Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2018 Dec 17:12:1-7.
doi: 10.2147/IMCRJ.S180602. eCollection 2019.

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

Hossein Karami  1 , Hadi Darvishi-Khezri  2 , Mehrnoush Kosaryan  1 , Rosetta Akbarzadeh  2 , Mojdeh Dabirian  3
Affiliations

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

Hossein Karami et al. Int Med Case Rep J. .

Abstract

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: bosentan; pulmonary arterial hypertension; pulsed Doppler echocardiography; right ventricular pressure; tricuspid regurgitation jet velocity; β-thalassemia major.

PubMed Disclaimer

Conflict of interest statement

Disclosure All authors declared no conflicts of interest in this work and that there was no source of extra institutional commercial funding, and the entire study was performed without external funding.

References

    1. Aessopos A, Farmakis D, Karagiorga M, et al. Cardiac involvement in thalassemia intermedia: a multicenter study. Blood. 2001;97(11):3411–3416. - PubMed
    1. Badesch DB, Champion HC, Sanchez MA, et al. Diagnosis and assessment of pulmonary arterial hypertension. J Am Coll Cardiol. 2009;54(1 Suppl):S55–S66. - PubMed
    1. Chueamuangphan N, Wongtheptien W, Nawarawong W, Sukornthasarn A, Chuncharunee S, Tawichasri C, Patumanond J. Clinical indicators for pulmonary arterial hypertension in thalassemia. J Med Assoc Thai. 2012;95(1):16. - PubMed
    1. Ussavarungsi K, Burger CD. Pulmonary arterial hypertension in a patient with β-thalassemia intermedia and reversal with infusion epoprostenol then transition to oral calcium channel blocker therapy: review of literature. Pulm Circ. 2014;4(3):520–526. - PMC - PubMed
    1. Taher AT, Musallam KM, Karimi M, et al. Overview on practices in thalassemia intermedia management aiming for lowering complication rates across a region of endemicity: the OPTIMAL CARE study. Blood. 2010;115(10):1886–1892. - PubMed