Korean Circ J.  2023 May;53(5):313-327. 10.4070/kcj.2022.0231.

Epidemiology of PAH in Korea: An Analysis of the National Health Insurance Data, 2002–2018

• Jang AY
• Lee HH
• Lee H
• Kim HC
• Chung WJ

• KMID: 2542301
• DOI: http://doi.org/10.4070/kcj.2022.0231

Abstract

Background and Objectives
Pulmonary arterial hypertension (PAH) is a rare but fatal disease. Recent advances in PAH-specific drugs have improved its outcomes, although the healthcare burden of novel therapeutics may lead to a discrepancy in outcomes between developing and developed countries. We analyzed how the epidemiology and clinical features of PAH has changed through the rapidly advancing healthcare infrastructure in South Korea.
Methods
PAH was defined according to a newly devised 3-component algorithm. Using a nationwide health insurance claims database, we delineated annual trends in the prevalence, incidence, medication prescription pattern, and 5-year survival of PAH in Korea. Cumulative survival and potential predictors of mortality were also assessed among 2,151 incident PAH cases.
Results
Between 2002 or 2004 and 2018, the prevalence and incidence of PAH increased 75-fold (0.4 to 29.9 per million people) and 12-fold (0.5 to 6.3 per million person-years), respectively. The proportion of patients on combination PAH-specific drug therapy has also steadily increased up to 29.0% in 2018. Among 2,151 incident PAH cases (median [interquartile range] age, 50 [37–62] years; 67.2% female), the 5-year survival rate and median survival duration were 71.8% and 13.1 years, respectively. Independent predictors of mortality were age, sex, etiology of PAH, diabetes, dyslipidemia, and chronic kidney disease.
Conclusions
This nationwide study delineated that the prevalence and incidence of PAH have grown rapidly in Korea since the early 2000s. The use of combination therapy has also increased, and the 5-year survival rate of PAH in Korea was similar to those in western countries.

Keyword

Pulmonary arterial hypertension; Big data; Survival; Asia; Drug combination

Figure

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  Figure 1 Annual trends in prevalence, incidence, and medication prescription pattern of pulmonary arterial hypertension in Korea. (A) Prevalence and incidence of PAH. (B) medication prescription pattern of PAH in Korea, 2002-2018. In 2018, the number (proportion) of patients on monotherapy, dual therapy, and triple therapy were 895 (71.0%), 309 (24.5%), and 57 (4.5%), respectively (pie chart).PAH = pulmonary arterial hypertension.

• 

  Figure 2 Annual trends in incidence of pulmonary arterial hypertension in Korea, 2002–2018. (A) sex-specific, (B) age-specific, and (C) etiology-specific incidence of pulmonary arterial hypertension in Korea.IPAH = idiopathic pulmonary arterial hypertension; PAH-CHD = pulmonary arterial hypertension related to congenital heart diseases; PAH-CTD = pulmonary arterial hypertension related to connective tissue disease.

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  Figure 3 Study flowchart for longitudinal analysis.PAH = pulmonary arterial hypertension.

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  Figure 4 Kaplan-Meier survival curves of patients with incident pulmonary arterial hypertension. Cumulative survival of (A) overall incident PAH cases and that of patients stratified by (B) etiology of PAH, (C) PAH-specific drug regimen, and (D) cancer history. IPAH = idiopathic pulmonary arterial hypertension; PAH = pulmonary arterial hypertension; PAH-CHD = pulmonary arterial hypertension related to congenital heart disease; PAH-CTD = pulmonary arterial hypertension related to connective tissue disease.

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