asians
FHx
Possible SLE triggers
Cold temperature
Fatigue
Stress
Chemicals exposures
Sunlight
Certain drugs
Cardiac manifestations of SLE
Pericarditis
Myocarditis
Libman-Sacks endocarditis
Systemic associations with berry aneurysms
ADPKD
Systemic arterial hypertension
Atherosclerosis
Histologic features of berry aneurysms
Deficiency of elastic and muscled tissues with dilatation of vessel wall
Arterial wall adjacent to neck of aneurysm shows intimal thickening and attenuation of media
Smooth muscle and intimal elastic lamina do not extend into the neck and are absent from aneurysm sac itself
Common causes of SAH
Head trauma
Rupture of saccular aneurysm
Vascular malformations
Tumors
Clinical findings of GPA
Persistent pneumonitis with bilateral nodular and cavitary infiltrates
Chronic sinusitis, mucosal ulceration of nasopharynx
Renal disease - hematuria and proteinuria
Histologic features of GPA
Acute necrotizing granulomata in resp tract
Necrotizing or granulomatous vasculitis affecting small to medium-sized vessels
Renal: focal necrotizing and crescentic GN
Common site involved in GPA
Upper and lower respiratory tract
Kidney
Peripheral vessels
ANCA subtype associated with GPA
PR3-ANCA
Pathogenetic causes of GPA
Hypersensitivity
Immune complexes
DDx of GPA
Sarcoidosis
polyarteritis nodosa
Clinical features of EGPA
Associated with asthma, allergic rhinitis, lung infiltrates, peripheral hypereosinophilia
Skin lesions - urticaria and palpable purpura
Young adults
MPO-ANCA
May present with GI bleeding
Renal disease - FSGS
Cardiomyopathy - half of syndrome-related deaths
histologic findings of EGPA
Small sized arteries and veins
Necrotizing vasculitis with eosinophilic infiltrate and granulomatous reaction
Define sudden cardiac death
Unexpected death from cardiac causes that occurs without symptoms or within 1-24h of symptom onset
Causes of SCD
Ischemic heart disease (80-90%)
Congenital structural or coronary arterial abnormalities
Aortic valve stenosis
Mitral valve prolapse
Myocarditis
Dilated or hypertrophic cardiomyopathy
Pulmonary hypertension
Arrhythmia
Isolated LVH
When should conduction system examination be done
Sudden death with normal heart examination
Cardiomyopathies
AV block
Arrhythmias with condition system anomalies
Sarcoidosis
Conduction problems post aortic valvular surgery
Post cardiac ablation
Signs at autopsy reflecting heart failure
Right:
- Acites
- hepatosplenomegaly
- peripheral efema
- stasis dermatitis
Left:
- pleural effusion
- heavy lungs
- pulmonary edema
Classification of Kaposi sarcoma
Classic: eastern European and mediterranean males
Endemic: people indigenous to central Africa
Epidemic: HIV+
Iatrogenic/Immunocompromised - transplant
Clinical features of kaposi sarcoma
Erythematous to violaceous cutaneous lesions (macular, patch, plaque, nodular, exophytic), can be solitary, localized, or disseminated
Can involve oral cavity, lymph nodes, viscera
Histologic features of kaposi sarcoma
Slit-like space, extravasated RBCs, plasma cells
Proloeration of spindle shaped cells arranged as short fascicles and diffuse proliferation of blood vessels
Later stage: greater degrees of cytological atypia, high mitotic rate
Cause of Kaposi sarcoma
HHV8
DDx of kaposi sarcoma
Benign vascular proliferations: targetoid hemosiderotic hemangioma, fibrous histocytoma
Angiosarcoma
Intracardiac complications of endocarditis
Valvular destruction
Paravalvular destruction/abscess
Valvular incompetence
Sinus of valsalva aneurysm
Common causative organisms of endocarditis in IVDU
S. Aureus
Staph epidermidis
Poor prognostic features of endocarditis
Acute S. auerus endocarditis
Heart failure
IVDU (often bilateral disease)
