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

• Jang SY
• Kim EK
• Huh J
• Song J
• Kang IS
• Park SW
• Kim DK
• Chang SA

• KMID: 2526976
• DOI: http://doi.org/10.3346/jkms.2022.37.e80

Abstract

Background
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.
Methods
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.
Results
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.
Conclusion
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.

Keyword

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

Figure

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

Reference

1. Simonneau G, Gatzoulis MA, Adatia I, Celermajer D, Denton C, Ghofrani A, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2013; 62(25):Suppl. D34–D41. PMID: 24355639.

2. National Health Insurance Sharing Service. Updated 2022. Accessed June 25, 2010. https://nhiss.nhis.or.kr/bd/ab/bdaba011eng.do .

3. Jang SY, Park TK, Kim DK. Survival and causes of death for Takayasu’s arteritis in Korea: a retrospective population-based study. Int J Rheum Dis. 2021; 24(1):69–73. PMID: 33089946.

4. Benza RL, Miller DP, Barst RJ, Badesch DB, Frost AE, McGoon MD. An evaluation of long-term survival from time of diagnosis in pulmonary arterial hypertension from the REVEAL Registry. Chest. 2012; 142(2):448–456. PMID: 22281797.

5. Gall H, Felix JF, Schneck FK, Milger K, Sommer N, Voswinckel R, et al. The giessen pulmonary hypertension registry: survival in pulmonary hypertension subgroups. J Heart Lung Transplant. 2017; 36(9):957–967. PMID: 28302503.

6. Escribano-Subias P, Blanco I, López-Meseguer M, Lopez-Guarch CJ, Roman A, Morales P, et al. Survival in pulmonary hypertension in Spain: insights from the Spanish registry. Eur Respir J. 2012; 40(3):596–603. PMID: 22362843.

7. Skride A, Sablinskis K, Lejnieks A, Rudzitis A, Lang I. Characteristics and survival data from Latvian pulmonary hypertension registry: comparison of prospective pulmonary hypertension registries in Europe. Pulm Circ. 2018; 8(3):2045894018780521. PMID: 29767576.

8. Strange G, Lau EM, Giannoulatou E, Corrigan C, Kotlyar E, Kermeen F, et al. Survival of idiopathic pulmonary arterial hypertension patients in the modern era in Australia and New Zealand. Heart Lung Circ. 2018; 27(11):1368–1375. PMID: 29029950.

9. Ogawa A, Satoh T, Tamura Y, Fukuda K, Matsubara H. Survival of Japanese patients with idiopathic/heritable pulmonary arterial hypertension. Am J Cardiol. 2017; 119(9):1479–1484. PMID: 28267959.

10. Fischler M, Speich R, Dorschner L, Nicod L, Domenighetti G, Tamm M, et al. Pulmonary hypertension in Switzerland: treatment and clinical course. Swiss Med Wkly. 2008; 138(25-26):371–378. PMID: 18587689.

11. D’Alonzo GE, Barst RJ, Ayres SM, Bergofsky EH, Brundage BH, Detre KM, et al. Survival in patients with primary pulmonary hypertension. Results from a national prospective registry. Ann Intern Med. 1991; 115(5):343–349. PMID: 1863023.

12. Chun KJ, Kim SH, An BJ, Kim SH, Ha JK, Hong TJ, et al. Survival and prognostic factors in patients with primary pulmonary hypertension. Korean J Intern Med. 2001; 16(2):75–79. PMID: 11590905.

13. Song S, Lee SE, Oh SK, Jeon SA, Sung JM, Park JH, et al. Demographics, treatment trends, and survival rate in incident pulmonary artery hypertension in Korea: a nationwide study based on the health insurance review and assessment service database. PLoS One. 2018; 13(12):e0209148. PMID: 30566510.

14. Lee WT, Ling Y, Sheares KK, Pepke-Zaba J, Peacock AJ, Johnson MK. Predicting survival in pulmonary arterial hypertension in the UK. Eur Respir J. 2012; 40(3):604–611. PMID: 22556026.

15. Santos M, Gomes A, Cruz C, Rocha J, Ricardo M, Gonçalves F, et al. Long-term survival in pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: Insights from a referral center in Portugal. Rev Port Cardiol (Engl Ed). 2018; 37(9):749–757. PMID: 30144959.

16. Mosca L, Barrett-Connor E, Wenger NK. Sex/gender differences in cardiovascular disease prevention: what a difference a decade makes. Circulation. 2011; 124(19):2145–2154. PMID: 22064958.

17. Shin HY, Lee JY, Song JH, Lee SM, Lee JH, Lim BS, et al. Cause-of-death statistics in the Republic of Korea,2014. J Korean Med Assoc. 2016; 59(3):221.

18. Jang SY, Huh J, Kim EK, Chang SA, Song J, Kang IS, et al. Impact of atrial fibrillation on survival in adults with congenital heart disease: a retrospective population-based study. J Korean Med Sci. 2021; 36(5):e43. PMID: 33527785.

19. Benza RL, Gomberg-Maitland M, Elliott CG, Farber HW, Foreman AJ, Frost AE, et al. Predicting survival in patients with pulmonary arterial hypertension: the REVEAL risk score calculator 2.0 and comparison with ESC/ERS-based risk assessment strategies. Chest. 2019; 156(2):323–337. PMID: 30772387.

20. Benza RL, Miller DP, Gomberg-Maitland M, Frantz RP, Foreman AJ, Coffey CS, et al. Predicting survival in pulmonary arterial hypertension: insights from the Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL). Circulation. 2010; 122(2):164–172. PMID: 20585012.

21. Chung WJ, Park YB, Jeon CH, Jung JW, Ko KP, Choi SJ, et al. Baseline characteristics of the Korean registry of pulmonary arterial hypertension. J Korean Med Sci. 2015; 30(10):1429–1438. PMID: 26425039.