Valvular Heart Disease & Tumors Flashcards
What valve is shown in the provided image?
What are important characteristic of this valve?
- Aortic Valve (Pulmonic looks exactly the same)
- 3 cup-shaped cusps
- thin, delicate, pliable
- plop open & are flexible
- no nodularity
- When they close, they are very tightly opposed, so there is no leakage around them
What is the the valve shown in the provided image?
What are its important characteristics?
- 2 Leaflets (not cusps)
- thin, delicate, pliable
- attached by chordae tendinae to the papillary muscles
- When leaflets close, they prevent back leakage (regurgitation) from ventricle to atrium
- Tricuspid is the same, but with 3 leaflets
What is Rheumatic Fever and how do you get it?
What should you be looking for in the history?
-
Immune-mediate inflammatory disease that occur few weeks after group A beta streprococcal pharyngitis (ie. strep pyogenes)
- Streprococcal M proteins that cross react with self-antigens in the heart, causing damage to the endocardium, myocardium, & pericardium
- Sterile condition, there are no organisms left (cultures will be negative, but can draw blood for antibodies)
- Episode Rheumatic Fever will predispose that person to recurrent Rheumatic Fever is they are infected again
- May not be aware they had strep throat, but maybe had just been tired, may not have had a serious sore throat, in other cases they will feel really terrible (headache, very sore throat, etc.)
- Major problem before widespread antibiotic therapy
What are the components of the Jones Criteria?
What are the major criteria for diagnosing Rheumatic Fever?
Minor criteria?
** will not be tested
- Jones Criteria: Evidence of a preceding group A streptococcal infection (culture, immunoassay, or positive serologic tests) and two major criteria or one major and two minor criteria
-
Major Criteria
- Carditis
- Polyarthritis (reactive arthritis)
- Chorea (rapid, jerky movements of the body)
- Erthema marginatum (upper arms, legs, & trunk)
- subcutaneous nodules (uncommonly seen)
-
Minor Criteria
- Fever
- arthralgia
- Previous ARF/RHD
- Increased acute-phase reactants
- Prolonged PR interval
What pathology is shown in the provided image?
Acute Rheumatic Fever: “Bread and Butter” Pericarditis
Gross: If you spread a lot of butter on a couple slices of bread & then stick them together and pull them apart, you get this kind of shaggy appearance & yellow color
Microscopic: lower left is myocardium, the middle where its a little lighter in color is the pericardium. May have a few scattered inflammatory cells (typically no neutrophils - nonsupperative). On the pericardium, you see what cause the shaggy, butter appearance, extensive fibrin deposition
What pathology is shown in the provided image?
Acute Rheumatic Fever: Inflammatory Mitral Valvulitis
Sterile condition, do not have organisms in them
- Little vegetations- verugae (different from infectious endocarditis) due to inflammation of the endocardium.
- Fibrinoid necrosis of the leaflets & subsequent scaring along the lines of closure, where they attach to the chordae tendinae.
- Mitral Valve most commonly involved
What pathology is shown in the provided image
- Rhematic Heart Disease
-
Aschoff body: small, subtle areas of necrosis- we can see the inflammatory cells responding to & causing the necrosis
- lymphocytes, plama cells, macrophages (Anitschkow cells
- Elongated cell on bottom photo are Anitschkow cells, which are specially activated, specialized macrophages (caterpillar cells)
- in cross section, look like Owl Eyes or Target
- Definitively diagnostic for Rheumatic Fever
What patology is shown in the provided image?
When does it typically present?
Mitral Valve Stenosis of Rheumatic Heart disease
Chronic Rheumatic Heart Disease, typically presents years or decades after the initial episode of Rhematic Fever
Mitral Valve is most commonly affected
Fish mouth stenosis: chronically scarred, very hard due to calcifications & fibrotic in other areas. Will not open or close properly – usually get stenosis & regurgitation. Surgical correction may be currative
What pathology is shown in the provided image?
Rheumatic Aortic Stenosis
Valve has a “dipped in candle wax” appearance
Commisures are completely fused & thickened (white solid arrows). No opening or closing, so will have stenosis & regurgitation
When do valvular heart disease become clinically apparent?
The severity of the disease depent on what criteria?
May be clinically silent when mild
- Become clinically apparent when one develops either or both:
- Stenosis
- Insufficiency (regurgitation)
- Depends on
- Valve involved
- Severity
- Speed of development
- Compensatory mechanisms
What is the most common cause of the following
Aortic Stenosis
Aortic Insufficiency
Mitral stenosis
Mitral insufficiency
- Aortic Stenosis: wear and tear, bicuspid AV, RHD
- Aortic Insufficiency: dilation of the ascending aorta, usually due to hypertension and aging; RHD
- Mitral stenosis: post-inflammatory scarring of RHD
- Mitral insufficiency: myxomatous degeneration (mitral valve prolapse); RHD
RHD = rheumatic heart disease
What pathology is shown in the provided image?
How common is it?
When is it most often diagnosed?
Congenital Bicuspid Aortic Valve
1.4% of live births
asymptomatic early
early calcific stenosis (b/c bloodflow will be more turbulant across the valve)
often diagnosed in young adulthood
larger cusp often has a midline raphe (yellow arrow)
What pathology is shown in the provided images?
