Determinants of Pulmonary Hypertension in Patients with Beta-Thalassemia Major and Normal Ventricular Function

作者：Vlahos Antonios R; Koutsouka Frideriki P; Papamichael Nikolaos D; Makis Alexandros; Baltogiannis Giannis G; Athanasiou Eleni; Chaliasos Nikolaos; Bourantas Konstantinos L; Kolettis Theofilos M^*

来源：Acta Haematologica, 2012, 128(2): 124-129.

DOI：10.1159/000338825

• Chelation therapy
• Ferritin
• Hemochromatosis
• Pulmonary hypertension
• beta-Thalassemia major

Background/Aims: We sought to define the incidence and predictive factors of pulmonary hypertension in beta-thalassemia major. Methods: We studied 27 consecutive patients (19 male, 38 +/- 9 years of age) with beta-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. Conclusion: Pulmonary hypertension in beta-thalassemia major is relatively infrequent and generally mild due to improved chelation therapy. The role of hemochromatosis in pulmonary hypertension development merits further study.

• 出版日期2012