Robbins Path 12: The Heart (560-585)

Front 1

stenosis

Back 1

failure of valve to open completely, impeding forward flow (P overload)

Front 2

insufficiency

Back 2

failure of valve to close completely, allowing reverse flow (V overload)

Front 3

functional regurgitation

Back 3

incompetence of a valve stemming from an abnormality in one of its support structures

Front 4

when is functional mitral valve regurgitation common

Back 4

Ischemic mitral regurgitation (IHD)

Front 5

valvular stenosis general cause

Back 5

chronic abnormality that become clinically evident after many years

Front 6

valvular insufficiency general causes

Back 6

intrinsic disease of valve cusps or damage to supporting structures; many causes and can be acute or chronic

Front 7

Major cause of aortic stenosis

Back 7

calcification of anatomically normal and congenitally bicuspid aortic valves

Front 8

major cause of aortic insufficiency

Back 8

dilation of ascending aorta, usually related to hypertension and aging; postinflammatory scarring (rheumatic heart disease)

Front 9

major cause of mitral stenosis

Back 9

rheumatic heart disease

Front 10

major cause of mitral insufficiency

Back 10

myxomatous degeneration (mitral valve prolapse)

Front 11

when do bicuspid valves and senile calcification of aortic valves come to light clinically

Back 11

50-70 and 70-90 respectively

Front 12

what cells are present in aortic calcification

Back 12

cells resembling osteoblasts that synthesize bone matrix proteins and promote deposition of calcium salts

Front 13

where does calcification of valves begin

Back 13

valvular fibrosa, at the points of maximal cusp flexion

Front 14

aortic valve sclerosis

Back 14

hemodynamically inconsequential stage of calcification process

Front 15

congenital bicuspid aortic valve specs

Back 15

1% prevalence; uncomplicated in early life; increased infective endocarditis, aortic stenosis/regurgitation, aortic dilation/dissection, progressive degenerative calcification (raphe major site)

Front 16

affected leaflets in mitral valve prolapse (MVP)

Back 16

enlarged, redundant, thick, and rubbery

Front 17

histology of valve in MVP

Back 17

attenuation of collagenous fibrosa layer, marked thickening of spingiosa layer with deposition of mucoid (myxomatous) material

Front 18

TGF-B and MVP

Back 18

can be prevented by inhibitors of TGF-B in mice studies; cause of MVP in marfans due to fibrillin mutation and TBF-B dysregulation

Front 19

MVP clinically

Back 19

most incidental by detection of midsystolic click

Front 20

3% MVP patients dvlp

Back 20

1) infective endocarditis 2) mitral insufficiency 3) stroke/systemic infarct 4) arrhythmias (ventricular and atrial)

Front 21

aschoff bodies in rheumatic fever

Back 21

foci of lymphocytes (mostly T cells), occasional plasma cells, and plump activated macrophages (Anotschkow cells)

Front 22

Anotschkow cells

Back 22

macrophages with abundant cytoplasm and central round-to ovoid nuclei in which chromatin is disposed in a central, slender, wavy ribbon (catepillar cells), and may be multi-nucleated

Front 23

where can diffuse inflammation and Anotschkow bodies by found during acute RF

Back 23

any of the three layers of the heart causing pericarditis, myocarditis, or endocarditis (pancarditis)

Front 24

verrucae

Back 24

overlye necrotic foci vegetations along lines of closure in RF

Front 25

MacCallum plaques

Back 25

subendocardial lesions, irregular thickenings exacerbated by regurgitant jets, generally in L atrium

Front 26

cardinal anatomic changes in mitral valve of RHD

Back 26

leaflet thickening, commisural fusion and shortening, thickening and fusion of tendinous cords

Front 27

cause of RHD/RF

Back 27

M proteins of streptococci cross-react with self-antigens in the heart; CD4+ T cells react with self proteins in heart and produce cytokines that activate macrophages (antibody and T-cell mediated rxns)

Front 28

RF manifestations

Back 28

1) migratory polyarthritis of large joints 2) pancarditis 3) subQ nodules 4) erythema marginatum of skin 5) Sydenha chorea

