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54 Cards in this Set
- Front
- Back
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cardiomyopathy def and diagnosis
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-abnormality in the myocardium
-primary or secondary to systemic disease (amyloidosis, sarcoidosis, hemochromatosis) -diagnose via endocardial biopsy |
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3 patterns of cardiomyopathy
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-Dilated CM = 90%
-Hypertrophic CM -Restrictive CM |
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DCM def
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-progressive cardiac dilatation and contractile (systolic) dysfunction of both ventricles
-usually with concomitant hypertrophy and eventually heart failure |
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DCM pathology
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-genetic (20-50%): mostly A/D, x-linked dystrophin, mitoch genes involved with oxidative phosphorylation and fatty acid b-oxidation
-infection (coxsackie B) -alcohol/toxins -iron overload |
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alcohol and DCM
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-direct toxic effect on myocardium
-assoc with thiamine deficiency: lends to beriberi heart disease |
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peripartum cardiomyopathy
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-DCM seen late in gestation up to several weeks/months post-partum
-multifactorial: pregnancy-assoc HTN, vol overload, gestational diabetes, immunologic responses |
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DCM gross morphology
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-four chamber dilatation
-normal or hypertrophied wall thickness -flabby -mural thrombi -valves normal but may see regurg due to dilation of chamber = functional regurgitation |
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DCM histology
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-changes are not specific for DCM
-except for iron overload-- stain with prussian blue -some fibers stretched/irreg -no necrosis -hypertrophy and fibrosis are usual |
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DCM clinical features
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-age 20-50
-dyspnea, easy fatigue, poor exertional capacity -low ejection capacity dt ineffective contraction -global hypokinesia on ECG |
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DCM death
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-50% of pts die in 2 years
-25% survive longer than 5 yrs -death dt cardiac failure, arrhythmia or thromboembolic complications |
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DCM treatment
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-cardiac transplant
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arrhythmogenic RV cardiomyopathy/dysplasia
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-transmural fibrofatty replacement of RV myocardium
-seen in young adults -arrhythmias (V tachy) and sudden death -RV failure |
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arrhythmogenic RV cardiomyopathy/dysplasia morphology
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-thin and dilated RV wall with fatty infiltration and interstitial fibrosis
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HCM is characterized by
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-myocardial hypertrophy
-abnormal diastolic filling (restriction of ventricular filling) -intermittent LV outflow obstruction (1/3) |
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HCM gross morphology
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-disproportionate thickening of septum, esp in subaortic region
-no ventricular dilation -atrium enlarged -endocardial thickening and mural plaques in outflow track |
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why do you see endocardial thickening and mural plaques in outflow tract in HCM
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-due to valve contacting septum during contraction
-functional outflow tract obstruction |
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HCM histology
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-marked hypertrophy
-interstitial fibrosis -myofiber disarray |
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HCM pathogenesis
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-ALL genetic-- A/D
-mutations inc myofilament activation resulting in myocyte hypercontractability -B myosin heavy chain= most common mutation |
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HCM clinical features
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-presents after puberty
-asymptomatic -exertional dyspnea -syncope (LV outflow obstruction) -sudden death in young athletes -a fib with mural thrombus formation |
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HCm treatment
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-surgical excision
-ventricular relaxing drugs |
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restrictive cardiomyopathy
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-primary dec in compliance, resulting in impaired ventricular filling during diastole
-idiopathic or secondary to post-radiation fibrosis, amyloidosis, sarcoidosis, metastases, inborn errors of metabolism |
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RCM gross morphology
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-normal ventricles
-bi-atrial dilation -firm myocardium and interstitial fibrosis |
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RCM histology
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-patchy or diffuse
-interstitial fibrosis -disease-specific changes |
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senile cardiac amyloidosis
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-most common cause of RCM
-transthyretin (prealbumin) deposits in ventricles and atria ->60 yo, AA vs caucasian -gene mutations in 4% |
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amyloidosis morphology/histology
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-firm to rubbery
-interstitial deposition -birefringence under polarized light |
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RCM clinical features
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-CHF (R and L)
-severe pulm congestion -hepatic congestion -similar to constrictive pericarditis -diagnose via endomyocardial biopsy |
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endomyocardial fibrosis
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-rare cause of RCM, seen in African kids
