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Archives of cardiovascular diseases
Volume 106, n° 4
pages 257-258 (avril 2013)
Doi : 10.1016/j.acvd.2013.03.034
Is there a cardiac involvement in patients with hemoglobin sickle cell disease?

N. Hammoudi a, M. Duprey a, M. Djebbar a, M. Charbonnier b, K. Stojanovic c, R. Girot c, P.-L. Michel a, R. Isnard a, F. Lionnet c
a Hôpital de la Pitié-Salpêtrière, Paris, France 
b Hôpital Saint-Antoine, Paris, France 
c Hôpital Tenon, Paris, France 

Background .– Adult's patients with homozygous sickle cell disease have usually a dramatic left ventricular enlargement and a cardiac high output. Pulmonary hypertension is frequent in this population.

Hemoglobin sickle cell disease is currently considered to be a mild variant of homozygous sickle cell disease with less severe anemia. At date no study have been dedicated to describe cardiac involvement of this disease.

Objective .– To investigate cardiac involvement in adults patients with hemoglobin sickle cell disease (SC).

Methods .– Using a case-control design, 64 adults with SC in stable condition (mean age 33.1±10.9years, 34 women) and 64 age and sex-matched healthy subjects were studied. All patients underwent a complete echocardiography including measurement of the following parameters: Left ventricular ejection fraction (LVEF) and left ventricular end-diastolic volume index (LVEDVi) by biplane method, cardiac index (CI), mitral E to Ea lateral ratio (E/Ea) and tricuspid regurgitation peak velocity (TRV). The exams were reviewed; each parameter was the average of 3 measurements.

Results .– The observed values of LVEDVi, LVEF and E/Ea in patients were significantly different than the corresponding values observed in the control group. However, no patient had LVEF<50% and no patient had E/Ea ratio>15.

CI and TRV were similar in the two groups (Table 1).

Conclusion .– Contrary to the patients with homozygous sickle cell disease, patients with hemoglobin sickle cell disease have only a mild left ventricular remodelling. In these patients, pulmonary pressure appears to be normal.

© 2013  Published by Elsevier Masson SAS.
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