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Journal of Rare Cardiovascular Diseases
ISSN: 2299-3711 (Print)
e-ISSN: 2300-5505 (Online)
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Pulmonary artery pressure matters – how to efficiently improve survival in pulmonary arterial hypertension (RCD code: II‑1A.1)
Hiromi Matsubara
,  
Piotr Błaszczak
,  
Piotr Podolec
,  
Grzegorz Kopeć
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Abstract
Pulmonary arterial hypertension is a disease characterized by poor prognosis despite treatment. Even in a modern era of pharmaco therapy there is a strong need to further improve survival of patients. The current therapeutic strategies do not offer a real break-through in terms of reducing mortality. In a search for better efficacy of treatment we discuss a strategy based on lowering pulmonary artery pressure as much as possible with epoprostenol in monotherapy or in combination. Epoprostenol with bosentan is an effective tool in achieving this goal. A rapid up-titration regardless of maximum epoprostenol dose achieved gives additional long-term benefit. Practical issues related to such modality of treatment are also discussed
Keywords
pulmonary arterial hypertension, treatment, epoprostenol, outcome
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Classification of Rare Cardiovascular Diseases anticoagulation atrial fibrillation atrial septal defect cardiomyopathy computed tomography congenital heart disease echocardiography electrocardiogram electrocardiography heart failure implantable cardioverter‑defibrillator magnetic resonance imaging pregnancy pulmonary arterial hypertension pulmonary hypertension rare cardiovascular disease rare disease right heart catheterization right ventricular failure
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