Pathology Ch. 12: Valvular Heart Disease

Front 1

The failure of a valve to open completely, which impedes forward flow

Back 1

Stenosis

Front 2

Failure if a valve to close completely, thereby allowing reversed flow

Back 2

Insufficiency

Front 3

Dilatation of the left ventricle that pulls the ventricular papillary muscles down and outward, thereby preventing proper closure of the normal mitral valve is an examle of?

Back 3

Functional regurgitation

Front 4

Is rheumatic mitral disease usually indolent or acute

Back 4

Indolent

Front 5

Leads to pressure overload on the heart?

Back 5

valvular stenosis

Front 6

Leads to volume overload on the heart

Back 6

Valvular regurgitation

Front 7

Most common valvular abnormality? Result of?

Back 7

Acquired aortic stenosis:
Wearing of anatomically normal valves or congenitally bicuspid valves.

Front 8

Aortic stenosis of previously normal valves is the result of?

Back 8

Senile calcific aortic stenosis:
Result of degeneration or chronic injury due to hyperlipidemia, hypertension, and inflammation, that lead to the accumulation of hydroxyapatite.

Front 9

How does the valve injury of calcific aortic stenosis differ from atherosclerosis

Back 9

Instead of accumulating smooth muscle cells with fat laden macrophages, the abnormal valves contain cells resembling osteoblasts that synthesize bone matrix.

Front 10

Where does the calcific process begin?

Back 10

In the valvular fibrosa, at the point of maximal cusp flexion, near the margins of attachment of the cusps

Front 11

Heaped up calcified masses within the aortic cusps that eventually protude through?

Back 11

calcific aortic stenosis:
ultimately protude into the sinuses of valsalva(aortic), preventing the opening of the cusps.

Front 12

Is commisural fusion seen in calcific aortic stenosis? Is the mitral valve normal?

Back 12

No, this is unlike that seen in rheumatic or congenital aortic stenosis
The mitral valve is normal

Front 13

In calcific aortic stenosis, what does the obstruction to outflow lead to?

Back 13

LVH due to pressure overload

Front 14

Most frequent congenital valvular malformation in humans?

Back 14

Bicuspid aortic valve:
50% of the cases of aortic stenosis

Front 15

Appearance of cusps in BAV

Back 15

Two functional cusps, usually of unequal size, with the larger cusp having a midline raphe.

Front 16

Is the mitral valve normal in BAV

Back 16

yes

Front 17

site of calcific deposition in BAV

Back 17

midline raphi on the larger cusp

Front 18

What abnormality usually accompanies BAV

Back 18

Structural abnormalities of the aortic wall. This may lead to aortic dilation or dissection

Front 19

Late complications of BAV

Back 19

Aortic stenosis or regurgitation, infective endocarditis, aortic dilation, and dissection

Front 20

How does mitral annular calcification cause either regurgitation or stenosis? What about arrythmias?

Back 20

Regurgitation:
Interfering with physiologic contraction of the annular ring
Stenosis:
Impairing opening of the mitral valve
Ca2+ deposits may penetrate to the AV node causing conduction abnormalities.

Front 21

Patients with mitral annular calcification are at an increased risk for?

Back 21

Stroke and endocarditis:
the ca2+ deposits are a prime place for thrombi or bacteria to attach.

Front 22

Floppy mitral valves that balloon back into the atrium on systole. Key histologic change?

Back 22

Mitral valve prolapse
Myxomatous degeneration:
Marked thickening of the spongiosa layer with deposition of mucoid material.

Front 23

Key anatomic change in mitral valve prolapse

Back 23

Interchordal ballooning of the mitral leaflets or portions thereof

Front 24

Rheumatic fever

Back 24

Acute, immunologically mediated, multisystem inflammatory disease that occurs a few weeks after an episode of Group A Strep pharyngitis.

Front 25

Mediate matrix remodeling in myxomatous heart valves

Back 25

activated interstitial myofibroblasts

Front 26

Secondary changes of mitral valve prolapse

Back 26

1. fibrous thickening of the valve leaflets due to friction
2. Linear fibrous thickening of the LV endocardium due to friction.
3. Thickening of the mural endocardium of the LV or atrium due to friction
4. Thrombi on atrial walls
5. focal calcifications at the base of the pos mitral leaflet

Front 27

Characterized principally bu deforming fibrotic valvular disease, particularly mitral stenosis, of which it is virtually the only cause

Back 27

RHD

Front 28

Lesions consistent with foci of lymphocytes, plasma cells, and plump activated macrophages called Anitschkow cells. Pathognomic for what?

