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The outcome of patients with pulmonary arterial hypertension (PAH) who undergo mechanical ventilation is not well known.The Nationwide Inpatient Sample for 2006 to 2012 was used to isolate patients with a diagnosis of PAH who also underwent invasive (MV) and noninvasive (NIMV) mechanical ventilation. The primary outcome was in-hospital mortality.The hospital records of 55 208 382 patients were studied, and there were 21 070 patients with PAH, of whom 1646 (7.8%) received MV and 834 (4.0%) received NIMV. Those receiving MV had higher mortality (39.1% vs 12.6%, P < .001) and longer hospital stays (11.9 days, interquartile range [IQR] 6.1-22.2 vs 6.7 days, IQR 3.4-11.9, P < .001) than those undergoing NIMV. Of the patients treated with MV, 4.4% also used home oxygen therapy and had similar overall mortality to those who did not use home oxygen (35.3% vs 39.1%, P = .46). Similarly, there was no relationship between home oxygen use and mortality in patients treated with NIMV (10.6% vs 12.6%, P = .48). Notably, more patients treated with NIMV used home oxygen than those treated with MV (14.4% vs 4.4%, P < .001).Patients with PAH who undergo invasive mechanical ventilation have an in-hospital mortality of 39.1%. Future work may help identify the types of patients who benefit most from advanced respiratory support in a critical care setting.