ISSN 2997-1036
International Journal of Hematology | Vol. 8, No. 10, October 2017 | pp. 73–80
DOI: 10.46882/2017/IJH/000094
Original Article
Title: Association between steady-state plasma endothelin-1 levels and pulmonary hypertension index indices in adult sickle cell anemia patients
Names of Authors: S. T. Adeyemi¹, U. V. Okoye², W. X. Salami³
Authors’ Affiliations: ¹Department of Haematology and Blood Transfusion, Obafemi Awolowo University, Ile-Ife, Nigeria; ²Department of Medicine, University of Nigeria Teaching Hospital, Enugu, Nigeria; ³Department of Paediatrics, Ahmadu Bello University, Zaria, Nigeria
Abstract: Chronic vascular endothelial damage in sickle cell anemia shifts the homeostatic balance toward continuous vasoconstriction, predisposing individuals to severe cardiopulmonary complications. This prospective study evaluated the relationship between steady-state plasma endothelin-1 levels, an endothelial-derived vasoconstrictor peptide, and pulmonary hypertension risk indices in 84 adult patients with stable sickle cell anemia (HbSS). Plasma endothelin-1 concentrations were quantified via an enzyme-linked immunosorbent assay, while pulmonary hypertension risk was estimated using tricuspid regurgitant jet velocity (TRJV) on transthoracic Doppler echocardiography. Elevated endothelin-1 levels (≥ 5.2 pg/ml) were identified in 35.7% (30 of 84) of the patients. The high endothelin-1 subgroup demonstrated a significantly higher mean tricuspid regurgitant jet velocity (2.92 ± 0.38 m/s versus 2.18 ± 0.22 m/s in lower endothelin cohorts, P < 0.001) and lower total hemoglobin values. Plasma endothelin-1 concentrations correlated positively with serum lactate dehydrogenase activity (r = 0.54, P < 0.01), linking peptide release to chronic intravascular hemolytic intensity. Elevated plasma endothelin-1 serves as a reliable marker of severe endothelial dysfunction, identifying adult sickle cell patients at high risk for progressive pulmonary vascular remodeling.
Keywords: Sickle cell anemia, endothelin-1, endothelial dysfunction, tricuspid regurgitant jet velocity, hemolysis
Manuscript Timeline: Received: July 10, 2017; Revised: August 20, 2017; Accepted: September 12, 2017; Published: October 14, 2017