Objective To assess the effects of lung transplantation (LT) in the treatment of end-stage pulmonary arterial hypertension (PAH) and evaluated its impact on quality of life (QoL). Methods A total of 54 patients with end-stage PAH admitted to the LT center of Wuxi People’s Hospital between January 2007 and June 2019 were retrospectively analyzed. The patients were divided into surgical and non-surgical groups. Clinical data were compared between the groups. The clinical data such as symptoms, signs, biochemistry, survival rate and quality of life were compared between the two groups. The effects of lung transplantation on survival and quality of life were analyzed. The quality of life was scored by SF-36 scale. Results A total of 54 patients with end-stage pulmonary arterial hypertension who met the indications for lung transplantation were enrolled. Among them, 36 patients underwent lung transplantation and 18 patients did not undergo surgery. There were no statistical differences in gender, course of disease, disease type, symptoms between the two groups. The uric acid of the non-operation group was significantly higher than that of the lung transplantation group (P=0.014). The systolic pulmonary artery pressure (SPAP) of the non-operation group was significantly lower than that of the lung transplantation group (P=0.046). Kaplan-Meier survival curve showed that the survival rate of the operation group was higher than that of the non-operation group (P=0.0087). The quality of life assessment showed that after lung transplantation, the physical component summary and mental component summary scores of operation group were higher than those before operation (P<0.01), and also higher than non-operation group in the same period (P<0.05). Preoperative low systemic circulation blood pressure, poor cardiac function, high NT-proBNP and high uric acid may be the risk factors for early death of patients with pulmonary arterial hypertension after lung transplantation. Conclusions Lung transplantation can effectively improve survival and also QoL for patients with end-stage PH. Early detection of risk factors and preoperative assessment can help improve the perioperative survival.