TY - JOUR T1 - Baseline characteristics of idiopathic PAH patients with severely reduced diffusion capacity JF - European Respiratory Journal JO - Eur Respir J VL - 40 IS - Suppl 56 SP - 370 AU - Pia Trip AU - Esther Nossent AU - Anco Boonstra AU - Harm Jan Bogaard AU - Frances de Man AU - Nico Westerhof AU - Katrien Grunberg AU - Anton Vonk-Noordegraaf Y1 - 2012/09/01 UR - http://erj.ersjournals.com/content/40/Suppl_56/370.abstract N2 - Introduction: Pulmonary arterial hypertension (PAH) is a disease affecting the vasculature of the lung and is known to decrease carbon monoxide diffusion capacity (DLCO). Although most patients have a moderately decreased DLCO, a group of patients has severely reduced DLCO. The reason for this low DLCO is unclear. Therefore, the aim of this study is to describe baseline characteristics of idiopathic PAH patients with a low DLCO and compare this group with a group of idiopathic PAH patients with moderate low or normal DLCO.Methods: Patients with idiopathic or familial PAH were included. Patients were grouped based on the finding of a bimodal distribution in DLCO-values.Results: A total of 170 patients were included. DLCO groups were DLCO <45% (N=58) and ≥45% of predicted (N=112). Group characteristics are shown in table 1.Conclusions: Compared with IPAH patients with moderate low or normal DLCO, IPAH patients with a severely reduced DLCO were characterized at baseline by a higher age, increased number of males, more often a history of smoking and coronary disease, and a lower exercise performance. No differences however were found in hemodynamic parameters. ER -