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Published on: June 14, 2016
Relation between extent of cardiac muscle cell disorganization and left ventricular wall thickness in hypertrophic
B J Maron1, J K Wolfson, W C Roberts
1Pathology Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland 20892.
Insights
In hypertrophic cardiomyopathy (HC), disorganized cardiac muscle cells are not solely found in thickened areas of the left ventricle (LV). Myocardial disarray and LV wall thickness do not correlate in patients with HC.
Area of Science:
- Cardiovascular Pathology
- Cardiac Morphology
- Heart Disease Research
Background:
- Hypertrophic cardiomyopathy (HC) is characterized by abnormal cardiac muscle cell arrangement in the left ventricular (LV) wall.
- This cellular disarray is thought to contribute to impaired LV compliance.
- The relationship between myocardial disorganization and LV wall thickness in HC remains undefined.
Purpose of the Study:
- To investigate the correlation between LV wall thickness and the extent of myocardial disorganization in patients with HC.
- To determine if cellular disarray is confined to hypertrophied regions of the LV.
Main Methods:
- Systematic comparison of LV wall thickness and percentage of disorganized myocardium within the same tissue sections.
- Analysis performed separately for the ventricular septum, anterolateral, and posterior free walls, as well as combined regions.
Main Results:
- No significant correlation was found between LV wall thickness and the degree of myocardial disorganization.
- Cellular disarray was observed in LV regions with normal or mildly increased thickness, not exclusively in greatly thickened areas.
- LV wall thickening and cellular disorganization appear to be independent manifestations of the cardiomyopathic process.
Conclusions:
- In hypertrophic cardiomyopathy, myocardial disorganization is not limited to thickened LV walls.
- The extent and distribution of LV wall thickening and cellular disorganization are not directly related in HC.
- These findings may improve understanding of the structure-compliance relationship in HC.
Abstract:
The presence of numerous, abnormally arranged, cardiac muscle cells distributed widely throughout the hypertrophied left ventricular (LV) wall has been considered a characteristic, morphologic feature of patients dying of hypertrophic cardiomyopathy (HC) and also probably a determinant of impaired LV compliance. However, the relation between such regions of myocardial cell disarray and the magnitude of wall thickness in the same areas of the left ventricle has not been defined. Therefore, the present study was undertaken in which LV wall thickness and the percent area of myocardium disorganized were systematically compared in the same tissue section. No correlation was identified between wall thickness and the amount of myocardium disorganized in the same tissue sections, either when calculated separately for the ventricular septum, and anterolateral and posterior free walls, or when expressed for all 3 regions combined. Therefore, in patients with HC: (1) disorganized myocardial architecture is not confined to greatly thickened portions of the LV wall, but regions of the left ventricle with normal or only mildly increased thickness may also be disordered; and (2) whereas both LV wall thickening and cellular disorganization are manifestations of the primary cardiomyopathic process, these 2 morphologic features do not appear to be directly related with regard to their extent and distribution within the LV wall. These observations will potentially enhance understanding of the relation between LV structure and compliance in HC.
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