| The Long-term Survival in Patients with Chronic Thromboembolic Pulmonary Hypertension:Experience from a Single Center in ChinaObjective:To analysis the long-term prognosis of patients with chronic thromboembolic pulmonary hypertension(CTEPH)receiving different treatments in modern era of CTEPH treatment.Methods:From May 2013 to May 2019,patients who diagnosed with CTEPH in Fuwai Hospital were included in the study.The long-term overall survival of CTEPH patients receiving different treatments,including pulmonary endarterectomy(PEA),balloon pulmonary angioplasty(BPA),and targeted drug,was analyzed by Kaplan-Meier method.Moreover,the effect of the main treatment modality on patients’ survival was also analyzed using Cox proportional hazards model.Results:A total of 364 patients diagnosed with CTEPH were retrospectively included,14 patients were lost during follow-up.Among 350 patients included in the final analysis:123 underwent pulmonary endarterectomy(PEA),121 received balloon pulmonary angioplasty(BPA),and 106 treated with targeted drug alone.The median period of followup was 51.2 months,the estimated survival at 1-,3-,5-and 7-year was 97.1%,93.3%,86.9%,and 82.0%for the whole cohort;100%,99.20%,96.5%and 92.5%in PEA group;98.4%,97.4%,95.3%and 89.3%in BPA group;92.5%,81.9%,70.1%and 66.8%in patients who receive targeted drug alone.In comparing with targeted treatment along,results of multivariate Cox analysis after adjusting the confounders showed that receiving PEA decreased the risk of death by 83%(HR:0.17,5%CI:0.07-0.44)and receiving BPA decreased the risk of death by 89%(HR:0.11,95%CI:0.04-0.29).Conclusion:The estimated survival of CTEPH patients at 1-,3-,5-and 7-year was 97.1%,93.3%,86.9%,and 82.0%respectively.The intervenes methods of revascularization,including PEA and BPA,were preferred than treatment with targeted drug alone in the view of long-term prognosis of patients with CTEPH.Development and Validation of a Nomogram for Predicting the Long-term Survival in Patients with Chronic Thromboembolic Pulmonary HypertensionObjective:There remains a lack of prognosis models for patients with chronic thromboembolic pulmonary hypertension(CTEPH).This study aims to develop a nomogram predicting 3-,5-and 7-year survival in patients with CTEPH and verify the prognostic model.Methods:Patients with CTEPH diagnosed in Fuwai Hospital were enrolled consecutively between May 2013 and May 2019.Among them,70%were randomly split into the training sett and the other 30%as validation set for external validation.Cox proportional hazards model was used to identify the potential survival-related factors which were candidate variables for establishment of nomogram and the final model was internally validated by bootstrap method.Results:A total of 350 patients were included in the final analysis and the median followup our whole cohort was 51.2 months.Multivariate analysis of Cox proportional hazards regression showed body mass index(HR=0.90,95%CI:0.82-0.99,P=0.042),mean right atrial pressure(HR=1.10,95%CI:1.04-1.17,P=0.001),N-terminal pro brain natriuretic peptide(per 500 ng/ml increase in concentration,HR=1.10,95%CI:1.03-1.18,P=0.008),presence of anemia(HR=4.95,95%CI:2.05-11.97,P<0.001)and main treatment choice(targeted drug alone as reference,PEA,HR=0.15,95%CI:0.06-0.38,P<0.001;BPA,HR=0.06,95%CI:0.02-0.19,P<0.001)were the independent risk factors of mortality.The nomogram demonstrated good discrimination with the corrected C-index of 0.82 in training set,and the C-index of 0.80(95%CI:0.70-0.91)in the external validation set.The calibration plots also showed a good agreement between predicted and actual survival in both training and validation sets.Conclusions:We developed an easy-to-use nomogram with good apparent performance using five readily available variables,which will help physicians to identify CTEPH at high risk for poor prognosis and implement medical interventions. |
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