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- Restrictive Cardiomyopathy
- This gross photograph shows a heart with idiopathic restrictive cardiomyopathy. This heart is enlarged due to the marked bi-atrial dilatation (only the left atrium is seen). Note that the left ventricular cavity and wall thickness are normal in size and caliber, respectively. This would be true of the right ventricle as well (not shown).
- -atria are large, bc they are trying to pump blood into stiff ventricle; atria is dilated & undergoes hypertrophy
- there may be varying levels of fibrosis
- Cardiac Amyloidosis
- secondary cardiomyopathy called cardiac amyloidosis- dense, abnormal protein deposited in tissue; causes stiffening of heart- one of the causes of restrictive cardiomyopathy
- For the left image, also note how large the left atrium is.
- -right: brown material is deposition of amyloid in the tissue
- Firm, rubbery, & noncompliant heart with thickened walls
- Multiple small, waxy plaques in atrial endocardium
- a disease we don’t understand; most pts have involvement of other organs
- -most pts with this disease don’t have heart problems; some have heart blocks, slower heart rate, sudden death (common initial & final presentation of cardial sarcoidosis)
- The upper panel depicts a low power H&E section of the heart in a patient with cardiac sarcoidosis. You can seen a lot of pink which is indicative of dense fibrosis. The more magenta structures represents the cardiac myocytes. If you look closely, there are small clusters of basophilic structures that represent granulomas (collection of histiocytes) with surround chronic inflammation.
- The lower panel shows a densely fibrotic endomyocardium with a single, discrete granuloma with surrounding chronic inflammation.
- -non-necrotizing granuloma (collection of inflammatory cells, seen in many infectious diseases like tuberculosis)- granulomas may disappear eventually, replaced by scars
- -don’t need to know specific causes of cardiomyopathy; but know the basic differences btw the types- dilated, hypertrophic, restrictive etc
- there are no real cures, only treatments
- This photomicrograph is from a heart with a severe lymphocytic myocarditis due to a viral infection. There is a very prominent, predominantly lymphocytic inflammatory infiltrate (many of the blue dots are lymphocytes). In addition, the interstitium is widened by the inflammatory infiltrate and edema. The myocytes (red strands in the photograph) are injured.
- -much of myocardium is gone, destroyed by intense infiltrate of inflammatory cells
- Giant Cell Myocarditis
- Upper panel shows an extensive inflammatory infiltrate (upper portion) with extensive myocardial necrosis (lower portion). There are scattered multinucleated cells within the infiltrate (arrows).
- The lower panel is a higher power photomicrograph of the above image and shows multinucleated giant cells with plasma cells, lymphocytes, and eosinophils (left side). The right side of the image shows necrotic myocytes.
- -instead of discrete granulomas, you have a more diffuse infiltrate with multinucleated giant cells
- Hypersensitive Myocarditis
- The upper panel is a lower magnification and the lower panel is a higher magnification photomicrograph of a case of hypersensitivity myocarditis. Both show a mixed inflammatory infiltrate containing a large number of eosinophils (bright red cells) [lymphocytes are the blue dots] within the myocardium.
Senile Calcific Aortic Stenosis
- Both upper and lower panels exhibit the same aortic valve as viewed from top.
- The upper panel shows the aortic valve when it is closed as in diastole. You can appreciate a significant number of heaped-up calcified masses mostly at the bases of the aortic cusps protruding through the outflow surfaces into the sinus of Valsalva. Also, there is no commissural fusion as in rheumatic heart disease, which is another potential cause for aortic stenosis.
- The lower panel shows the aortic valve when it is opened as in systole. You can see that the calcified masses are preventing the valve cusps from opening completely resulting in obstruction to flow.
- -calcification on valves > stenosis; yet valves can close properly, so there is little leakage
- -problem: once pts get symptomatic, mortality is very high; there is no contraindication to surgery for treatment
- -new therapy: insertion of artificial heart valve; valve replacement
JONES MAJOR CRITERIA
- Joints (migratory arthritis)
- O [heart-shaped “O”] (carditis)
- Nodules (subcutaneous)
- Erythema marginatum
- Evanescent, non-pruritic, erythematous rings on trunk
- Sydenham’s chorea
- Rapid, involuntary, uncoordinated jerking movements of body (mainly face, hands, and feet)
Acute Rheumatic Fever Diagram
- This gross photograph depicts a case of acute rheumatic fever with valvular involvement by small, irregular, warty nodules along the line of closure of the mitral valve. Histologically, these would correspond to Aschoff nodules.
