Abstract
Background
Homozygous sickle-cell anemia and β-thalassemia are characterized by impaired endothelial
function, while data on arterial stiffness have hitherto been conflicting. We sought
to investigate aortic elastic properties and endothelial function in sickle-thalassemia,
which combines molecular and clinical features of the above conditions.
Methods and results
Forty-seven sickle-thalassemia patients, younger than 45 years, with preserved left
ventricular (LV) function and no history of smoking, systemic or pulmonary hypertension,
diabetes mellitus, dyslipidemia or thyroid disease, along with 40 healthy controls
were studied. Aortic strain, distensibility and stiffness index were calculated by
echocardiographically-obtained aortic root diameters. Brachial artery endothelial
function was assessed by ultrasonographic evaluation of flow-mediated dilatation (FMD)
and nitrate-mediated dilatation (NMD). Left ventricle was assessed by echocardiography.
Patients had an impaired FMD (4.2 ± 2.9% versus 9.2 ± 3.8% in controls, p < 0.001) with a preserved NMD (16.9 ± 5.6% versus 15.2 ± 4.8% in controls, p > 0.05). Aortic strain and distensibility were lower and aortic stiffness index was
higher in patients compared to controls (8.1 ± 4.6 versus 5.8 ± 2.9, p < 0.01). Indexed LV diameters and mass were higher in patients. Systolic LV function
was preserved, while 14.9% of patients had an impaired relaxation transmitral inflow
pattern. Patients’ LV mass index and diastolic mitral E wave deceleration time were
positively correlated with aortic stiffness index (p < 0.001).
Conclusion
Sickle-thalassemia is characterized by a complex vasculopathy, consisting of endothelial
dysfunction and increased arterial stiffness, with a global effect on cardiovascular
function.
Keywords
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Article info
Publication history
Published online: May 24, 2006
Accepted:
April 7,
2006
Received in revised form:
April 7,
2006
Received:
January 8,
2006
Identification
Copyright
© 2006 Elsevier Ireland Ltd. Published by Elsevier Inc. All rights reserved.
