Original Paper

Pulmonary Hypertension in Sickle-Cell Disease: Comorbidities and Echocardiographic Findings
Ferit Akgül^a, Fatih Yalçn^a, Ergün Seyfeli^a, Edip Uçar^b, Sinem Karazincir^c, Ali Balci^c, Edip Gali^d

Departments of
^aCardiology,
^bInternal Medicine, and
^cRadiology, Faculty of Medicine, Mustafa Kemal University, and
^dDepartment of Paediatrics, Antakya State Hospital, Antakya, Turkey

Address of Corresponding Author

Acta Haematol 2007;118:53-60 (DOI: 10.1159/000102588)

  Key Words

• Pulmonary hypertension
• Sickle-cell disease
• Ventricular function

  Abstract

Background: Our aim is to determine comorbidities associated with pulmonary hypertension (PHT) in clinically stable sickle-cell disease (SCD) patients and to evaluate left ventricular (LV) and right ventricular (RV) function in those patients. Methods: Echocardiography was performed in 87 SCD patients that were divided into group I (without PHT) and group II (with PHT). Both groups were compared with healthy controls. Results: A history of retinopathy and leg ulcer was more frequent in group II than group I (p < 0.01). Haemoglobin levels were lower (p < 0.05), whereas blood urea nitrogen, lactate dehydrogenase and total bilirubin levels were higher in group II (p < 0.01). Although group II patients had larger LV end-diastolic, LV end-systolic and RV diastolic diameters compared with group I patients and controls (p < 0.05), LV ejection fraction was similar in the three groups. The mitral peak early diastolic inflow velocity to peak late diastolic inflow velocity (E/A) ratio was similar in group I, group II and the control group. The tricuspid E/A ratio was lower in group II than group I and controls (p < 0.05). Conclusion: End organ damage occurs more often and haemolysis is severer in SCD patients with PHT than SCD patients without PHT. Although LV systolic and diastolic function is well preserved, RV diastolic function is disturbed in those patients with PHT.

Copyright © 2007 S. Karger AG, Basel

  Author Contacts

Ferit Akgül
Mustafa Kemal Üniversitesi, Aratrma ve Uygulama Hastanesi
Baryank Mah. 31100
Antakya-Hatay (Turkey)
Tel. +90 326 214 0649, Fax +90 326 614 3834, E-Mail feritakgul1@yahoo.ca

  Article Information

Received: October 26, 2006
Accepted after revision: February 21, 2007
Published online: May 14, 2007
Number of Print Pages : 8
Number of Figures : 2, Number of Tables : 6, Number of References : 31