-fibrosis of ventricular endocardium and subendocardium --> diminshed vol and compliance --> restrictive; mural thrombi |
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Loeffler eosinophilic endomyocarditis
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-rare cause of RCM
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etiology of myocarditis
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-viral (coxsackie A and B, CMV, HIV)
-protozoa (trypanosoma cruzii-- Chagas, trichinosis) -bact (diptheria, Lyme) -hypersensitivity -systemic autoimmune |
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myocarditis morphology
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-normal or dilated
-flabby -mottled -mural thrombi may be present |
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myocarditis histology
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-commonly myocyte necrosis and interstitial infiltrate of lymphocytes
-hypersensitive myocarditis-- eosinophils (think DRUGS) -parasites in Chagas |
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myocarditis clinical features
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-variable
-asymptomatic, abrupt onset of heart failure, arrhythmias, sudden death -can resolve wo/premanent sequelae or cause devel of dilated CM years later |
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doxorubicin and myocardial disease
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-dose-dep myofiber toxicity
-lipid perox of myofiber membrane, myofiber swelling and vacuolization, fatty change, myocytolysis |
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catecholamines and myocardial disease
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-pheochromacytoma, dopamine, cocaine
-cause Ca2+ overload directly damaging myocytes -vasoconstriction in heart causing ischemia |
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iron overload in heart
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-caused by hereditary hemochromatosis or multiple transfusions
-causes systolic dysfunction dt interference with metal dependent enzymes |
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morph/histology of iron overload
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-gross: similar to DCM, plus rust-brown color
-iron accum in myocytes--> prussian blue stain |
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fluid accumulation
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-normally ~30mL in pericardium
-rapid--> cardiac tamponade (200-300mL) -slow: up to 500mL may be tolerated |
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hydropericardium
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-CHF
-nephrotic syndrome -chronic liver disease |
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hemopericardium
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-ruptured MI
-trauma -ruptured aortic dissection |
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acute pericarditis clinical features
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-pericardial pain
-friction rub -ECG changes (diffuse ST elevations) -distant heart sounds |
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acute pericarditis causes
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-idiopathic
-primary-- usually viral -secondary-- from bact, TB, fungi, rheumatic fever, SLE, post-MI |
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serous acute pericarditis
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-non-infectious inflamm
-rheumatic fever, SLE, scleroderma, tumors, uremia -protein rich fluid with small numbers of mixed inflamm cells |
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fibrinous/serofibrinous acute pericarditis
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-most freq
-fibrin-rich exudates, lymphocytes -loud pericardial friction rub -from viral infections, acute MI, post-infarct (Dressler), uremia |
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purulent/suppurative acute pericarditis
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-almost always bact (direct extension, seeding, lymphatic extension, direct introduction)
-grossly cloudy or purulent inflamm exudate (pus) -can cause fibrosis and constrictive pericarditis |
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hemorrhagic pericarditis
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-malignancy
-TB |
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chronic pericarditis
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-plaque-like fibrous thickening of serosal membranes (soldier's plaque) or inconspicuous mild adhesions (adhesive pericarditis)
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adhesive mediastinopericarditis
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-suppurative or caseous
-cardiac surgery, radiation -increased work load --> cardiac hypertrophy and dilatation= DCM-like |
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constrictive pericarditis
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-obliteration of pericardial sac
-limitation of diastolic filling resembling restrictive CM and R heart failure -from idiopathy, post radiation/infection/surgery |
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metastatic heart tumors
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-more common than primary neoplasms
-from lung, lymphomas, breast, leukemias -may be diffuse, multinodular, or single mass -occurs in 5% of pts dying of cancer |
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primary heart neoplasms
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-most are benign
-myxoma (90% in atria, L:R=4:1) -sessile or pedunculated (ball-valve obstruction) -myoma cells embedded in abundant ground substance -10% have Carney syndrome |
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rhabdomyoma
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-most common heart tumor in kids
-hamartomas or malformations but in bad place -assoicated with tuberous sclerosis -obstructive symptoms, CHF |
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rhabdomyomas morphology
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-usually multiple firm, white nodules
-spider cells (altered myocytes with vacuolation) |
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cardiac transplantation indications
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-dilated CM
-end-stage ischemic heart disease -congenital heart disease -valvular heart disease |
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graft arteriopathy
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-low grade chronic rejection
-intimal proliferation of coronary arteries -~50% of transplant pts have in 10 years -can lead to silent MI/CHF/arryth/sudden death |
