Sickle cell disease related chronic thromboembolic pulmonary hypertension: challenging clinical scenario.
J Thromb Thrombolysis
; 53(2): 467-470, 2022 Feb.
Article
em En
| MEDLINE
| ID: mdl-34043152
ABSTRACT
Sickle cell disease (SCD), a haemoglobinopathy characterized by chronic haemolysis with acute exacerbations and vascular occlusion episodes, may be complicated by pulmonary hypertension. The latter may be caused by chronic thromboembolic disease of pulmonary artery branches and its management is not well-defined. Herein, we present a case of SCD complicated by chronic thromboembolic pulmonary hypertension of subsegmental pulmonary artery branches successfully treated with endothelin receptor antagonists, orally administered prostacyclin analogs and balloon pulmonary angioplasty. This challenging case highlights the need for clinical awareness of chronic thromboembolic pulmonary hypertension as a specific and potentially curable form of pulmonary hypertension complicating SCD course that may necessitate combined pharmacologic and interventional management.
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Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Embolia Pulmonar
/
Angioplastia com Balão
/
Hipertensão Pulmonar
/
Anemia Falciforme
Tipo de estudo:
Diagnostic_studies
/
Etiology_studies
Limite:
Humans
Idioma:
En
Ano de publicação:
2022
Tipo de documento:
Article