Background: Pulmonary hypertension (PH) is a haemodynamic condition, secondary to different causes. Thalassaemia may lead to PH of different origin and needs a comprehensive analysis to be correctly characterized and possibly treated. Case summary: We present a case study of a patient with a non-transfusion-dependent thalassaemia and a previous diagnosis of group 5 PH. A complete diagnostic assessment led to a specific diagnosis of chronic thromboembolic PH. Thus, we were able to start a specific therapy with riociguat that provided an improvement in terms of haemodynamic, imaging, and functional status. Discussion: A correct characterization and treatment of PH are essential in order to change the patient's prognosis. Chronic thromboembolic PH is a treatable cause of PH in thalassemic patients and should be investigated.

The tailor-made treatment in a particular case of pulmonary hypertension in thalassaemia intermedia : A case report / F.B.M. Blasi, I. Rota, G. Graziadei, M. Vicenzi. - In: EUROPEAN HEART JOURNAL. CASE REPORTS. - ISSN 2514-2119. - 5:6(2021 Jun 16), pp. ytab193.1-ytab193.5. [10.1093/ehjcr/ytab193]

The tailor-made treatment in a particular case of pulmonary hypertension in thalassaemia intermedia : A case report

G. Graziadei;M. Vicenzi
Ultimo
2021-06-16

Abstract

Background: Pulmonary hypertension (PH) is a haemodynamic condition, secondary to different causes. Thalassaemia may lead to PH of different origin and needs a comprehensive analysis to be correctly characterized and possibly treated. Case summary: We present a case study of a patient with a non-transfusion-dependent thalassaemia and a previous diagnosis of group 5 PH. A complete diagnostic assessment led to a specific diagnosis of chronic thromboembolic PH. Thus, we were able to start a specific therapy with riociguat that provided an improvement in terms of haemodynamic, imaging, and functional status. Discussion: A correct characterization and treatment of PH are essential in order to change the patient's prognosis. Chronic thromboembolic PH is a treatable cause of PH in thalassemic patients and should be investigated.
Case report; Chronic thromboembolic pulmonary hypertension; Pulmonary hypertension; Riociguat; Thalassaemia intermedia
Settore MED/11 - Malattie dell'Apparato Cardiovascolare
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Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/2434/873201
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