Elevated plasma endothelin-1 levels in sickle cell anemia: Relationships to oxygen saturation and left ventricular hypertrophy

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Abstract
Endothelin-1 (Et-1) is a vasoconstrictor produced by endothelial and vascular smooth muscle cells in response to hypoxia, which induces hypertrophy in cultured cardiac myocytes. We measured plasma Et-1 levels and left ventricular dimensions in 13 patients with sickle cell anemia (SCD) and in 12 African-American controls ages 16–29 years. Endothelin-1 concentrations are significantly higher in SCD subjects than controls (10.6 + 1.9 vs. 3.0 + 1.3 pmol/L). There was a negative correlation between oxygen saturation and Et-1 levels in SCD patients (r = -0.71, P = 0.01). SCD subjects have more dilated and hypertrophied hearts corrected for body surface area than controls as evidenced by significant increases in left ventricular end diastolic dimension (31 + 0.8 vs. 24 + 0.9 mm/m2, P < 0.001), left ventricular end systolic dimension (20 + 0.9 vs. 16 &plus 0.8 mm/m2, P = 0.002), left ventricular posterior wall thickness (5.0 &plus 0.1 vs. 4.0 &plus 0.1 mm/m2, P < 0.001), and left ventricular mass (125 + 7.2 vs. 69 + 5.1 g/m2, P < 0.001). The index of left ventricular function, the shortening fraction, was not different between groups (34 + 1.2% in SCD vs. 35 + 1.5% in controls). The correlation between left ventricular mass and levels of Et-1 in SCD subjects was not significant (r = 0.47, P = 0.121). Am. J. Hematol. 58:195–199, 1998.