Back 28

Aschoff bodies
RF

Front 29

Macrophages with abundant cytoplasm and central round nuclei, in which the chromatin resembles a wavy ribbon. pathognomic for

Back 29

Anitschkow cells
RF
found in foci of Aschoff bodies

Front 30

Irregular thickenings of the left atrium found in subendocardial lesions as those caused by RHD

Back 30

MacCallum plaques:
exacerbated by regurgitant jets

Front 31

Marked by the presence of small multiple vegetations along the lines of closure of the valve leaflets

Back 31

RF phase of RHD

Front 32

In chronic RHD, what valve is almost always involved?

Back 32

mitral

Front 33

Inflammation of the endocardium and left-sided valves in RF, typically results in?

Back 33

Fibrinoid necrosis within the cusps or along the tendinous chords. Overlying the necrotic foci are small vegetations called verrucae, along the lines of closure

Front 34

Cardinal anatomic changes in RHD

Back 34

Leaflet thickening, commisural fusion and shortening, and thickening and fusion of the tendinous cords.

Front 35

Are Aschoff bodies seen in chronic RHD?

Back 35

No, they are apart of the acute RF stage

Front 36

Microscopic appearance of the mitral leaflets in RHD

Back 36

Inflammation and subsequent diffuse fibrosis and neovascualrization that obliterates the originally layered and avascular leaflet architecture.

Front 37

The effects of acute rheumatic fever are the result of?

Back 37

1. Antibodies that react to M proteins of group A streptococci that cross react with self-antigens in the heart.
2. Cd4+ T cells that react with self-proteins and activate macrophages seen in Aschoff bodies.

Front 38

After an initial attack of RF, what can cause another attack?

Back 38

Pharyngeal infections

Front 39

Characterized by large, irregular vegetations on the valve cusp that may extend into the chordae

Back 39

Infective endocarditis

Front 40

Severity and tempo of infective endocarditis is determined by?

Back 40

The virulence of the organism

Front 41

Form of infective endocarditis that produces necrotizing, ulcerative, destructive lesions

Back 41

acute infective endocarditis

Front 42

Endocarditis in which inflammation and destruction occur on an abnormal valve

Back 42

Subacute-endocarditis

Front 43

Presence of friable, bulky, destructive vegetations. What compose the vegetations?

Back 43

Iinfectious Endocarditis:
fibrin, inflammatory cells, and bacteria

Front 44

Most common sites of infection in endocarditis

Back 44

Mitral and aortic valve, although the right valve may be affected in IVDU's

Front 45

Most consistent sign of IE

Back 45

fever

Front 46

Microscopic differentiation of vegetations in acute and subacute IE

Back 46

subacute have granulation tissue at their base to signify healing. With time, fibrosis, calcification, and chronic inflammatory infiltrate can develop.

Front 47

Deposition of small sterile thrombi on the leaflets of cardiac valves. Why are they dangerous?

Back 47

NBTE:
Not invasive and do not produce an inflammatory rxn locally, but they may produce systemic emboli in the brain, heart, or elsewhere.

Front 48

NBTE is frequently found in what pts? In what type of systemic state?

Back 48

Those with cancer or sepsis, debilitated pts.
occurs concamitantly in pts with hypercoagable states

Front 49

Major clinical criteria for diagnosis of infective endocarditis

Back 49

Positive blood culture
Positive echo
New valvular regurgitation

Front 50

Presence of small, bland vegetations attached to the line of cusp closure

Back 50

NBTE

Front 51

Characterized by episodic flushing of the skin, cramps, nausea, vomiting, and diarrhea. What side of the heart does it generally involve?

Back 51

Carcinoid syndrome:
Involves the endocardium and right side of the heart

Front 52

Plaquelike fibrous thickenings composed of smooth muscle cells and absent elastin on the endocardial surfaces of the of the cardiac chambers and the tricuspid and pulmonary valves

Back 52

Carcinoid sydrome

Front 53

Plasma levels of what, correlate with the severity of heart disease in carcinoid syndrome?

Back 53

Serotonin and urinary excretion of the serotonin metabolite 5-hydroxyindoleactic acid

Front 54

Mitral and tricuspid valvulitis with small, sterile vegetations . Occurs in what conditions?

Back 54

Libman-Sacks endocarditis

Front 55

Characterized by vegetations that consist of finely granular, fibrinous eosinophilic material that may contain hematoxylin bodies, remants of nuclei damaged by anti-nuclear antibodies. Usual functional abnormality?

