Key result
Evaluation of pathological criteria in 39 autopsy patients showed that none of the current criteria for diagnosing hypertrophic cardiomyopathy can be used as a 'gold standard'.
Why the study?
Which pathological criteria are most reliable for the diagnosis of hypertrophic cardiomyopathy in autopsy specimens?
Observational (n=39)
Which pathological criteria are most reliable for the diagnosis of hypertrophic cardiomyopathy in autopsy specimens?
Current pathological criteria for hypertrophic cardiomyopathy are imperfect, and no single criterion can be used as a definitive gold standard.
Answers are grounded in this paper and cite their sources.
No current pathological criterion qualifies as gold standard for HCM at autopsy; leaves open validation of refined diagnostic thresholds.
This study aims to evaluate and compare the current pathological criteria for the diagnosis of hypertrophic cardiomyopathy (HOCM). The following criteria were applied to 39 autopsy patients whose hearts showed myofibre disarray: (i) disarray involving more than 5% of ventricular septal myofibres, (ii) asymmetrical septal hypertrophy (ASH), (iii) mirror-image subaortic plaque, and (iv) a positive histologic HOCM index (HHI). Group 1 (27 patients) with greater than 5% ventricular septal myofibre disarray were diagnosed as HCM, whereas group 2 (12 patients) had less than 5% disarray. The mean disarray value in group 1 was 52% compared to 1.2% in group 2. The two groups showed significant differences regarding ASH and in the amounts of myofibre disarray in the free walls of both ventricles. A new finding was that the histologic HOCM index was significantly higher in patients who died suddenly. The HHI was the commonest positive criterion in group 1, followed by ASH and a mirror-image endocardial plaque. None of the current imperfect pathological criteria for the diagnosis of HCM can be used as the 'gold standard'.
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Alan G. Rose (1984) conducted an observational in Hypertrophic cardiomyopathy (n=39). Pathological criteria evaluation (Group 1: >5% ventricular septal myofibre disarray) vs. Group 2: <5% ventricular septal myofibre disarray was evaluated on Mean ventricular septal myofibre disarray. Evaluation of pathological criteria in 39 autopsy patients showed that none of the current criteria for diagnosing hypertrophic cardiomyopathy can be used as a 'gold standard'.
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