Abstract
Disproportionate septal thickening (DST) (septal-free wall thickness ratio ≥1.3) is the most characteristic anatomic feature of hypertrophic cardiomyopathy (HCM). To assess prevalence of DST in infants with congenital heart diseases, and to determine whether septal-free wall ratio ≥1.3 should be utilized as the diagnostic criteria of DST in infancy (when absolute wall thicknesses are small), 129 patients <2 yrs old were studied at necropsy. DST was present in 33 (25%) of 129 patients; in these patients septal-free wall ratios ranged from 1.3-2.5 but were ≤1.7 in 32. DST was not common in patients with any particular cardiac malformation. Of note, if a septal-free wall ratio ≥1.5 was used as the criteria for DST, only 7% of patients were abnormal. In only 3 of 33 patients with septal-free wall ratio ≥1.3 was marked disorganization of septal myocardium, characteristic of HCM, present. Hence: 1) DST is common in infants with congenital heart diseases studied at necropsy; 2) the vast majority of such patients with septal-free wall ratios of ≥1.3 do not have typical necropsy findings of HCM, since disorganization of septal myocardium is rarely present; and 3) septal-free wall ratio ≥1.3 alone is not reliable in identifying associated HCM in infants with congenital heart disease, especially if marked absolute septal thickening or disorganization of septal myocardium are absent.
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Maron, B., Edwards, J. & Epstein, S. 917 IS DISPROPORTIONATE VENTRICULAR SEPTAL THICKENING A RELIABLE MARKER OF HYPERTROPHIC CARDIOMYOPATHY IN INFANTS?. Pediatr Res 12 (Suppl 4), 516 (1978). https://doi.org/10.1203/00006450-197804001-00922
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DOI: https://doi.org/10.1203/00006450-197804001-00922