1 Department of Pediatrics, Baylor University College of Medicine, 6621 Fannin Street, Houston, Texas 77025
In early infancy congenital adrenal insufficiency in the male may be manifest primarily by a markedly altered electrolyte pattern. The profound toxic effect of the resulting hyperkalemia on the myocardium may clinically suggest the presence of congenital heart disease. Typical changes on the ECG make this a valuable diagnostic tool in hyperkalemia. The dramatic response to specific therapy illustrates the capacity of the myocardium to overcome the transient adverse effects of potassium.