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Objective: Atrial septostomy (AS) is a palliative treatment for right ventricular failure from severe pulmonary arterial hypertension (PAH). We sought to investigate the effect of AS, alone or combined with PAH-specific pharmacotherapy, on the survival of patients with PAH. Methods: We performed a retrospective analysis of the functional and hemodynamic changes in patients with PAH following AS and of long-term survival characteristics for the whole group and separately for the subgroup who received post-procedural pharmacotherapy. Results: Fifty procedures performed in 34 patients (age: 35 ± 10 years) resulted in hemodynamic and symptomatic improvement in most. Only one procedure-related death occurred (2%). Due to spontaneous closure of the defect, AS was repeated in 10 patients. In 21 patients, AS was the only form of treatment while eleven received additional pharmacotherapy after AS. During follow-up (mean 58.5 ± 38 months) 21 patients died; median survival of the group was 60 months (95%CI, 43 to 77). Media