Front 29

sydenham chorea

Back 29

neurologic disorder with involuntary rapid, purposeless movements

Front 30

antibodies in RF patients

Back 30

atreptolysin O and Dnase B

Front 31

acute infective endocarditis cause

Back 31

infection of perviously normal heart by highly virulent organism that produces necrotizing, ulcerative, destructive lesions; difficult to cure with antibiotics and usually require surgery

Front 32

subacute endocarditis cause

Back 32

organisms of lower virulence and cause insidious infections of deformed valves that ate less destructive; antibiotics often cure

Front 33

most common cause of native endocarditis (50-60% cases)

Back 33

S. viridans-part of normal flora of oral cavity; generally infect previously injured/congenital abnormal valves

Front 34

S. aureus as cause of endocarditis

Back 34

10-20% cases; healthy or deformed valves; IV drug users

Front 35

remaining organisms that cause endocarditis

Back 35

enterococci and HACEK group (Haemophilis, Actinobacillus, Cardiobacterium, Eikenella, and Kingella)-all in oral cavity; also gram-neg bacteria and fungi

Front 36

prostetic valve endocarditis common cause

Back 36

coagulase-neg staphlococci (S. epidermidis)

Front 37

rate of culture neg endocarditis

Back 37

10-15%

Front 38

hallmark of infectious endocarditis

Back 38

presence of friable, bulky, potentially destructive vegetations containing fibrin, inflammatory cells, and bacteria or other organisms on the heart valves

Front 39

septic infarcts and mycotic aneurysms in endocarditis

Back 39

organisms embolize and cause abscesses in other sites

Front 40

Janeway lesions

Back 40

erythematous or hemorrhagic nontender lesions on the palms or soles

Front 41

Osler nodes

Back 41

subQ nodules in pulp of digits

Front 42

Roth spots

Back 42

retinal hemorrhages in the eyes

Front 43

what causes noninfected vegetations

Back 43

nonbacterial thrombotic endocarditis and endocarditis of systemic lupus erythematous (Libman-Sacks endocarditis)

Front 44

nonbacterial thrombotic endocarditis (NBTE)

Back 44

characterized by deposition of small sterile thrombi on leaflets of cardiac valves; not invasive and do not elicit inflammatory response

Front 45

who does NBTE generally affect

Back 45

debilitated patients (cancer, sepsis)

Front 46

Libman-Sacks endocarditis specs

Back 46

small, sterile vegetations, often with warty appearance; finely granular, fibrinous eosinophilic material that may contain hematoxylin bodies, hemogenous remnats of nuclei damaged by anti-nucleat antigen bodies

Front 47

carcinoid syndrome characteristics

Back 47

episodic flushing of skin, cramps, nausea, vomiting, and diarrhea

Front 48

cardiovascular lesions associated with carcinoid syndrome

Back 48

distinctive, consisting of firm plaquelike endocardial fibrous thickenings on the inside surfaces of the cardiac chamcer and the tricuspid/pulmonary valves; occasionally involve major bvs of R side, inferior VC, and pulmonary artery

Front 49

what are the plaquelike thickenings composed of in carcinoid syndrome

Back 49

predominantly smooth muscle cells and sparse collagen fibers embedded in an acid mucopolysaccharide-rich matrix material; elastic fibers NOT present; structures underlying plaques intact

Front 50

what correlates with severity of right heart lesions in carcinoid syndrome

Back 50

serotonin metabolite 5-hydroxyindoleacetic acid in urine and serotonin plasma levels

Front 51

why don't GI carcinoids cause cardiac lesions

Back 51

venous drainage to liver breaks down bioactive mediators before they reach heart

Front 52

why is only R heart involved in carcinoid syndrome

Back 52

serotonin and bradykinin are inactivated in lungs via monoamine oxidase

Front 53

most common cardiac manifestation of carcinoid syndrome

Back 53

tricuspid insufficiency followed by pulmonary valve insufficiency

Front 54

drugs that cause carcinoid-like effects in L heart

Back 54

fenfluramine, some anti-parkinsonian drugs, methysergide or ergotamine (used in migraine headaches)

