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Systemic Pathology (Autopsy) Quiz Case 1

Diagnosis and case discussion:

Dr Sampurna Roy MD   

 

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(1) Cross section through the ventricles - Gross examination of the heart shows unusually small cavity of the left ventricle. The wall of the left ventricle is circumferentially thickened. There is thickening of the interventricular septum, specially anteriorly.

(2) and (3) There is complete myocardial disarray. The normal myocardial fibers (4) are arranged in parallel bundles. In the diseased heart there is random orientation of the  fibres. The small bundles and individual fibres are arranged at various angles from  adjacent bundles of fibres.

 This is a case of Hypertrophic Cardiomyopathy     

 

Points to Note:  Like other manifestations of the disease, myofiber disarray was considered to be diagnostic of Hypertrophic Cardiomyopathy.

However, myofiber disarray has been noted in the following conditions:

1) It is normal at the junction where fibers of the right ventricle intersect with fibers of the interventricular septum.

2) In some congenital heart defects.

3) Adjacent to interstitial myocardial scars caused by hypertensive or ischemic heart disease.

Hence one should be cautious before making a diagnosis of Hypertrophic Cardiomyopathy. 

It has been observed that myofiber disarray is quantitatively more severe in hypertrophic cardiomyopathy than in the other conditions in which disarray has been reported.

 

                                                                                                                              

 

For more information visit my post on Cardiomyopathy

 

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Dr  Sampurna Roy  MD

Consultant Histopathologist (Kolkata - India)

   


 

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