Ji Hyun Cha I-Seok Kang, Shin Yi Jang, June Huh Jeong Hoon Yang, Jinyoung Song, Taek Kyu Park, Seung Woo Park, Hojoong Kim, Duk-Kyung Kim Sung-A Chang
Samsung Medical Center, Samsung Changwon Hospital and Sungkyunkwan University School of Medicine.
Republic of Korea
Korean Circulation Journal
Korean Circ J 2024;
DOI: 10.4070/kcj.2023.0316
Abstract
Background and objectives: The transformation of pulmonary arterial hypertension (PAH) treatment in Korea, ushered by targeted therapy’s advent, prompted our analysis of baseline attributes, treatment trends, and survival shifts within our single-center registry.
Methods: We examined 230 patients (72.6% female, mean age 40.6±17.4 years) diagnosed and/or treated between 1980 and 2021 in our PAH clinic. Given targeted therapy’s introduction and active use since 2007, we compared diagnostic classification, demographics, and treatment patterns at that juncture. Survival analysis encompassed PAH types and the overall population. For historical survival comparison, 50 non-registry patients were retrospectively added, and age-sex matching enabled pooled analysis.
Results: Congenital heart disease-associated pulmonary arterial hypertension (CHD-PAH) constituted the largest subset (43.0%), trailed by connective tissue disease-associated PAH (CTD-PAH, 29.6%) and idiopathic PAH (IPAH, 19.1%). Post-2007, CTD-PAH proportions surged, notably with an elevated initiation rate of targeted therapy (95.4%). Overall survival rates at 1, 5, and 10 years stood at 91.3%, 77.4%, and 65.8%, respectively, with CHD-PAH exhibiting superior survival to idiopathic or CTD-PAH. Age-sex matching analysis indicated survival disparities between those starting immediate targeted therapy vs. conservative treatment upon diagnosis, especially driven by IPAH.
Conclusions: In the post-introduction of the targeted therapy era, patients with PAH promptly started treatment right away, and higher survival rates of patients who started initial PAH-targeted therapy were demonstrated. The transition towards early treatment initiation might have likely contributed to the elevated survival rates observed in Korea’s PAH patient cohort.
Category
Class I. Idiopathic Pulmonary Hypertension
Class I. Pulmonary Hypertension Associated with Congenital Cardiovascular Disease
Class I. Pulmonary Hypertension Associated with Connective Tissue Disease
Medical Therapy. Efficacy or Lack of Efficacy
Age Focus: Adult Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: Yes