The improvement of pulmonary artery pressure after bosentan therapy in patients with β-thalassemia and Doppler-defined pulmonary arterial hypertension

Abstract

Hossein Karami,1 Hadi Darvishi-Khezri,2 Mehrnoush Kosaryan,1 Rosetta Akbarzadeh,2 Mojdeh Dabirian3 1Department of Pediatrics, Thalassemia Research Center (TRC), Hemoglobinopathy Institute, Mazandaran University of Medical Sciences, Sari, Iran; 2Student Research Committee, Thalassemia Research Center (TRC), Hemoglobinopathy Institute, Mazandaran University of Medical Sciences, Sari, Iran; 3Department of Cardiology, Cardiovascular Research Center, Mazandaran University of Medical Sciences, Sari, Iran Introduction: Pulmonary arterial hypertension (PAH) is relatively prevalent in patients with thalassemia. PAH treatment is necessary as the prevalence of Doppler-estimated PAH and the resultant mortality is high in such patients. Materials and methods: This study aimed at evaluating the effect of bosentan therapy on patients with thalassemia suspected of PAH. Based on pulsed Doppler echocardiography, all the cases were suspected of severe PAH. Consequently, bosentan was initiated at a dose of 62.5 mg twice a day for 4 weeks, which was increased to 62.5–125 mg twice a day, if no adverse side effects were observed. Results: The results of this study showed that pulmonary artery pressure (PAP) decreased after the administration of bosentan in three cases, from 160 to 120, 110 to 65, and 60 to 25 mmHg; in other words, the PAP reduced in the mentioned cases by 25%, 36.4%, and 58.4%, respectively. Conclusion: In this study, PAP improved after bosentan therapy in patients with β-thalassemia suspected of PAH; however, further studies are required to confirm the findings. Keywords: β-thalassemia major, pulmonary arterial hypertension, pulsed Doppler echocardiography, bosentan, tricuspid regurgitation jet velocity, right ventricular pressure

Keywords

• β-thalassemia major
• Pulmonary Arterial Hypertension
• Doppler Pulsed Echocardiography
• Endothelin-1
• Bosentan