Current status of pulmonary arterial hypertension in Korea

Abstract

Pulmonary arterial hypertension (PAH) is characterized by remodeling of the distal pulmonary arteries resulting in high pulmonary vascular resistance and, eventually, right ventricular heart failure. Although current advances in PAH therapy have improved outcomes, poor survival remains a reality worldwide, including Korea. One of the most important issues in PAH is the late diagnosis, since screening or diagnostic efforts are often overlooked due to the rarity of disease. Data from Korean registries and observational cohorts show that delayed detection leads to increased morbidity. Additionally, low percentages of Korean patients are committed to intensive PAH-targeted therapy. Current Korean health insurance policies' lack of coverage for new PAH-targeted drugs and upfront combination therapy may also hamper the improvement of treatment outcomes. Understanding individual variability in response to therapeutics through deep phenotyping is a novel strategy that should be considered when treating PAH. Overall, early detection of PAH by proactive screening together with early, intensive, individualized PAH therapy using deep phenotyping is crucial for improving prognoses for PAH patients in Korea.

Keywords: Early diagnosis; Pharmacotherapy; Phenotyping; Precision medicine; Pulmonary arterial hypertension.

Figure 1.

Cumulative survival and comparison of survival among etiologies. (A) Cumulative survival curve of the incident cases in the Korean Registry of Pulmonary Arterial Hypertension (KORPAH; n = 297). The 1st-, 2nd- and 3rd-year estimated survival rates were 90.8%, 87.8%, and 84.4%, respectively. (B) Comparison of survival according to the etiologies of pulmonary arterial hypertension (PAH) of the incident cases in the KORPAH (n = 297). This figure presents a comparison of prognoses according to the etiologies of PAH. PAH with connective tissue disease (CTD) corresponded to the highest mortality (18.8%), followed by idiopathic PAH (IPAH) (8.1%), and PAH with congenital heart disease (CHD; 3.9%) (p = 0.043).

Figure 2.

Comparison of survival based on initial symptom severity and type of therapy. (A) Median overall survival time of patients based on New York Heart Association (NYHA) functional classification, where patients with NYHA class I or II at the time of diagnosis showed significantly better survival than those with a more severe functional class. (B) Median overall survival time of patients according to the treatment is shown. Patients with molecular targeted therapy showed significantly better survival than those with conventional therapy only.