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BACKGROUND: Platelet distribution width (PDW) has been recognized as risk predictors of idiopathic pulmonary arterial hypertension. This study aims to investigate whether in-hospital PDW would be useful to predict all-cause death in patients with severe pulmonary hypertension due to chronic lung diseases (CLD-PH).METHODS: Early in-hospital PDW was measured in 67 severe CLD-PH patients who were confirmed by right heart catheterization and followed up. Event-free survival was estimated using the Kaplan–Meier method and analyzed with the log-rank test. Cox proportional hazards models were performed to determine the association between the PDW level and all-cause death.RESULTS: Pulmonary function test and echocardiography parameters were different among patients divided by 17% (the upper reference range of the PDW). There were no significant differences in both clinical variables and RHC parameters among patients with PDW ≥ or < 17%. During median of 2.4 (2.5, 3.7) years of follow-up, 44 patients died. A significant association was noted between in-hospital PDW level and the adjusted risk of all-cause mortality (hazard ratio [HR], 1.245; 95% confidence interval [CI], 1.099-1.409). Compared with those with PDW < 17%, the HR for all-cause death increased to 5.067 (95% CI: 2.420-10.609, P < 0.001) among patients with PDW ≥ 17 %. Higher levels of PDW were also associated with increased risk of all-cause death.CONCLUSIONS: In-hospital PDW was independently associated with all-cause death in patients with severe CLD-PH. This potentially could be used to estimate the severity of severe CLD-PH.