Elastic properties of the ascending aorta in patients with beta-thalassemia major.
BACKGROUND
Beta-thalassemia major (beta-TM) is a congenital hemolytic disorder characterized by impaired left ventricular and endothelial function. However, elastic properties of the aorta have not been sufficiently investigated in patients with beta-TM. We investigated whether beta-TM is related to impaired ascending aortic elastic properties.


METHODS
We studied 36 patients with beta-TM (age: 15.8 +/- 2.6 years) and 30 age- and sex-matched control subjects by echocardiography. Aortic elastic indexes, aortic strain (%), distensibility (cm(2) dyn(-1) 10(-3)), and stiffness index were calculated from the echocardiographically derived thoracic aortic diameters (mm/m(2)), and the measurement of pulse pressure obtained by cuff sphygmomanometry.


RESULTS
Patients versus control subjects had increased aortic diameters (P < 0.001), lower mean aortic strain (9 +/- 3.6 vs. 14.9 +/- 3.2, P < 0.001) and distensibility (0.6 +/- 0.36 vs. 0.8 +/- 0.2, P < 0.012), and higher mean stiffness index (5.3 +/- 2.4 vs. 2.8 +/- 0.6, P < 0.001). Aortic elastic indexes were significantly associated with ferritin level, while stiffness index was significantly related to platelet count.


CONCLUSIONS
Elastic properties of ascending aorta are impaired in patients with beta-TM. Impaired functions of aorta may lead to deterioration of left ventricular function.
Gedikli
Omer
O
Department of Cardiology, Sevket Demirel Heart Center, Suleyman Demirel University, Isparta, Turkey.
Altinbas
Ahmet
A
Orucoglu
Aslihan
A
Dogan
Abdullah
A
Ozaydin
Mehmet
M
Aslan
Suleyman Murat
SM
Acar
Gurkan
G
Canatan
Duran
D
D000293:Adolescent; D001012:Aorta, Abdominal / Q000000981:diagnostic imaging / Q000503:physiopathology*; D001018:Aortic Diseases / Q000150:complications / Q000000981:diagnostic imaging / Q000503:physiopathology*; D001794:Blood Pressure; D016009:Chi-Square Distribution; D004452:Echocardiography / Q000379:methods; D004548:Elasticity; D005260:Female; D006801:Humans; D016014:Linear Models; D008297:Male; D017086:beta-Thalassemia / Q000150:complications / Q000503:physiopathology*