Abstract
Asymmetrical septal hypertrophy (ASH)has been defined as thickness of the interventricular septum exceeding that of the left ventricular posterior wall by a ratio of 1-3:1. To date it has been associated only with idiopathic hypertrophic subaortic stenosis (IHSS) or its precursor. Therefore, one would expect to find ASH only in the cardiomyopathic patient or in his relatives. It is a dominant with complete penetrance.
A previously undescribed presentation of ASH is now documented in a group of pediatric patients demonstrating ASH associated with right ventricular wall hypertrophy in various cardiac lesions and in some normal newborns. The lesions studied include VSD, coarctation of the aorta, severe congenital aortic stenosis with pulmonary arterial hypertentension, pulmonary vascular obstructive disease, and patent ductus arteriosus. None of these patients had a family history of IHSS and none of the family members who had echocardiograms were affected. The normal newborns with ASH with or without right ventricular hypertrophy had regression of ASH with time.
Therefore, septal thickness must be interpreted in relation to the thickness of the right ventricular wall as well as the left ventricular wall and with caution in newborns as there may be temporal regression to normal.
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Larter, W., Allen, H. & Goldberg, S. THE THICK SEPTUM: ECHOCARDIOGRAPHIC DIFFERENTIATION OF ITS CAUSES. Pediatr Res 8, 352 (1974). https://doi.org/10.1203/00006450-197404000-00070
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DOI: https://doi.org/10.1203/00006450-197404000-00070