Valvular heart diseases come on two varieties.

What are they?

Stenosis:

norrowed channel from inability to open completely.

forward flow hampered

usually chronic

Insufficiency (regurgitation):

failure to completely close

allows back flow

acute or chronic

*both can happen at the same time! Lesions prefent closure and opening, for example

Hypertrophy or dilation?

Stenosis

Insufficiency

Stenosis - hypertrophy

Insufficiency - dilation

2/3 of valve diseases involve the mitral or aortic valves.

What are the common causes of valve lesions (4)

(id. cause of stenosis and insufficiency for each valve)

1. Aortic stenosis -- calcification

2. Aortic insufficiency -- dilation of aorta and pulling valves apart, 2ndary to HTN and aging

3. Mitral Stenosis -- Rheumatic heart disease

4. Mitral insufficiency -- Myxomatous degeneration, prolapses the valve.

What are the three types of Valvular calcification?

1. Calcific Aortic Stenosis -- normal aging, appears in old age.

2. Calcific stenosis of congenital bicuspid Aortic valve, show signs in middle age.

3. Mitral annular calcification

When does calcification of the aortic valves typically occur?

How would you characterize the calcium lesions?

What complications can this cause a person?

Usually a process of aging/wear and tear. Happens in middle age when the Aortic valve is bicuspid.

The lesions are heaped, calcium nodes that grow on the outflow side of the valve (prevents opening). Usually at the base of the cusps. Causes LV hypertrophy. The cusps fibrose and thicken but DO NOT FUSE

This can lead to IHD, CHF, syncopy, angina

What are the clinical complications seen in Mitral Annular Calcification?

Affecting the fibrous ring "annulus" of the Mitral valve, it is typically out of the way of the valves opening and asymptomatic. The nodules are behind the leaflets

Generally occurs in women >60 with myxomatous valve. Increases left ventricular pressure.

The nodules may serve as a platform for thrombus formation -- stroke due to emboli, or site of infection.

Myxomatous degeneration of Mitral valve

What is the clinical term for what is seen?

Mitral valve Prolapse

The mitral valve leaflets "balloon" back into the left atrium when ventricle contracts. This causes the chordae tendinae to elongate and thin. This is a degenerative disease of the connective tissue which accumulates GAGs and dermatan sulfate, creating a mucous around the valve -- often seen in Marfans.

What can you do to detect a myxomatous degeneration of the mitral (or tricuspid valve 20-40%)?

ECHO of the heart -- most patients are asymptomatic, though thrombi can form on atrial surface.

Listening, you may hear a mid-systolic CLICK when the valve whips back into the atrium.

Patient may have chest pain, dyspnea (blood flows back into atria --> lungs), fatigue, and psych issues.

What are the possible serious complications of Myxomatous degenerating of the MV?

Infective endocarditis, mitral valve insufficiency with a holosystolic mumur, stroke/infarct from the thrombi that can develop, arrhythmias.

Chordae tendinae may rupture and create a severe mitral regurgitating.

What is the causative agent of Rheumatic Fever heart disease?

what is the most common consequence?

Caused by Group A beta hemolytic streptococci infection . Antibody formed against Protein-M produced by the bacteria which is a molecular mimic for proteins found throughout the body, esp the heart.

Most important consequence is Mitral Stenosis.

What is morphologically apparent with histological examination of a rheumatic mitral valve?

1. Aschoff bodies -- swollen collagen (red-staining), infiltrated with lympho's, plasma cells, and macrophages.

Also contains Anitshow cells -- macrophage "caterpillar" cell *Pathognomonic

2. Bread and butter fibrous pericarditis

3. Verrucous vegetations along closure lines of valve

4. Subendothelial lesions -- irreg. thickening containing aschoff bodies (called McCallum plaques)

Thickened valve leaflets WITH commisural fusion -- lesions are along lines of closure, followed by shortening and thickening of fused chordae tendinae.

(remember, calcification does not cause fusion)

The fusion of the leaflets makes the characteristic "Fish mouth"

Left atria dilates

What are the Major Criteria for diagnosing Rheumatic heart disese?