What is the major difference between the two images?
Calcific Aortic Stenosis
Congenital bicuspid valve– will develope decades earlier (young adults)
Normal 3 cusps (60-70s)
What pathology is shown in the provided image?
- Gross: calcifications start at the base and eventually extend to the edge of thos cusps & commisures
- Microscopic: nodule that is markedly calcified in teh center (decalcified section, so you aren’t seeing that typical blue)
What are the clinical manifestations of aortic stenosis?
- Clinical Manifestations
- angina
- syncope (fainting)
- congestive heart failure
- murmur
- palpitations
- sudden cardiac death
What pathological condition is depicted by the provided image?
Common causes?
Aortic Regurgitation
- Caused by
- Aortic stenosis
- endocarditis
- rheumatic heart disease
- dilation of arotic root (aneurysm)
- Syphilis
- Marfan’s syndrome
- autoimmune disorders
What pathology is shown in the provided image?
This commonly leads to what clinical problem?
Mitral Valve Prolapse
When the ventricle contracts, the valve has a tendency to flop (parachute) into the atrium, so you get regurgitation since the valve is not closing properly
long axis of left ventricle demonstrating hooding with prolapse of the poterior mitral leaflet intot he left atrium (arrow)
What pathology is shown in the provided image?
What is different about the two image?
Mitral Valve Prolapse
- Stain color
- Collagen = yellow
- proteoglycans = blue
- elastin = blue/black
- Normal valve
- very organized
- Myxomatous valve
- collagen & elastin are much more loose and disorganized
- you lose the elastic layers
- deposition of proteoglycans in the spongiosa
What pathology is shown in the provided image?
It is most commonly seen in what demographic?
Mitral Annular Calcification
Most commonly seen in elderly women
Most often does not impact function of valve, but in severe case it can impede function– can also extend down into the superficial portion of the superficial portions of the myocardium
What pathology is shown in the provided image?
Features of the disease?
Subacute Bacterial Endocarditis: Viridans group Streptococci
Features: large, bulky vegetations found over the valve cusps. They are damaging and sometimes destroying the valves themselves - NOT just fibrin, you have actual organisms
Will have fibrin & acute inflammation
What pathology is shown in the provided image?
Infective Endocarditis Microscopic
Fibrin in the top left & lots of neutrophils in the right side of the image
If a gram stain were performed, you would see organisms
What pathology is shown by the provided image?
Acute Bacterial Endocarditis: Staphylococcus arueus
Congenitally bicuspid aortic valve with extensive cuspal destruction and ring abscess (black arrow) - can arise in previously normal valves (different from subacute endocarditis)
More explosive presentation with a 50% mortality rate
What pathology is shown in the provided image?
Non-bacterial thrombotic endocarditis (NBTE)
Small vegetations along closure lines
No significant inflammation, no organisms, no valve damage
(important because infectious endocarditis would be on the diferential)
What pathology is shown in the provided image
Characteristics of this disease?
Libman-Sachs Endocarditis
Small, fibrinous, sterile vegetations may be found on either side of valve leaflets- typically seen in things like systemic lupus erythematosus
Inflammation & damage to the valve - can result in something that looks like chronic rheumatic heart disease with all the scarring
Identify the disease depicted by the provided diagram
** Nonbacterial thrombotic endocarditis usually looks more like rheumatic heart disease with the small vegetations– but CAN have some large vegetations
-
RHD: Acute Rheumatic Heart Disease
- small, warty vegetations along the lines of closure of the valve leaflets
-
IE: Infective Endocarditis
- large, irregular masses ont eh valve cusps may extend onto the chordae
-
NBTE: Nonbacterial thrombotic endocarditis
- one to several small, bland vegetations, usually attached at the line of closure
-
LSE: Libman-Sachs Endocarditis
- small or medium-sized vegetations on either or both sides of the valve leaflets
What pathology is shown int he provided image?
Where in the heart is this most likely to occur & unique features?
Most commonly affected demographic?
Cardiac (Atrial) Myxoma
Can occur in any chamber, but much more common in the atria
Can be pedunculated or sessile - Typically rounded tumors attached to the wall
Can have a smooth surface or a more hemorrhagic surface – flop around a bit & if the block the valve, the person can pass out
Proliferation fo Myxoma cells in a Myxoid background (arrows in micrscopic image)
Most common primary tumor of the heart & is most common in young adults
What pathology is shown in the provided image?
Unique features?
What is the major concern with this type of tumor?
Cardiac Fibroelastoma
most common tumore of the cardiac valves
little sea anemone-type projections made of valvular material
benign tumor – but they can break off & embolize
Treatment: excise & done
What pathology is shown in the provided image?
Unique characteristics?
Most commonly affected demographic?
Rhabdomyoma - benign tumor of cardiac myocyte origin
Black arrow = one large one
White arrows = smaller ones
Most common cardiac tumor in kids - most associate with tubrasclerosis conflicts & other congenital disorders
What pathology is shown in the provided image?
Most common tumors metastatic to the heart?
- Lung carcinoma
- Melanoma
- Breast carcinoma
- Renal cell carcinoma
- Germ cell tumors
- Childhod rhabdomyosarcoma
