Key result
Aortic and pulmonary atresia exhibit ~62% myocardial disarray, showing it is not pathognomonic for HCM.
Why the study?
Myocardial fiber disarray is a distinctive histopathologic finding in hypertrophic cardiomyopathy, but its presence in other cardiac pathologies and its diagnostic specificity remain unclear.
Comparison
Hearts with aortic atresia vs pulmonary atresia vs normal hearts vs hypertrophic cardiomyopathy heart
Design
Observational quantitative histopathologic analysis of myocardial fiber disarray
Authors
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Fiber disarray in atresia hearts questions HCM specificity; leaves open its diagnostic value without clinical context.
Observational (n=29)
Absolute Event Rate: 62.1% vs 8.7%
Bulkley et al. (1983) conducted an observational in Aortic atresia, pulmonary atresia, and hypertrophic cardiomyopathy (n=29). Aortic and pulmonary atresia vs. Normal infant hearts was evaluated on Overall myocardial fiber disarray. Aortic and pulmonary atresia exhibited extensive myocardial fiber disarray (62.1% and 70.4%) compared to normal hearts (8.7%), demonstrating it is not pathognomonic for hypertrophic cardiomyopathy.
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