Prosthetic valve infection
Fungal endocarditis
COmplications with or root abscesses or fistulas
Common causes of aortic dissection
Inherited syndromes - Marfan, Loey-Dietz, Ehlers-Danlos
Systemic arterial hypertension
Trauma
Infection
Pathogenesis of aortic dissection
Occur when a tar of intima of aortic wall causes dissection of blood between and along the laminar planes of the media
Classifications of Aortic dissection
Stanford:
- Type A involved either ascending only or ascending and descending
- Type B descending only
DeBakey:
- I involved ascending and rest of aorta
- II involves ascending aorta only
- III arises after ascending aorta
Clinical features of aortic dissection
Severe pain
Dyspnea
Limb pain 2’ ischemia
Organ/visceral pain 2’ infarction
Stroke signs
Effects of chronic systemic arterial hypertension on heart, large vessels, and small vessels
Heart: LVH, cardiac myocyte hypertrophy, interstitial myocardial fibrosis
Large vessels: increased atherosclerosis, aortic aneurysms and dissection
Small vessels: retinopathy, nephropathy, nephrosclerosis, cerebral infarct, hemorrhage
Types of ANCA and their associated conditions
PR3-ANCA: GPA
MPO-ANCA: microscopic polyangiitis, ANCA-associated GN, EGPA
Also associated with UC, ank spon
Define ANCA
Antineutrophil cytoplasmic antibody
Acts against antigens in cytoplasm of neutrophil granulocytes and monocytes
Mostly IgG
Particular association with systemic vasculitis
2 common subtypes of ANCA
MPO-ANCA and PR3-ANCA
MPO-ANCA: myeloperoxidase, perinuclear staining
PR3-ANCA: proteinase 3, diffuse, granular cytoplasmic
Types of vessels most at risk for ANCA-associated vasculitis
Small arteries
Arterioles
Venules
Veins
Capillaries
Rarely large elastic arteries
How is ANCA measured
ELISA and direct IF
Pathogenesis of noninfectious vasculitis
Main pathogenesis is immune complex deposition, ANCA, Antiendothelial cell antibodies
5 Immune complex vasculitidies
Cryoglobulinemic vasculitis: cryglobulins (association with HepC)
Hypersensitivity vasculitis/leukocytoclastic: meds/drug
HSP: IgA-complexes
Lupus: Full house
Rheumatoid vasculitis
Most common primary cardiac neoplasms in adults
Myxoma
Papillary fibroelastoma
Rhabdomyoma
Most common primary cardiac neoplasms in children
Rhabdomyoma
Fibroma
Sarcoma
Origin of myxoma tumor cells
Primitive multipotent mesenchymal cells
Most common location for cardiac myxoma
Left atrial septum near fossa ovalis
Gross and microscopic features of cardiac myxoma
Gross: soft, gelatinous, papillary to firm, smooth
- cut surface variegated with areas of hemorrhage and degeneration, calcified changes
Microscopic: myxoma cells are stellate, ovoid, or polygonal with inconspicuous nucleoli, eosinophilic cytoplasm and indistinct cell borders
- cells may form rings, ribbons, glandular structures, and cords
- background of myxoid and loose fibrous tissue with scatters lymphs, hemosidering-laden macrophages, and a capillary network
IHC for cardiac myxoma
CD34+ Calretinin+
Classification of primary cardiomyopathies
Dilated
Hypertrophic
Restrictive
Etiology, functional impact, gross and microscopic features of dilated cardiomyopathy
Etiology: 30-40% gene mutations, other causes include myocarditis, toxic, peripatum, idiopathic
Functional impact: ventricular systolic dysfunction
Gross: 4 chamber dilation, bilateral ventricular hypertrophy or thinning, mural thrombi, endocardial fibrosis
Micro: variable myocyte hypertrophy, interstitial and endocardial fibrosis
Etiology, functional impact, gross and microscopic features of hypertrophic cardiomyopathy
Etiology: 100% gene mutations in sarcomere, AD inheritance
Function: diastolic dysfunction
