Chest
Volume 108, Issue 5, November 1995, Pages 1214-1219
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Clinical Investigations: Cardiology
The Heart in Sickle Cell Anemia: The Cooperative Study of Sickle Cell Disease (CSSCD)

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The objective of this study was to obtain representative echocardiographic measurements of cardiac size and function in stable patients with sickle cell disease. This prospective, multicenter study utilized central reading of echocardiograms by an investigator blinded to other patient data. Stable outpatients from a balance of inner city and rural settings with SS phenotype and a broad age range were selected, because conflicting results from earlier studies were believed to be due to these patient selection criteria. Right and left ventricular dimensions and wall thickness, left atrial and aortic root dimensions, and systolic time intervals were measured. Body surface area indexed chamber dimensions and septal thickness were significantly increased from normal. Except for the right ventricle, chamber dimensions and wall thickness 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 ventricular ejection time was prolonged. Shortening fraction was normal but velocity of circumferential fiber shortening was abnormally low. Stable patients with sickle cell disease have dilated chambers, septal hypertrophy, and normal contractility. Though left ventricular dilatation was inversely related to hemoglobin, age (duration of illness) was an important factor in that relationship. No specific cardiomyopathy was associated with sickle cell anemia.

Section snippets

Methods

The CSSCD was a multi-institutional investigation of the natural history of sickle cell disease from birth to adulthood. It involved data collection at 23 institutions in a uniform standardized fashion on 3,800 patients. A special effort was made to include patients of all ages, mildly affected patients (to ensure that the study did not collect only a severe hospital-based population), and patients from rural areas. To ensure inclusion of patients who were mildly affected, all 23 clinics

Results

Indexing of cardiac measurements (dividing by body surface area) effectively eliminated any gender relationship. Indexed right ventricular, left ventricular, and left atrial chamber dimensions were significantly increased in patients when compared with normal subjects (Table 1). Indexed aortic root dimensions were significantly increased from normal. Though the left ventricular free wall thickness was not significantly increased in the study population, the interventricular septal thickness was

Discussion

The hemodynamic consequence of a reduced oxygen-carrying capacity due to anemia is the need for an increased cardiac output.4 Cardiac output at rest in patients with sickle cell anemia with hemoglobin levels in the 6 to 8 g/dL range tends to be 50% above normal.24 The successful adaptation to chronic sickle cell anemia results in only minimal increases in heart rate.4,8 The major contributor to the increased cardiac output is an increase in stroke volume. It is, therefore, not surprising to

Acknowledgments

The following were members of the cardiology component of the Cooperative Study of Sickle Cell disease: Participating Investigators: J. Smith, J. Brown, M. Geer, Harlem Hospital (New York); H. Dosik, R. Bellevue, S. Leff, Interfaith Medical Center (Brooklyn, NY); P. Milner, W. Covitz, Medical College of Georgia (Augusta); H. Pearson, N. Talner, W. Hellenbrand, Yale University (New Haven, Conn); Statistical Coordinating Centers: P. Levy, D. Gallagher, A. Koranda, Z. Flournoy-Gill, E. Jones,

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    Supported by the Cooperative Study of Sickle Cell Disease, a program of the Sickle Cell Disease Branch of the National Heart, Lung, and Blood Institute.

    The members of the cardiology component of the CSSCD are listed in the Acknowledgments.

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