Sickle cell disease related chronic thromboembolic pulmonary hypertension: challenging clinical scenario.
Balloon pulmonary angioplasty
Chronic thromboembolic pulmonary hypertension
Endothelin receptor antagonist
Oral prostacyclin agonist
Sickle cell disease
Journal
Journal of thrombosis and thrombolysis
ISSN: 1573-742X
Titre abrégé: J Thromb Thrombolysis
Pays: Netherlands
ID NLM: 9502018
Informations de publication
Date de publication:
Feb 2022
Feb 2022
Historique:
accepted:
18
05
2021
pubmed:
28
5
2021
medline:
7
4
2022
entrez:
27
5
2021
Statut:
ppublish
Résumé
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.
Identifiants
pubmed: 34043152
doi: 10.1007/s11239-021-02483-7
pii: 10.1007/s11239-021-02483-7
doi:
Types de publication
Case Reports
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
467-470Informations de copyright
© 2021. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.
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