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). METHODSï¼ 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 KaplanâMeier 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. RESULTSï¼ 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]: 1.117-1.386, P < 0.001). Compared with those with PDW < 16.1%, the HR for all-cause death increased by 5.278 (95% CI: 2.711-10.276, P < 0.0001) among patients with PDW ⥠16.1 %. 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.