Current Therapeutic Patterns of Pulmonary Arterial Hypertension in Korea: A Five-Year Follow-up of the PHOENIKS Longitudinal Cohort

BACKGROUND AND OBJECTIVES: The 2022 revised pulmonary hypertension guidelines emphasize risk stratification to enhance outcomes, necessitating investigation into adherence. This study aimed to examine the treatment patterns of patients in the Pulmonary Arterial Hypertension (PAH) Platform for deep Phenotyping in Korean Subjects (PHOENIKS) study cohort. METHODS: A total of 321 patients from the PHOENIKS cohort were included, consisting of 101 patients in phase 1 (2018-2020) and 220 in phase 2 (2021-2023). A total of 275 patients with either idiopathic PAH or associated PAH were included in the final analysis. Risk assessment was conducted utilizing an online calculator that integrates multiple validated risk stratification models. Adherence to the revised guidelines was evaluated, and survival outcomes were analyzed over the study period. RESULTS: The cohort consisted primarily of middle-aged and female patients (mean age 51.96±15 years, 71% female). A total of 53% had idiopathic PAH and 47% had associated PAH. Intermediate-risk patients comprised 62% of the cohort during the initial diagnosis. The proportion of low-risk patients increased from 34% to 64% throughout the follow-up period. Adherence to the revised guidelines slightly improved during the study period, though only 26% of patients demonstrated compliance. The one-year survival rate was 96%, whereas the 3-year survival rate was 87%. CONCLUSIONS: The current Korean PAH treatment strategy must be improved to align with the revised guidelines and optimize patient outcomes. Further efforts are needed to change health insurance coverage criteria to enhance guideline adherence.