Intravenous prostanoids in systemic sclerosis-associated pulmonary arterial hypertension: a single-centre experience.

Objectives: The current study evaluates survival rates among SSc-associated pulmonary arterial hypertension (SSc-PAH) patients on i.v. prostanoids, and short-term impact of i.

Ambulatory prostanoid therapy: safe reduction in duration of inpatient training.

Aims: IV prostanoid therapy for advanced pulmonary arterial hypertension requires lifelong, continuous infusion, and extensive self-care. The inpatient training pathway (ITP) ensures patient competency but can be psychologically and physically demanding. Therefore, an alternative Elective Prostanoid Admission Pathway (EPAP) was developed.

Optical coherence tomography evaluation of pulmonary arterial vasculopathy in Systemic Sclerosis.

Our current understanding of the pathophysiology of pulmonary vascular disease is incomplete, since information about alterations of the pulmonary vasculature in pulmonary arterial hypertension (PAH) is primarily provided by autopsy or tissue specimens. The aim of this study was to compare the distal pulmonary vasculature of <2 mm in diameter in Systemic Sclerosis (SSc) patients with (n = 17) and without (n = 5) associated PAH using Optical Coherence Tomography during Right Heart catheterization. SSc-PAH patients showed significant thickening of Intima Media Thickening Area compared to patients without PAH (27 +/- 5.