J Korean Med Sci.  2022 Mar;37(10):e80. 10.3346/jkms.2022.37.e80.

A Retrospective Population-Based Survival Study of Idiopathic Pulmonary Arterial Hypertension in Korea

  • 1Division of Cardiology, Department of Medicine, Heart Vascular Stroke Institute, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
  • 2Division of Cardiology, Department of Pediatrics, Adult Congenital Heart Clinic, Heart Vascular Stroke Institute, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea


Few studies used nationwide data to assess the survival rates (SRs) and death risk for idiopathic pulmonary arterial hypertension (IPAH; ICD-10 I27.0) in Korea.
IPAH data (N = 9,017; female:male = 6:4) were collected from the National Health Insurance Service in Korea, from 2006 through 2017. The data consisted of primary diagnoses related to IPAH. The Kaplan–Meier method and Cox proportional-hazards analyses were carried out.
The mean age was 62.3 (± 19.4) years, 64.2 (± 18.9) years in female and 59.4 (± 19.8) years in male (P < 0.001). The one-, three-, five- and 10-year SRs for IPAH were 89.0%, 79.8%, 72.3% and 57.0%, respectively. The adjusted hazard ratio (HR) of IPAH was 1.81 (95% confidence interval [CI], 1.26–2.59) in 60–69 age group, 3.42 (95% CI, 2.40–4.87) in 70–79, and 7.73 (95% CI, 5.43–11.0) in 80s. Other risk factors were male, low-income status, diabetes, myocardial infarction, atrial fibrillation, ischemic stroke, hemorrhagic stroke, and malignant neoplasm.
The 10-year SR of IPAH was 57% in Korea. The HR for IPAH was significantly high in patients with older age and other risk factors.


Survival Rates; Death; Risk; Causes of Death; Idiopathic Pulmonary Arterial Hypertension


  • Fig. 1 Survival curve of idiopathic pulmonary arterial hypertension (IPAH) in Koreans. (A)Survival rates overall and by sex and (B) by age group.


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