Observational studyBMC pulmonary medicine2025
Ten-year survival, trends and risk stratification of newly diagnosed idiopathic pulmonary arterial hypertension in China: insights from a national multicenter prospective registry.
Observational study in BMC pulmonary medicine, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT01417338 (National, Prospective, Multicenter,Observational Registry Study on Pulmonary Arterial Hypertension or Chronic Thromboembolic Pulmonary Hypertension in China), which is not on this map. Not yet cited in PubMed.
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The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
National, Prospective, Multicenter,Observational Registry Study on Pulmonary Arterial Hypertension or Chronic Thromboembolic Pulmonary Hypertension in China
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0 citing papers in PubMed.
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Corrections and comments
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Authors and funding
15 authors.
Funding
Abstract
backgroundData on the 10-year survival of incident IPAH in China is lacking. The changing trend of characteristics and survival of IPAH over the past decade has yet been demonstrated. The 4-stratum risk model needs further validation in different cohorts.
methodsIncident cases of IPAH identified in the national multicenter prospective registry of PAH from 2009 to 2019 were analyzed. Patients with at least two of the three acquired variables (WHO-FC, 6MWD, NT-pro BNP/BNP) were included for risk stratification.
results605 incident patients with IPAH were included (age: 35 ± 12 years; 75.2% females). The 1-, 3-, 5-, and 10-year survival of the whole cohort was 95.4%, 84.3%, 72.3%, and 53.4%, respectively. Aside from traditional prognostic factors of PAH, TG/HDL-c ratio > 1.4 was independently associated with 10-year mortality (HR 1.458). Compared with 2009–2014, patients enrolled in 2015–2019 showed shorter diagnostic delays and less severe diseases at baseline. The 1-year and 3-year survival improved over time, with increased prescription of targeted therapy and initial combination therapy. With the 4-stratum risk model, patients classified into different risk groups showed significantly different survival at both baseline (C-statistic 0.66) and follow-up (C-statistic 0.83) when three variables were available.
conclusionsThe 10-year survival of IPAH patients in China has shown improvement, though further gains are needed. TG/HDL-c > 1.4 was associated with increased10-year mortality. Over the past decade, advances in the management of IPAH have contributed to better survival. The 4-stratum model demonstrated a good performance in Chinese patients with IPAH when three variables were available.
trial registrationNo. NCT01417338 (Registration Date 200908).
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