• Giorgio Derchi, Renzo Galanello, Patrizio Bina, Maria Domenica Cappellini, Antonio Piga, Maria-Eliana Lai, Antonella Quarta, Gavino Casu, Silverio Perrotta, Valeria Pinto, Khaled M Musallam, Gian Luca Forni, and Webthal Pulmonary Arterial Hypertension Group*.
• Galliera Hospital, Genoa, Italy (G.D., V.P., G.L.F.); University of Cagliari, Cagliari, Italy (R.G., P.B., M.-E.L.); IRCCS Ca' Granda Foundation Maggiore Policlinico Hospital, University of Milan, Milan, Italy (M.D.C., K.M.M.); University of Turin, Turin, Italy (A.P.); Perrino Hospital, Brindisi, Italy (A.Q.); S. Francesco Hospital, Nuoro, Italy (G.C.); and Seconda University, Naples, Italy (S.P.).
• Circulation. 2014 Jan 21; 129 (3): 338-45.

BackgroundPulmonary 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 ResultsThis 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).ConclusionsThe 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 Urlhttp://www.ClinicalTrials.gov. Unique identifier: NCT01496963.

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