Gross: LVH, banana-like config of left ventricular cavity, asymmetrical septal thickening, endocardial thicking of aortic outflow tract and anterior mitral leaflet
Micro: extensive myocyte hypertrophy, haphazard disarray of bundles, myocytes, and fibres, interstitial fibrosis
Etiology, functional impact, gross and microscopic features of restrictive cardiomyopathy
Etiology: amyloid, sarcoid, endomyocardial fibrosis, loeffler endomyocarditis, endocardial fibroelastosis, radiation, metabolic diseases, idiopathic
Function: diastolic dysfunction
Gross:
- amyloid: firm, rubbery, thick walls
- sarcoid: firm, parenchymal scarring/fibrosis
Micro: appearance of amyloid, sarcoid, or interstitial fibrosis
Morphologic feature of contraction band necrosis
Eosinophilic bands crossing the short axis of the myocyte
Pathogenesis of contraction band necrosis
Irreversible myocardial injury
May occur in setting of reperfusion injury
Mediated by fluctuations in calcium concentration causing sarcomere hypercontraction
Can be an artifact seen in endomyocardial biopsy specimen
Role of endomyocardial biopsy
Gold standard for surveillance of cardiac transplant rejection and for diagnosing myocarditis
Helpful for diagnosis or monitoring of primary cardiomyopathies, amyloidosis, sarcoidosis, drug toxicities, fabry disease, endocardial fibrosis, neoplasia
Approach to endomyocardial biopsy
Requires clinical history
Adequacy: at lest 4 good pieces of myocardium
Evaluation of endocardium, myocardium, interstitium, vasculature
Report should include biopsy site, type of biopsy, diagnosis, grade, microscopic description
Characteristic findings of arterial and venous diseases of legs
Arterial disease: deep distinct ulcers, gangrene, muscular atrophy, hair loss, toenail thickening, mottling
Venous disease: congestion, varices, shallow medial malleolar ulcer, stasis dermatitis with skin flaking and brownish discolouration
Clinical manifestations of cardiac tamponade
Decreased heart sounds, decreased pulse, increased JVP
Differentiate constrictive pericarditis and restrictive cardiomyopathy on endomyocardial biopsy
Constrictive pericarditis: atrophic cardiac myocytes or normal myocardium
Restrictive cardiomyopathy: fibrosis, myocyte hypertrophy, degeneration
What kind of calcification is calcific aortic stenosis
Dystrophic calcs - occurs in degeneration or necrotic tissue
Active calcification with cholesterol, inflammation, fibrosis
Examples of dystrophic calcification
Leiomyoma
Calcification of postinfarct ventricular aneurysm
Hyalinized scar
Tumor necorsis
Old granuloma
Fat necrosis
Gross features of rheumatic heart disease
Acute - small verrucous vegetations along lines of valve closure
MacCallum patch - thickening of left atrial endocardium proximal to base to posterior mitral valve leaflet
Leaflets thickened with commissural fusion
Chordae thickened, shortened, fused
Fibrosis and calcs create fish mouth orifice of mitral valve
Elements to report with malignant cardiac tumor
Specimen procedure
Specimen integrity
Specimen laterality
Tumor site
Tumor size
Histologic type and grade
Tumor extension
Margins
Treatment effect
LVI
Grading of cardiac tumors
Mostly sarcomas so FNCLCC used:
Tumor diff:
- Score 1: sarcomas closely resembling normal adult mesenchymal tissue
- Score 2: sarcomas for which histologic typing is certain
- Score 3: undifferentiated, angiosarcoma
Mitosis:
- Score 1: 0-9/10 hpf
- Score 2: 10-19/10 hpf
- Score 3: >19/10 hpf
Tumor necrosis:
- Score 0: none
- Score 1: <50% tumor necrosis
- Score 2: >50% tumor necrosis
Histologic grade:
- Grade 1: total score 2-3
- Grade 2: total score 4-5
- Grade 3: total score 6-8
pTNM staging for heart tumors
No published staging system
