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- Title
Prevalence and risk factors for pulmonary hypertension in patients with hemoglobin E/β-thalassemia disease.
- Authors
Atichartakarn, Vichai; Chuncharunee, Suporn; Archararit, Napaporn; Udomsubpayakul, Umaporn; Lee, Ratchanee; Tunhasiriwet, Anusith; Aryurachai, Katcharin
- Abstract
Objectives To find the prevalence and risk factors of pulmonary hypertension ( PHT) in adult patients with hemoglobin E/β-thalassemia disease ( E/β-Thal). Methods One hundred and ten clinically stable E/β- Thal outpatients, sixty-one of whom had undergone splenectomy, were prospectively studied using their clinical profiles, selected blood tests, chest roentgenogram, and transthoracic echocardiogram. Based on the pulmonary artery systolic pressure ( PASP) values estimated by the echocardiogram of ≥36 mmHg, they were dichotomized into those with ( PHT+) and without ( PHT−) PHT. Results PHT was found in 41 (37.3%) patients without gender preponderance. It was not due to the left heart and was not severe ( PASP = 46.3 ± 10.4 mmHg). PASP was higher in splenectomized patients (48.0 ± 11 vs. 40.3 ± 4.7 mmHg ( P = 0.004)). PHT was found in 32 of 61 (52.5%) splenectomized patients, mostly (53%) in the second decade, and rarely (6.3%) during the first 5 yr after splenectomy. PHT+ patients had more hemolysis ( P = 0.001-0.04 depending on the parameters), more asplenic cases ( P < 0.001), and higher serum soluble vascular cell adhesion molecule-1 (s VCAM-1) and high-sensitivity C-reactive protein levels ( P = 0.004 and 0.008, respectively). Strong risk factors by univariate analysis were serum s VCAM-1 levels ≥1600 ng/mL, serum cell-free Hb ≥ 3 mg/dL, asplenia, and amount of NRBCs/100 WBCs >40. Conclusions Prevalence of PHT in E/β- Thal patients was 37.3% without gender preponderance. Those with severe hemolysis and asplenia invariably had severer PHT. Strong risk factors were asplenia and associated markedly elevated values of s VCAM-1, cell-free Hb, and NRBCs in blood.
- Subjects
PULMONARY hypertension; SYSTOLIC blood pressure; HEMOGLOBINS; THALASSEMIA; BLOOD plasma
- Publication
European Journal of Haematology, 2014, Vol 92, Issue 4, p346
- ISSN
0902-4441
- Publication type
Article
- DOI
10.1111/ejh.12242