Front 55

complications of prosthetic valves

Back 55

thromboembolic, infective, structural, other

Front 56

infective endocarditis of artificial valves-location

Back 56

interface of valve and tissue; often formation of ring abscess

Front 57

what organisms cause endocarditis directly on bioprosthetic valvular cusps

Back 57

Staph (S. epidermidis), S. aureus, strep, and fungi

Front 58

cardiomyopathy

Back 58

heart disease resulting from an abnormality in the myocardium

Front 59

primary cardiomyopathies

Back 59

predominantly confined to the heart muscle

Front 60

secondary cardiomyopathies

Back 60

myocardial involvement as a component of a systemic or multiorgan disorder

Front 61

clinical approach to cardiomyopathies

Back 61

one of 3 clinical, fxnal, and pathological patterns present: 1) dilated cardiomyopathy (DCM) 2) hypertrophic cardiomyopathy (HCM) 3) restrictive cardiomyopathy

Front 62

left ventricular noncompaction

Back 62

distinctive "spongy" appearance of L venricular myocardium; congenital disorder associated with heart failure or arrhythmias

Front 63

what is arrhythmogenic R ventricular dysplasia a variant of

Back 63

DCM

Front 64

characteristics of DCM

Back 64

progressive cardiac dilation and contractile (systolic) dysfunction, usually with concomitant hypertrophy; aka congestive cardiomyopathy

Front 65

coronary arteries in DCM

Back 65

free of significant narrowing or obstructions present insufficient to explain degree of cardiac dysfunction

Front 66

histology of DCM

Back 66

nonspecific; severity of changes doesn't reflect degree of dysfunction or prognosis

Front 67

cause of DCM

Back 67

genetic; acquired myocardial insults or interactions of genetics and environment such as 1) myocarditis 2) toxicities 3) childbirth

Front 68

genetic influences on DCM

Back 68

20-50% cases; autosomal dominant; generally cytoskeletal protein mutations expressed by myocytes

Front 69

mitochondrial defects and DCM

Back 69

most frequently cause DCM in children

Front 70

when does X-linked DCM present

Back 70

teen years or early 20s; rapidly progressive; dystrophin mutation

Front 71

cardiac alpha-actin fxn

Back 71

links sarcomere with dystrophin

Front 72

myocarditis and DCM

Back 72

viral nucleic acids from coxackievirus B and other enteroviruses detected in myocardium of patients with DCM

Front 73

Alcohol and DCM

Back 73

alcohol strongly associated; no morphologic featuers distinguish from other DCM causes

Front 74

thiamine deficiency leads to

Back 74

beriberi heart disease (indishinguishable from DCM)

Front 75

other toxins associated with DCM

Back 75

chemotherapeutic agents like doxorubicin (Adriamycin); cobalt

Front 76

peripartum cardiomyopathy (form of DCM) occurs when

Back 76

late in pregnancy or several months postpartum; multifactoral, poorly understood; relationship to elevated levels of anti-angiogenic cleavage product of hormone prolactin

Front 77

clinical featuers of DCM

Back 77

usually 20-50; slowly progressive signs and symptoms of CHF (SOB, fatigue, poor exertional capacity); 50% die within 2 years, 25% >5 yrs

Front 78

secondary effects of DCM

Back 78

mitral regurg and abnormal cardiac rhythms; embolism

Front 79

arrhythmogenic R ventricular cardiomyopathy/dysplasia (ARVC)

Back 79

inherited disease of cardiac muscle that causes R ventricular failure and various rhythm disturbances; autosomal dominant, variable penetrance

Front 80

R ventricle in ARVC

Back 80

severely thinned due to loss of myocytes, with extensive fatty infiltration and fibrosis

Front 81

mutation in ARVC

Back 81

defective cell adhesion proteins in desmosomes that link adjacent cardiac myocytes

Front 82

Naxos syndrome

Back 82

arrhythmogenic R ventricular cardiomyopathy and hyperkeratosis of plantar palmer skin surfaces; mutation in plakoglobin

Front 83

Hypertrophic cardiomyopathy (HCM) characteristics

Back 83

myocardial hypertrophy, poorly compliant L ventricular myocardium leading to abnormal diastolic filling; in 1/3 intermittent ventricular outflow obstruction; primarily diastolic dysfunction

