Survival rate and death risk for associated pulmonary arterial hypertension A retrospective population-based study

Purpose: This study aimed to assess the survival rate (SR) and death risk for associated pulmonary arterial hypertension (aPAH; 10th revision of the International Statistical Classification of Diseases [ICD-10], I27.2) in Koreans. Methods: The data were collected from the Korean National Health Insurance Service from 2006 through 2017 (n=15,448). We analyzed the SR using the Kaplan-Meier method and carried out Cox proportional hazards analyses. Results: Patients' mean age upon aPAH diagnosis was 60.1 +/- 24.0 years, and 60.7% of the patients were female. The 10-year SR of aPAH was 46.3% (95% confidence interval, 45.0 to 47.6). The factors associated with an increase in the adjusted death risk included age of 0 to 9 years, advancing age, male sex, lower income level, and comorbidities including diabetes mellitus, myocardial infarction, heart failure, hemorrhagic stroke, chronic kidney disease, malignant neoplasm, hereditary hemorrhagic telangiectasia, and systemic lupus erythematosus. Conclusion: The 10-year SR of aPAH was over 46%. The risk of death from aPAH was significantly higher with advancing age, sex, lower income level, and comorbidities.