Scarring of the left ventricular papillary muscles in sickle-cell disease

Abstract

A number of publications have described various findings in the heart in patients with sickle-cell disease. 1,2 In general, both ventricular cavities are dilated, cardiac mass is increased, and the epicardial coronary arteries are dilated. Although the cardiac valve leaflets are usually normal anatomically, precordial systolic murmurs are common in these patients. 2,3 Although infarcts are common at necropsy in the lungs, spleen, liver, kidneys and even brain in sickle-cell disease, grossly visible myocardial infarcts are rare in these patients. 4,5 Because patients with sickle-cell disease usually have severe and chronic anemia, and because the papillary muscles are believed to be the last portion of the heart to be perfused by blood through the coronary system, we systematically examined the left ventricular papillary muscles at necropsy in a group of patients with sickle-cell anemia for foci of necrosis or fibrosis, findings indicative of inadequate myocardial perfusion.