Front 84

HCM genetics

Back 84

mutations in sarcomeric proteins

Front 85

heart in HCM

Back 85

thick walled, heavy, hypercontracting

Front 86

heart in DCM

Back 86

flabby, hypocontracting

Front 87

2 diseases HCM must be distinguished from

Back 87

deposition diseases (amyloidosis, Fabry's disease) and hypertensive heart coupled with age-related subaortic septal hypertrophy; sometimes valvular or congenital subvalvular aortic stenosis can mimic

Front 88

classic pattern of HCM

Back 88

disproportionate thickening of ventricular septum as compared to free wall of L ventricle (ratio greater than 1:3); most prominent in subaortic region

Front 89

most important histological features of HCM

Back 89

1)extensive myocyte hypertrophy (diameter >40 um, normal 15 um) 2) haphazard disarray of bundles of myocytes, individual myocytes, and contractile elements in sarcomeres 3) interstitial and replacement fibrosis

Front 90

most common mutations in HCM

Back 90

B-myosin heavy chain; cardiac TnT, alpha-tropomyosin, and myosin-binding protein C

Front 91

basic physiologic abnormality in HCM

Back 91

reduced stroke volume due to impaired diastolic filling, resulting in reduced chamber size and compliance of massively hypertrophied L ventricle; 25% dynamic obstruction to L ventricle outflow

Front 92

what does limitation of CO and secondary increase in pulmonary venous P cause

Back 92

exertional dyspnea

Front 93

auscultation of HCM

Back 93

harsh systolic ejection murmur

Front 94

focal myocardial ischemia and HCM

Back 94

common even in absence of coronary artery disease; anginal pain frequent

Front 95

complications of HCM

Back 95

A fib, mural thrombus, intractable cardiac failure, ventricular arrhythmias, sudden death

Front 96

basic HCM problem

Back 96

sarcomere impaired cardiac fxn leading to compensatory hypertrophic response; recently-defect inenergy transfer from source of generation (mitochondria) to site of use (sarcomeres), leading to subcellular energy deficiency

Front 97

DCM basic problem

Back 97

abnormal force generation, transmission, or myocyte signaling due to cytoskeletal protein abnormalities

Front 98

restrictive cardiomyopathy characteristics

Back 98

primary decrease in ventricular compliance, resulting in impaired ventricular filling during diastole; contractile fxn generally unaffected

Front 99

what is commonly observed in restrictive cardiomyopathy

Back 99

biatrial dilation; patchy/diffuse interstitial fibrosis (minimal to extensive)

Front 100

endomyocardial fibrosis

Back 100

disease of children and young adults in Africa and other tropical areas characterized by fibrosis of ventricular endocardium and subendocardium that extends from the apex upward (often involved mitral and tricuspid valves); unknown etiology

Front 101

Loeffler endomyocarditis

Back 101

endomyocardial fibrosis, typically with large mural thrombi; peripheral eosinophilia and eosinophilic infiltrates in other organs

Front 102

endocardial fibroelastosis

Back 102

uncommon heart disease of obscure etiology characterized by focal or diffuse fibroelastic thickening usually involving mural L ventricular endocardium; first 2 yrs of life, accompanied by aortic valve obstruction or other congenital cardiac abnormalities in 1/3 cases

Front 103

myocarditis

Back 103

diverse group of pathologic entities in which infectious microorganisms and/or inflammatory process couse myocardial injury

Front 104

what must myocarditis be differentiated from

Back 104

injuries that cause inflammation secondarily like ischemic heart disease

Front 105

most common cause of myocarditis

Back 105

viral infections-coxsackieviruses A and B and other enteroviruses

Front 106

Chagas disease

Back 106

Trypanosoma cruzi-important cause of infectious myocarditis

Front 107

Trichinosis

Back 107

most common helminthic disease associated with myocarditis

Front 108

lyme myocarditis

Back 108

primarily manifests as self-limited conduction system disorder that frequently required a temporary pacemaker

Front 109

AIDS myocarditis (2 types)

