当前位置： SCI文献检索 > CIRCULATION期刊下所有文献 > Prevalence and risk factors for pulmonary arterial hypertension in a large group of β-thalassemia patients using right heart catheterization: a Webthal study.

Prevalence and risk factors for pulmonary arterial hypertension in a large group of β-thalassemia patients using right heart catheterization: a Webthal study.

Abstract：

BACKGROUND:Pulmonary arterial hypertension (PAH) remains a concern in patients with β-thalassemia major (TM) and intermedia (TI); however, studies evaluating its prevalence and risk factors using systematic confirmation on right heart catheterization are lacking. METHODS AND RESULTS:This was a multicenter cross-sectional study of 1309 Italian β-thalassemia patients (mean age 36.4±9.3 years; 46% men; 74.6% TM, 25.4% TI). Patients with a tricuspid-valve regurgitant jet velocity ≥3.2 m/s (3.6%) on transthoracic echocardiography further underwent right heart catheterization to confirm the diagnosis of PAH (mean pulmonary arterial pressure ≥25 mm Hg and pulmonary capillary wedge pressure ≤15mm Hg). The confirmed PAH prevalence on right heart catheterization was 2.1% (95% confidence interval [CI], 1.4-3.0) and was higher in TI (4.8%; 95% CI, 3.0-7.7) than TM (1.1%; 95% CI, 0.6-2.0). The positive predictive value for the tricuspid-valve regurgitant jet velocity ≥3.2 m/s threshold for the diagnosis of pulmonary hypertension was 93.9%. Considerable functional limitation and decrease in the 6-minute walk distance were noted in patients with confirmed PAH. On multivariate logistic regression analysis, independent risk factors for confirmed PAH were age (odds ratio, 1.102 per 1-year increase; 95% CI, 1.06-1.15) and splenectomy (odds ratio, 9.31; 95% CI, 2.57-33.7). CONCLUSIONS:The prevalence of PAH in β-thalassemia patients as confirmed on right heart catheterization was 2.1%, with an ≈5-fold higher prevalence in TI than TM. Advanced age and splenectomy are risk factors for PAH in this patient population. CLINICAL TRIAL REGISTRATION URL:http://www.ClinicalTrials.gov. Unique identifier: NCT01496963.

journal_name

Circulation

journal_title

Circulation

authors

Derchi G,Galanello R,Bina P,Cappellini MD,Piga A,Lai ME,Quarta A,Casu G,Perrotta S,Pinto V,Musallam KM,Forni GL,Webthal Pulmonary Arterial Hypertension Group*.

doi

10.1161/CIRCULATIONAHA.113.002124

subject

Has Abstract

pub_date

2014-01-21 00:00:00

pages

338-45

issue

eissn

0009-7322

issn

1524-4539

pii

CIRCULATIONAHA.113.002124

journal_volume

129

pub_type

杂志文章,多中心研究

在线工具

Prevalence and risk factors for pulmonary arterial hypertension in a large group of β-thalassemia patients using right heart catheterization: a Webthal study.