Primary cardiomyopathy in nonanemic patients: Association with sickle cell trait

Abstract

In 20,599 admissions to the medical service of the Queens Hospital Center during a 41 month period, 23 cases of idiopathic or primary cardiomyopathy were found. Six patients had both cardiomyopathy and sickle cell trait. The coexistence of these two conditions is probably not fortuitous, but the data so far are inadequate for proof. A theory is presented implicating the presence of S hemoglobin, perhaps facilitated by chronic alcohol ingestion, as an important determinant in the pathogenesis of certain forms of cardiomyopathy. Anemia was not deemed necessary for the development of such heart disease. Heterozygous sickle cell states may otherwise be entirely asymptomatic and would go unrecognized but for the development of severe cardiac disability or death.Further studies of larger groups of Negroes for the early detection of heterozygous sickle cell states should be undertaken to determine more fully the incidence of serious pulmonary and cardiac complications in susceptible individuals and to delineate further the pathogenesis of this type of primary myocardial disease.