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            Fibrous Hamartoma of Infancy

 


            
The cardiac valves are simple tissue and the number of pathological  lesions are limited.  

Critical information relative to removed valves is obtained from the gross examination of the specimens. Histologic study is valuable to address specific questions such as endocarditis.

All prosthetic valves, intact native valves and any unusual lesions (vegetations, fibroelastoma) are photographed, as close-up as possible. Consider both acute and underlying chronic lesions.

Fibrous thickening and calcification are the commonest responses. The degree varies with the functional lesion and etiology.  Stenotic valves always have severe diffuse fibrous thickening and usually moderate to heavy amounts of of calcific deposits.

In post inflammatory valve disease, the fibrous thickening obliterates the normal trilaminar structure of the cusp and is associated with growth of new vessels into the cusp from the base.

The fibrosis results in commissural fusion and chordal thickening and fusion.

All these features usually lead to stenosis but combinations of fibrous retractions of cusps and chordae with commissural fusion can lead to a mixture of stenosis and regurgitation which is typical of chronic rheumatic disease.

Purely regurgitant valves have mild to moderate degrees of focal superficial fibrous thickening at the free edge due to mechanical trauma, which does not efface the underlying structure of the valve.

Regurgitant atrioventricular valves may have chordae tendineae abnormalities (elongated and/or ruptured) and abnormal papillary  muscles with dilated annular circumference.

Calcification is a non-specific response, which occurs in collagen under mechanical stress and with increasing age of the subject. Calcification causes stenosis and in the aortic valve there is a direct relation between the amount of calcium and the gradient across the valve.

In floppy mitral valve disease the dense collagen of the central fibrous core becomes replaced by more loosely arranged connective tissue rich in glycoaminoglycans and mucopolysaccharides (myxomatous degeneration).  The cusp and chordae elongate and stretch leading to regurgitation. Vascularisation and calcification are not a feature.

Minimal if any structural valvular alterations can occur in purely regurgitant excised aortic valves associated with aortic root dilatation and the purely regurgitant tricuspid valve excised from patients with pulmonary hypertension, except for dilated annular circumference.

                   

Leaflets or cusps: Number of recognisable leaflets (atrio-ventricular valves) or cusps (semilunar valves), size, consistency (thickened, fibrotic, calcified, thinned, redundant [ballooned] , perforated), additional fragments.

If abnormality present: Distribution (focal, diffuse), surface (atrial , ventricular, both), location (free edge, base).

Commissures: Relationship to each other (fused, completely, partially)

Chordae tendineae: Length (shortened, elongated), status (intact, thickened, ruptured, fused).

Papillary muscles:  Dimensions, abnormalities (hypertrophied, elongated, scarred).

Vegetations:  Colour,  size,  location, consistency (firm, friable) , ?destructive to underlying tissue.

Submit one cassette with representative sections taken from the free edge of the annulus. It may be necessary to decalcify some specimens.

Endocarditis is a life threatening disease and any indication that acute endocarditis is present should immediately be brought to the attention of the cardiac pathologist and the clinician (ie. either following gross or microscopic examination).  Order Gram stain if there is a question of endocarditis.

GROSS MORPHOLOGIC ASSESSMENT OF ABNORMAL CARDIAC VALVULAR FUNCTION:
Pathologic feature    Stenotic valve Purely regurgitant valve
For all valves :
 Valve weight    Increased   Normal or slightly increased or decreased
 Fibrous  thickening    Diffuse   Diffuse, focal or none
 Calcific deposits    Heavy   Minimal (if any)
 Tissue loss (perforation)          None   May be present           
 Vegetations    Minimal   May be present
 Commissural Fusion    May be present   Minimal (if any)
 Annular circumference    Normal   Normal or increased
For Aortic valves:
 Number of cusps   1, 2 or 3   2 or 3
For Mitral (or Tricuspid) valves :
 Abnormal papillary muscles   No  May be present
 Chordae tendineae Fusion   Usually present  Absent
 Elongation   Absent   May be present
 Shortening   Usually present   May be present
 Rupture   Absent   May be present

A practical approach to examination of cardiac valves

CARDIAC  VALVE  DISEASE

MITRAL VALVE DISEASE

PULMONARY VALVE DISEASE

TRICUSPID VALVE DISEASE

Etiology of valvular heart disease.Expert Rev Cardiovasc Ther. 2003 Nov;1(4):523-32. 

Etiology of valvular heart disease.Curr Opin Cardiol. 1996 Mar;11(2):98-113

 

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