Back 55

Libman-Sacks endocarditis:
Mitral valve regurgitation

Front 56

Restriction of cardiac changes to the right side of the heart in carcinoid heart disease is explained by?

Back 56

Inactivation of seritonin and bradykinin by MAO in the vascular endothelium of the lungs

Front 57

Most common cardiac manifestation in carcinoid heart disease

Back 57

tricuspid insufficiency, followed by pulmonary valve insufficiency

Front 58

Carcinoid heart disease usually occurs during?

Back 58

1. Hepatic metastases that release mediators into the IVC
2. Primary carcinoid tumors located in organs outside the portal system (lungs, ovaries), that empty directly into the IVC.
In carcinoid tumors that originate in organs of the portal system, the liver usually metabolizes the mediators, those not causing carcinoid heart disease.

Front 59

Heart disease resulting from an abnormality in the myocardium

Back 59

Cardiomyopathy:
Produce abnormalities in cardiac wall thickness and chamber size, and mechanical/electrical dysfunction

Front 60

Cardiac myopathy characterized by progressive dilation and contractile dysfunction, with hypertrophy. Are the valves affected? Causes?

Back 60

DCM:
No primary valvular abnormality, rather mitral regurgitation occurs as result of chamber dilation(functional regurgitation).
Genetic, alcohol, myocarditis, doxorubicin, hemochromatosis chronic anemia, peripartum myocarditis

Front 61

X-linked cardiomyopathy is usually caused by?

Back 61

Mutations in the gene that encodes dystrophin, a cell-membrane associated cytoskeleton protein.

Front 62

Predominant pattern of inheritance in DCM

Back 62

Autosomal-dominant

Front 63

30 y/o female, 3 wks post partum, develops SOB, fatigue, and poor exertional capacity. Cause?

Back 63

Peripartum cardiomyopathy:
Pregnancy associated hypertension, volume overload, nutritional deficiency, metabolic derangements, or immunological rxn

Front 64

Very common in DCM

Back 64

mural thrombi that may be a source of thromboemboli

Front 65

Ischemic, valvular, hypertensive, or congenital heart disease mimics what?

Back 65

DCM

Front 66

Pericardial restriction is known to mimic what type of cardiomyopathy? Dysfunction characterized by? Caused by?

Back 66

restrictive:
Reduced compliance (diastolic dys)
Amyloidosis, radiation-induced fibrosis

Front 67

Disorder characterized by arrhythmogenic R ventricular cardiomyopathy and hyperkeratosis of plantar palmar surfaces. Gene?

Back 67

Naxos syndrome:
Gene encoding plakoglobin

Front 68

15 y/o develops ventricular tachycardia and then fibrillation, from which he succumbs. Hx shows an autosomal dominant genetic disorder affecting the R ventricle.What is it? Cause?

Back 68

Arrythmogenic Right ventricular cardiomyopathy:
Causes R ventricular failure and arrhythmias. Right ventricular wall is thin, with loss of myocytes and infiltration with fat and fibrosis.
Defective cell adhesion proteins in desmosomes that link cardiac muscle.

Front 69

Myocardial hypertrophy with poor compliant left ventricular myocardium, leading to abnormal diastolic filling and intermittent ventricular outflow obstruction. Type of cardiomyopathy? Cause?

Back 69

Hypertrophic cardiomyopathy:
Autosomal dominant inheritance of missense mutations in genes encoding sarcomeric proteins. Most commonly B-myosin heavy chain.

Front 70

Most common cardiovascular disorder caused by single gene mutations. Essential feature?

Back 70

HCM:
Massive hypertrophy without ventricular dilation w disproportionate thickening of the septum as compared to the free wall.

Front 71

How does contraction differ in DCM and HCM?

Back 71

DCM is hypocontraction, and HCM is hypercontraction. This is due to the dilation seen in the ventricle of DCM while HCM is thickened (asymmetric septal hypertrophy).

Front 72

Basic physiologic abnormality of HCM

Back 72

decreased stroke volume due to impaired diastolic filling, which results from the reduced chamber size and compliance of the hypertrophied heart. In addition, outflow obstruction of the left ventricle is seen.

Front 73

20 y/o male athlete collapses and dies suddenly in a college basketball game. Most likely cause?

Back 73

HCM:
Leads to Afib and thrombus-embolus formation with sudden terminal arrhythmias. And also to focal myocardial ischemia. Defect in sarcomeric genes.