(5)

Migraptory Polyarthritis of LARGE joints

Carditis

Subcutaneous nodules

Erythema marginatum (skin) -- pink rings on trunk and inner surface of limbs

Sydenham chorea (jerky movements affecting mainly the face, feet, and hands)

Fever

Arthralgia

elevated acute phase reactants (produces increase ESR)

What is the Jones Criteria for Diagnosing Rheumatic Heart Disease?

Need 2 of the major criteria

OR

1 major and 2 minor criteria

Anti streptolysin O

DNAse B

Furthur complications of Rheumatic disease include

arrhythmias (a fib), thromboemboli, infective endocarditis

Endocarditis is a general term for inflammation of:

What are the components of the resulting vegetations found in the various forms of endocarditis?

inflammation of the endocardium AND valvs

Components of vegetation: fibrin, platelets, blood components...may host bacteria

Rheumatic Heart Disease

Infectious Endocarditis

Non-Bacterial Thrombotic Endocarditis (NBTE)

Libman Sachs Endocarditis (found in SLE)

What are the common events that may play host to infectious endocarditis?

Hint: they are all things that affect valves

Myxomatous degeneration of MV

Degerative calcific stenosis

Bicuspid valve (fusion)

Artifical/Prosthetic valve placement

These create places where bacteria can get a foothold. In the case of replacement valves, bact may be implanted.

Other predisposing events include neutropenia (defect in clearing bact from system), immune deficiency, diabetes melitis, alcohol abuse, Intravenous drug use

Strep Viridins (50-60%)

Staph Aureus (10-20%) common in IVDA

Enterococci

HACEK organisms (Haemophilus, actinobacillus, cardiobacterium, eikenella, kingella) all slow-growing GN bacteria that are part of normal human flora -- systemic entry following dental procudures

Coagulase negative Staph (on prosthetic valves)

What is the morphology of infective endocarditis?

What valves are commonly affected?

Friable, bulky vegetations on the valves. Can eat through valves and break apart (friable). Composed of fibrin, inflam. cells, and bacteria.

They erode the underlying myocardium causing ring abscesses.

Most commonly found on the mitral and aortic valves -- Tricuspid valve for IV drug users because bact. is entering throught he venous circulation and going straight to heart.

Friable vegetations can break apart and travel systemically.

Therefore, systemic emboli and septic infarcts can be found.

Microemboli in systemic circulation can cause petechiae, splinter hemorrhages, Janeway lesions (lesions on palms and soles), Osler nodes (subq nodules in pulp of digit), Roth spots (retinal hemorrhages).

Non-bacterial Thrombotic Endocarditis is also known as what?

What are its characteristics?

Also known as Marantic endocarditis

Sterile inflammation.

May be caused by a mucinous adenocarcinoma of the pancreas.

Produces nondestructive vegetations composed of fibrin and plt. thrombi along lines of closure.

It is related to a hypercoagulable state (hence the thrombi)

The opening and closing of the valve can cause chronic lysis of RBCs. May produce enlarged livers and spleens plus other hemolytic syndroms (circulating Hgb is irritating to endothelium).

The valve surface may be thrombogenic. Recipient will likely be on anticoagulant therapy long term.

What are some possible early complications of prosthetic valves?

(5)

Myocardial injury, Vascular injury, Damage to conduction system (may be due to the surgury to sew in the valve)

Paravalvular leaks due to dehiscence

Infectious endocarditis (coag neg. staph)

What are some late complications of prosthetic heart valves?

(5)

Thromboembolic phenom.

infectious endocard. (always provides a safe place for bact. to hide)

hemolysis

Hemorrhage (related to the antithrombotic therapy)

Structural failure -- may include pieces breaking off, stenosis, tearing, rupture.

Carcinoid Heart Disease

Dense endocardial Fibrosis of the Right heart and valves.

Serotonin and Urine 5HA correlate with disease.

May also occur in Left heart with Phen-fen (Phentermine) and ergots.

Carcinoid = slow-growing type of neuroendocrine tumor