Abstract
The aim of our study was to describe the efficacy of addition of intravenous or subscutaneous prostanoids in idiopathic pulmonary arterial hypertension (PAH) patients deteriorating on bosentan or on bosentan-sildenafil. PAH treatment at our hospital is standardized with first-line oral therapy in New York Heart Association class III patients followed by addition of prostanoids on clinical worsening. Mean improvement in 6-minute walk distance after 4 months of prostanoids was 86 m (p < 0.01) in the bosentan group versus 41 m (p < 0.05) in the bosentan-sildenafil group, and these improvements persisted at long-term follow-up. From these results we conclude that addition of subcutaneous or intravenous prostanoids can be efficacious in PAH deteriorating on oral therapy.
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