Back 109

1) inflammation and myocyte damage without clear etiologic agent 2) caused by HIV directly or by opportunistic pathogen

Front 110

noninfectious causes of myocarditis

Back 110

hypersensitivity rxns (drugs (antibiotics, diuretics, antihypertensive), systemic disease of immune origin (rheumatic fever, lupus, polymyositis), sarcoidosis, rejection of transplanted heart

Front 111

active phase of myocarditis

Back 111

heart may appear normal or dilated, some hypertrophy depending on duration

Front 112

advanced stages of myocarditis

Back 112

ventricular myocardium flabby and often mottled by wither pale foci or minute hemorrhagic lesions

Front 113

infiltrate in active myocarditis

Back 113

interstitial inflammatory infiltrate associated with fical myocyte necrosis; diffuse, mononuclear, predominately lymphocytic most common

Front 114

hypersensitivity myocarditis histo

Back 114

perivascular infiltrates composed of lymphocytes, macrophages, and high proportion of eosinophils

Front 115

giant-cell myocarditis histo

Back 115

wide-spread inflammatory cellular infiltrate containing multinucleate giant cells interspersed with lymphocytes, eosinophils, plasma cells, and macrophages; poor prognosis

Front 116

Chagas disease histo

Back 116

parasitization of scattered myofibrils by trypanosomes accompanied by inflammatory infiltrate of neutrophils, lymphocytes, macrophages, and occasional eosinophils

Front 117

anthracycline cardiotoxicity (chemo agent) mechanism

Back 117

peroxidation of lipids in myocyte membranes

Front 118

drug cardiomyopathy general findings

Back 118

myofibril swelling, cytoplasmic vacuolization, and fatty change

Front 119

pheochromocytoma (catecholamine) cardiomyopathy

Back 119

foci of myocardial necrosis with contraction bands, often sparse mononuclear inflammatory infiltrate consisting of macrophages

Front 120

Takotsubo cardiomyopathy

Back 120

sudden, intense emotional or physical stress induce acute L ventricular dysfxn due to myocardial stunning

Front 121

mechanism proposed for catecholamine heart effects

Back 121

direct or via calcium overload or focal vasoconstriction in coronary arterial macro/microcirculation in face of increased HR

Front 122

amyloidosis

Back 122

prototypical myocardial disorder caused by deposition of an abnormal substance in the heart-insoluble extracellular fibrillar deposits of protein fragements prone to forming B-pleated sheets

Front 123

transthyretin

Back 123

normal serum protein synthesized in liver, responsible for transporting thyroxine and retinol-binding protein; senile cardiac amyloidosis is caused due to deposition in heart

Front 124

amyloidosis morphology

Back 124

numerous small, semitranslucent nodules resembling drips of wax may be seen at atrial endocardial surface; eosinophilic deposits of amyloid

Front 125

how are amyloid deposits distinguished from other hyaline deposits

Back 125

Congo red-produces classic apple-green birefringence when viewed under polarized light

Front 126

what can cause iron overload

Back 126

hereditary hemochromatosis or multiple blood transfusions

Front 127

result of iron overload on heart

Back 127

heart dilated, more prominent in ventricles and myocardium; interferes with metal-dependent enzyme systems or induces O2 free-radical injury

Front 128

Prussian blue stain

Back 128

shows hemosiderin accumulations

Front 129

siderosomes

Back 129

iron-containing lysosomes

Front 130

hyperthyroid and heart

Back 130

tachycardia, palpitations, cardiomegaly; supraventricular arrhythmias occosionally; failure uncommon

Front 131

hypothyroidism and heart

Back 131

CO decreased due to reduction in stroke V and HR; increased peripheral resistance and decreased blood V narrow pulse P-prolongation of circulation time, decreased flow to peripheral tissues

Front 132

morphology of well-advanced hypothyroidism

Back 132

heart flabby, enlarged, and dilated; myofiber swelling with loss of striations and basophilic degeneration, interstitial mucopolysaccharide-rich edema fluid = myxedema heart

