Objective: The objective of this study is to review the research on the prognostic markers of idiopathic pulmonary arterial hypertension (IPAH).
Date sources: We searched literature from PubMed and CNKI databases both in English and Chinese up to 2013.
Study selection: Data about mortality and cut-off value are from clinical trials and identified by analysis.
Results: IPAH is an unexplained, progressive, and rare disease characterized by increased pulmonary artery pressure and pulmonary vascular resistance. The diagnosis is difficult, mortality of IPAH is high, and the survival periods are only 2-3 years after diagnosis. Investigations in recent years have identified a range of prognostic markers for IPAH, including the 6-minute walking test, red blood cell distribution width, and platelet levels, as well as imaging findings. Changes in these markers are important sources of information to predict the prognosis of patients with IPAH, which carries significant benefits for treatment planning.
Conclusion: Even though the prognosis of IPAH has been investigated, the mortality is also high. More accurate and meaningful assessment for the prognosis of IPAH is required.