Chest
Clinical Investigations: CardiologyThe Heart in Sickle Cell Anemia: The Cooperative Study of Sickle Cell Disease (CSSCD)†
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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Cited by (125)
Cardiac complications of sickle cell disease in pediatric patients: A case report and contemporary literature review
2022, Progress in Pediatric CardiologyCitation Excerpt :Our patient has no cardiovascular symptoms but did have incidental cardiomegaly on echocardiogram. SCD patients have increased plasma volume as a response to chronic anemia, which leads to ventricular dilatation and compensatory hypertrophy [8,9]. He will require close monitoring and will likely benefit from starting hydroxyurea.
Longitudinal analysis of cardiac abnormalities in pediatric patients with sickle cell anemia and effect of hydroxyurea therapy
2021, Blood AdvancesCitation Excerpt :Over time, progressive dilation leads to increased wall stress and an increase in left ventricular (LV) mass.6 Children and adults with SCA are known to have a higher incidence of cardiac disease such as pulmonary hypertension, LV hypertrophy, LV dilation, and diastolic dysfunction.6-8 Many studies have shown that LV systolic function as assessed by shortening fraction (LVSF) and ejection fraction (LVEF) is preserved in most children with SCA.9
Sickle Cell Disease: Clinical Features and Management
2018, Hematology: Basic Principles and PracticeSickle Cell Disease: Clinical Features and Management
2017, Hematology: Basic Principles and PracticeCardiomyopathy with Restrictive Physiology in Sickle Cell Disease
2016, JACC: Cardiovascular ImagingBlood cell membrane omega-3 (n-3) fatty acid abnormality and supplementation in patients with sickle cell anemia
2016, Handbook of Lipids in Human Function: Fatty Acids
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.