Front 74

Are there more gods than one?

Back 74

No, there is only one God.

Front 75

Primary decrease in ventricular compliance, resulting in impaired ventricular filling during diastole, but without ventricle enlargement or dilation

Back 75

restrictive cardiomyopathy

Front 76

Syndrome of fibrotic ventricular endocardium with eosinophilic inflammatory infilitrate that reduces compliance via endocardial necrosis and scarring.

Back 76

Loeffler syndrome:
PDGFR is implicated
Scarring of the endocardium leads to thrombus formation

Front 77

Chemotherapeutic drugs most often associated with cardiotoxicity

Back 77

Anthrocyclines:
doxorubicin and daunorubicin
Peroxidication of lipids in myocyte membranes.

Front 78

Common findings in hearts injured by chemicals and drugs

Back 78

Myofiber swelling, vacuolar swelling, and fatty changes

Front 79

Characterized by interstitial infiltrates, principally perivascular, composed of lymphocytes, macrophages and a high proportion of eosinophils

Back 79

hypersensitivity myocarditis:
often due to allergic rxn to drugs

Front 80

Parasitization of scattered myofibers accompanied by an inflammatory infiltrate of neutrophils, lymphocytes, macrophages, and occasional eosinophils

Back 80

chagas disease

Front 81

Characterized by a diffuse, mononuclear, mostly lymphocytic infiltrate that affects the heart

Back 81

myocarditis

Front 82

Characterized by a wide spread inflammatory infiltrate containing multinucleate giant cells

Back 82

Giant cell myocarditis

Front 83

Myocarditis is most frequently caused by?

Back 83

Viral infections:
Coxsackie viruses and enteroviruses

Front 84

Senile amyloidosis is caused by deposition of?

Back 84

transthyretin:
leads to restrictive cardiomyopathy

Front 85

Amyloidosis causes what type of cardiomyopathy

Back 85

restrictive

Front 86

Amyloid deposits stain how on HandE?

Back 86

Eosinophilic

Front 87

Resmebles a dilated hypertrophic cardiomyopathy except their is marked hemosiderin within myocytes

Back 87

Iron overload:
Interfers with metal-dependant enzymes and causes free radical energy

Front 88

Primary pericarditis is unusual and almost always of what origin?

Back 88

viral

Front 89

Type of pericarditis that is characteristically produced by noninfectious inflammatory diseases, such as rheumatic fever, SLE, and tumors

Back 89

serous pericarditis:
Mild inflammatory infiltrate within the epipericardial fat consisting of lymphocytes.

Front 90

Most common form of pericarditis that occurs after an acute MI, post-MI (Dressler) syndrome, SLE, or rheumatic fever

Back 90

Fribrinous and serofibrinous pericarditis:

Histo presentation of leukocytes, rbc's, and fibrin exudates.

Front 91

Most striking clinical presentation of fibrinous pericarditis

Back 91

pericardial friction rub

Front 92

A harsh systolic ejection murmur is clinically apparent in what type of cardiomyopathy?

Back 92

HCM:
Ventricular outflow obstruction due to enlargement of the anterior mitral leaflet that results in contact with the ventricular septum during systole, and leads to endocardial thickening and mural plaque formation that inhibit blood from passing. Focal myocardial ischemia results with angina pn

Front 93

Caseous pericarditis has what origin? leads to?

Back 93

TB:
Fibrocalcific, chronic constrictive pericarditis

Front 94

Pericarditis that is most often caused by the malignant spread of a neoplasm to the pericardial space, but may also occur after cardiac surgery

Back 94

Hemorrhagic pericarditis

Front 95

Follows Suppuritive pericarditis, irradiation, or cardiac surgery, in which the pericardial sac obliterated and adherence of the parietal layer to surrounding mediastinal structures occurs

Back 95

Adhesive mediastinopericarditis:
Causes the heart to pull against the parietal pericardium and mediastinal structures. Leads to hypertrophy and dilation.

Front 96

Pt presents with systolic retraction of the rib cage, and pulsus paradoxus. Pt had cardiac surgery 3 months ago w/ subsequent suppuritive pericarditis. What should you be thinking?

Back 96

Adhesive mediastionopericarditis

Front 97

Mimics restrictive cardiomyopathy, in that a fibrocalcific or fibrous scar limits diastolic expansion and cardiac output. Frequently occurs due to what?

Back 97

Constrictive pericarditis:
suppurative pericarditis