Front 133

fluid in pericardial sac

Back 133

30-50mL clear, straw colored

Front 134

chronic effusions less than 500 mL clinical significance

Back 134

globular enlargement of heart shadow on chest radiograph

Front 135

cardiac tamponade

Back 135

cardiac filling restricted due to fluid in pericardium

Front 136

primary pericarditis

Back 136

rare and almost always viral

Front 137

acute vs chronic pericarditis

Back 137

usually acute, only few things cause chronic (TB, fungi)

Front 138

serous pericarditis

Back 138

noninfectious inflammatory diseases like RF, SLE, scleroderma, tumors, and uremia; sometimes due to infection of contiguous tissues

Front 139

fibrinous and serofibrinous pericarditis

Back 139

most frequent type of pericarditis; composed of serous fluid mixed with fibrinous exudate; commonly follows acute MI, routine cardiac surgery

Front 140

purulent or suppurative pericarditis

Back 140

invasion of pericarial space by microbes

Front 141

serosal surface in purulent pericarditis

Back 141

reddened, granular, and coated with exudate

Front 142

mediastinopericarditis

Back 142

acute inflammatory rxn that extends into surrounding structures

Front 143

adhesive pericarditis

Back 143

usually has no efect on cardiac fxn

Front 144

adhesive mediastinopericarditis

Back 144

puts strain on heart-hypertrophy and dilation

Front 145

constrictive pericarditis

Back 145

heat encases in dense fibrous or fibrocalcific scar that limits diastolic expansion and CO; cardiac hypertrophy and dilation can't occur due to restriction, heart has little/no capacity to increase CO; heart sounds distant/muffled

Front 146

how often is heart involved in severe prolonged RA

Back 146

20-40%; most common fibrinous pericarditis

Front 147

most common primary heart tumors in order of frequency

Back 147

myoxomas, fibromas, lipomas, papillary fibroelastomas, rhabdomyomas, angiosarcomas, and other sarcomas; 5 most common = benign

Front 148

myxoma

Back 148

benign neoplasms; clonal abnormalities of chromosomes 12 and 17; arise from primitive mulitpotent mesenchymal cells; 90% atria, most L heart

Front 149

histo of myxoma

Back 149

stellate or globular myxoma cells embedded within an abundant acid mucopolysaccharide ground substance; peculiar vessel-like/gland-like structures characteristic; hemorrhage and mononuclear inflammation usually present

Front 150

most common clinical manifestation of myxoma

Back 150

due to valvular obstruction, embolization; fever, malaise

Front 151

IL-6

Back 151

major mediator of acute-phase rxn and elaborated by some myxomas

Front 152

Carney complex

Back 152

10% myxomas; familial autosomal dominant; multiple cardiac and extracardiac myxomas, pigmented skin lesions, and endocrine overactivity

Front 153

Carney complex gene

Back 153

PRKAR1 chromosome 17; encodes regulatory subunit of cyclic adenosine monophosphate-dependent protein kinase A, possibly a tumor suppressor gene

Front 154

where do lipomas occur in the heart

Back 154

L ventricle, R atrium, or atrial septum

Front 155

papillary fibroelastoma

Back 155

incidental, sea-anemone-like lesions, most often found at autopsy; may embolize; resemble much smaller, trivial Lambl excrescences on aortic valves of older ppl

Front 156

where are papillary fibroelastomas located

Back 156

ventricular surfaces of semilunar valves and atrial surfaces of AV valves

Front 157

rhabdomyoma

Back 157

infants and children; obstruction of valvular chamber; associated with tuberous sclerosis (TSC1 or 2); often regress spontaneously

Front 158

morphology of rhabdomyomas

Back 158

small, gray-white myocardial masses; multiple in #, involve ventricles preferentially

Front 159

histo of rhabdomyomas

Back 159

bizarre, markedly enlarged myocytes; myocyte cytoplasm often reduced to thin webs or strands that extend to cell membranes (spider cells)

Front 160

most common metastatic tumors to heart

Back 160

lung and breast, melanomas, leukemias, lymphomas

Front 161

how do tumors reach heart

Back 161

reach heart via retrograde lymphatic extension (carcinomas), hematogenous seeding (many), direct extension, venous extension (kidney and liver)