- -valves with fibrination- inflammation will eventually scar valve
- Acute Rheumatic Fever Micro
- This is a photomicrograph of an Aschoff nodule (or body), which essentially consists of necrobiotic collagen and Anitschkow cells. The latter represent plump macrophages with a characteristic chromatin pattern, i.e., wavy, caterpillar-like, and hence, the other name for these cells, “caterpillar cells”. There are also scattered lymphocytes and plasma cells which are much smaller than the macrophages.
- -Aschoff body- collection of macrophages in heart muscle, characteristic of acute rheumatic fever
- -Anitschkow cells- elongated macrophages
- Chronic Rheumatic Fever
- This gross photograph exhibits an aortic valve as viewed from the top. It is involved by chronic rheumatic valvular disease and demonstrates fibrous thickening of the valve cusps as well as commissural fusion. There are also some calcified masses on the valve outflow surfaces. This valve will not open properly (stenosis) but it will also not close properly either (regurgitation).
- -different from calcific aortic stenosis, bc there is commissural fusion, where valves cannot close all the way
- Mitral Valve Prolapse
- Gross findings
- Intercordal ballooning of mitral leaflets – often enlarged, redundant, thick, rubbery
- Elongated, thinned, & occasionally ruptured chordae tendinae
- Annular dilatation
- Commissural fusion is absent
- The upper panel demonstrates a mitral valve with myxomatous degeneration in which there is intercordal ballooning or parachuting of the mitral valve leaflets (most prominent in the posterior mitral valve leaflet). These leaflets are enlarged, redundant, thick, and rubbery. The chordae tendinae are also somewhat elongate and thinned.
- The lower panel demonstrates a mitral valve as viewed from the top showing ballooning of the anterior leaflet. Note also that there is annular dilatation and that valve leaflets do not appose one another, potentially resulting in a regurgitant valve.
- -valves thick, with scalloped appearance; loose connective tissue that replaced normal collagenous structure; mitral valve prolapsed into atrium (mitral regurgitation)
- -why is it important to know abt valve disease? Bc many pts with valve problems require prophylactic antibiotics before operations
- Mitral Valve Prolapse Micro
- This photomicrograph is from a mitral valve with myxomatous degeneration (mitral valve prolapse) showing marked attenuation of the fibrosa layer (pink/magenta colored tissue) and marked accumulation of myxomatous material in the spongiosa layer (blue colored tissue).
- -instead of the normal valve, which has thin spongiosa, there is additional material in this layer
- Infective Endocarditis
- Pathological findings
- Friable, bulky, & potentially destructive vegetations containing fibrin, inflammatory cells, & organisms on valves
- SBE vegetations have granulation tissue at bases suggesting chronicity
- Chronic inflammation, fibrosis, & calcification may be present with time
- The upper panel shows a mitral valve with a large, friable vegetation in a patient with infective endocarditis.
- The lower panel is from a mitral valve with multiple vegetations. Note also that there is perforation of the anterior leaflet as indicated by the wooden stick.
- -vegetations can break off; infection also destroys the valve so that there are holes/perforations in the valves > may need valve replacement
- Fibrous Pericarditis
- This gross photograph is a case of fibrinous pericarditis. The pericardial cavity is shown after the parietal pericardium has been reflected. The visceral pericardial surface contains granular strands of fibrinous material akin to a cat’s tongue or peanut butter sandwich in which one portion has been peeled off. This is consistent with fibrinous pericarditis.
- -fibrinous pericarditis- looks like 2 pieces of buttered bread put together, then pulled apart
- Dressler’s syndrome- immune-mediated; body makes antibodies & there is inflammatory reaction in the pericardium
- Constrictive Pericarditis
- The upper panel is a gross photograph from a heart with constrictive pericarditis. As can be seen, the heart has been encased by dense fibrous tissue.
- The lower panel is a photomicrograph of a slice of heart that has been stained with Masson trichrome (blue = collagen, red = myocardium). There is a thick, dense fibrous rind of tissue surrounding the heart. Note the absence of hypertrophy and dilatation.
- -constrictive pericarditis- physiology is like restrictive heart disease, where heart cannot expand
- -scarring develops, so there are symptoms of heart failure; heart cannot fill, but pumps properly
- -bottom right: dense scar tissue such that heart cannot expand; surgery: scar tissue peeled off of heart, but very few surgeons can do this
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