BackgroundFew published studies have evaluated the power of the oxygen uptake efficiency slope (OUES) to predict outcomes in patients with idiopathic pulmonary arterial hypertension (IPAH), who typically die of right‐sided heart failure. Our study sought to evaluate the power of OUES to predict clinical worsening and mortality in patients with IPAH.Methods and ResultsPatients with newly diagnosed IPAH who underwent symptom‐limited cardiopulmonary exercise testing from November 11, 2010, to June 25, 2015, in our hospital were prospectively enrolled and followed for up to 66 months. Clinical worsening and mortality were recorded. A total of 210 patients with IPAH (159 women; mean age, 32±10 years) were studied with a median follow‐up of 41 months. Thirty‐one patients died, 1 patient underwent lung transplantation, and 85 patients presented with clinical worsening. The univariate analysis revealed that OUES, OUESI (OUESI=OUES/body surface area), peak oxygen uptake (normalV˙normalO2), peak normalV˙normalO2/kg, ventilation (normalV˙E)/carbon dioxide output (normalV˙CO2) slope, peak systolic blood pressure, heart rate recovery, pulmonary vascular resistance, cardiac index, N‐terminal prohormone brain natriuretic peptide, and World Health Organization functional class were all predictive of clinical worsening and mortality (all P<0.05). Multivariate analysis demonstrated that OUESI and cardiac index were independently predictive of clinical worsening, and OUESI and N‐terminal prohormone brain natriuretic peptide were independently predictive of mortality. Patients with OUESI ≤0.52 m−2 had a worse 5‐year survival rate than patients with OUESI >0.52 m−2 (41.9% versus 89.8%, P<0.0001).ConclusionsThe OUES, a submaximal parameter obtained from cardiopulmonary exercise testing, provides prognostic information for predicting clinical worsening and mortality in patients with IPAH.