THE HEART IN SICKLE-CELL-ANEMIA - THE COOPERATIVE STUDY OF SICKLE-CELL DISEASE (CSSCD)

The objective of this study was to obtain representative echocardiogra phic measurements of cardiac size and function in stable patients with sickle cell disease. This prospective, multicenter study utilized cen tral reading of echocardiograms by an investigator blinded to other pa tient data, Stable outpatients from a balance of inner city and rural settings with SS phenotype and a broad age range were selected, becaus e conflicting results from earlier studies were believed to be due to these patient selection criteria. Right and left ventricular dimension s and wall thickness, left atrial and aortic root dimensions, and syst olic time intervals were measured, Body surface area indexed chamber d imensions and septal thickness were significantly increased from norma l. Except for the right ventricle, chamber dimensions and wall thickne ss were inversely correlated with hemoglobin, The relationship between left ventricular dimension and hemoglobin was significantly dependent on age, Systolic time interval ratios were normal though left ventric ular ejection time was prolonged, Shortening fraction was normal but v elocity of circumferential fiber shortening was abnormally low, stable patients with sickle cell disease have dilated chambers, septal hyper trophy, and normal contractility. Though left ventricular dilatation w as inversely related to hemoglobin, age (duration of illness) was an i mportant factor in that relationship. No specific cardiomyopathy was a ssociated with sickle cell anemia.