Journal article
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
Pulmonary circulation, Vol.4(3), pp.520-526
09/01/2014
DOI: 10.1086/677367
PMCID: PMC4278612
PMID: 25621166
Abstract
Pulmonary arterial hypertension (PAH) is a potentially life-threatening complication of thalassemia. A sexagenarian with β-thalassemia intermedia presented with new-onset dyspnea and syncope. Right heart catheterization confirmed severe PAH. Her functional class IV symptoms and severely elevated mean pulmonary artery pressure prompted the initiation of continuous epoprostenol therapy. Clinical follow-up documented significant improvement in functional class, 6-minute walk distance, and right ventricular size and function as well as pulmonary arterial pressure on echocardiogram. At the patient’s request, epoprostenol was down-titrated and eventually discontinued. The patient was then safely transitioned to nifedipine therapy after verification of vasoresponsiveness.
Details
- Title: Subtitle
- 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
- Creators
- Kamonpun Ussavarungsi - Pulmonary and Critical Care Medicine, Mayo Clinic, Jacksonville, Florida, USACharles D Burger - Pulmonary and Critical Care Medicine, Mayo Clinic, Jacksonville, Florida, USA
- Resource Type
- Journal article
- Publication Details
- Pulmonary circulation, Vol.4(3), pp.520-526
- Publisher
- University of Chicago Press
- DOI
- 10.1086/677367
- PMID
- 25621166
- PMCID
- PMC4278612
- ISSN
- 2045-8932
- eISSN
- 2045-8940
- Language
- English
- Date published
- 09/01/2014
- Academic Unit
- Pulmonary, Critical Care, and Occupational Medicine; Internal Medicine
- Record Identifier
- 9984094368802771
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