Clinical Characteristic And Survival Of Patients With Idiopathic Pulmonary Arterial Hypertension In Different Treatment Era:Results From Beijing And Shanghai Registry

Introduction:Pulmonary Arterial Hypertension(IPAH)were characterized as a potentially fatal pulmonary vascular disease with pulmonary vascular remodeling which lead to increase in pulmonary vessel resistance,right heart remodeling and worse prognosis.Idiopathic Pulmonary Arterial Hypertension were characterized as a Pulmonary Arterial Hypertension.Multiple IPAH registries from different centers around the world were established since 1980s.Baseline characteristics and long-term prognosis were described.European IPAH registries in 2000s as well as 2010s revealed increased in age comparing to NIH registries in 1980s.Elderly patients with IPAH and its differences in survival and clinical presentation were compared with IPAH patients with younger age.Our center had published data from Chinese IPAH registries from 1999-2004,and 2007-2009,where combination therapy was not available yet,long-term survival of Chinese IPAH registries were also under investigation.Method:In this registry study,IPAH patients from Shanghai Pulmonary Hospital were collected between 2010 to 2012 as Shanghai cohort,IPAH patients form Fuwai Hospital&Chinese Academy of Medical Science were collected between 2013 to 2018 as Beijing cohort.A total number of 540 patients were collected.Beijing cohort was separated into 18-30,30-40,40-50,and 50-years.Clinical characteristics,hemodynamic profile as well as treatment were compared between Beijing and Shanghai registry.Survival analysis were estimated after follow-up in different cohorts.Primary endpoints were defined as cardiovascular death and heart lung transplantation.Kaplan-Meier analysis was used for survival analysis.Baseline characteristics and long-term survival of Beijing and Shanghai cohorts were compared with Chinese 1999-2004,2007-2009 published data,East Asian IPAH registries(Japan,Korea,Singapore)and European registries(Sweden,UK-Irish,COMPERA).Result:Median age of Shanghai Registry and Beijing Registry were 36 years and 33 years with no major difference comparing to 1999-2004,2007-2009 Chinese registry data.Comparing to Beijing cohort,IPAH patients from Shanghai cohort present were older,worse in heart function and lower in 6-minute walking distance.In hemodynamic analysis,decrease in mPAP and PVR were seen when comparing data from 99-04,07-09,Shanghai and Beijing registries,although NT-proBNP level were increased in these studies.Elderly patients present with more comorbidities,worse heart function,and lower mPAP levels.One-drug treatment was the major therapeutic option in Shanghai registry;however,dual therapy was the major therapeutic option in Beijing registry.Baseline data of Chinese IPAH cohorts were similar with younger IPAH subgroups in European cohorts.As for survival analysis,five-and ten-year survival rate in Shanghai cohort were 55.5%and 37.7%,3-and 5-year survival rate in Beijing registry were 88.9%and 74.1%,which increase dramatically comparing to previous Chinese studies.No major difference was seen between Beijing studies and East-Asian,European registries.Conclusion:Increase of age were not observed in Beijing and Shanghai cohort.Elder IPAH patients present with poorer tolerance of high pulmonary vascular resistance and lower heart function.Although early diagnosis and enhancement of drug availability were appeared in the last 10 years,long-term survival of IPAH were still poor.However,dramatic increase of 3 to 5-year survival were seen in the more recent Beijing cohort.Definition and description of elderly IPAH and its baseline characteristics and survival were urgently needed.