title: Determinants of pulmonary hypertension in patients with Beta-thalassemia major and normal ventricular function.
abstract: OBJECTIVE
  We sought to define the incidence and predictive factors of pulmonary hypertension in β-thalassemia major.
  
  
  METHODS
  We studied 27 consecutive patients (19 male, 38 ± 9 years of age) with β-thalassemia major. All the patients had normal (left and right) ventricular (systolic and diastolic) function and underwent echocardiographic assessment of pulmonary artery systolic pressure. Univariate regression and discriminant function analyses were used to identify predictive factors of pulmonary hypertension.
  
  
  RESULTS
  Pulmonary hypertension was observed in 18.5% of the patients, but clinically significant disease was detected in only 3.7%. A total of 14 (51.8%) patients had been receiving a combined administration of deferoxamine and deferiprone for 7.0 ± 1.3 years. Amidst a large number of variables examined, ferritin levels and delayed onset of chelation therapy were the only predictors of pulmonary hypertension.
  
  
  CONCLUSIONS
  Pulmonary hypertension in β-thalassemia major is relatively infrequent and generally mild due to improved chelation therapy. The role of hemochromatosis in pulmonary hypertension development merits further study.
authors: Department of Child Health, Hospital of Ioannina, Ioannina, Greece.
authors: Antonios P
authors: AP
authors: Vlahos
authors: Frideriki P
authors: FP
authors: Koutsouka
authors: Nikolaos D
authors: ND
authors: Papamichael
authors: Alexandros
authors: A
authors: Makis
authors: Giannis G
authors: GG
authors: Baltogiannis
authors: Eleni
authors: E
authors: Athanasiou
authors: Nikolaos
authors: N
authors: Chaliasos
authors: Konstantinos L
authors: KL
authors: Bourantas
authors: Theofilos M
authors: TM
authors: Kolettis
mesh_terms: D000328:Adult; D016022:Case-Control Studies; D002614:Chelating Agents / Q000627:therapeutic use; D004452:Echocardiography; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D017086:beta-Thalassemia / Q000188:drug therapy / Q000503:physiopathology*
chemical_list: D002614:Chelating Agents