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A Single Center Experience of Pulmonary Arterial Hypertension Management in Korea: A 25-Year Comparative Analysis Following the Introduction of Targeted Therapy

  • Ji Hyun Cha (Department of Critical Care Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Shin Yi Jang (Pulmonary Hypertension Center, Heart Vascular Stroke Institute, Samsung Medical Center) ;
  • Jinyoung Song (Pulmonary Hypertension Center, Heart Vascular Stroke Institute, Samsung Medical Center) ;
  • I-Seok Kang (Pulmonary Hypertension Center, Heart Vascular Stroke Institute, Samsung Medical Center) ;
  • June Huh (Pulmonary Hypertension Center, Heart Vascular Stroke Institute, Samsung Medical Center) ;
  • Taek Kyu Park (Division of Cardiology, Department of Medicine, Heart Vascular Stroke Institute, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Jeong Hoon Yang (Department of Critical Care Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Seung Woo Park (Division of Cardiology, Department of Medicine, Heart Vascular Stroke Institute, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Hojoong Kim (Pulmonary Hypertension Center, Heart Vascular Stroke Institute, Samsung Medical Center) ;
  • Duk-Kyung Kim (Division of Cardiology, Department of Medicine, Heart Vascular Stroke Institute, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Sung-A Chang (Division of Cardiology, Department of Medicine, Heart Vascular Stroke Institute, Samsung Medical Center, Sungkyunkwan University School of Medicine)
  • 투고 : 2023.11.28
  • 심사 : 2024.06.04
  • 발행 : 2024.10.01

초록

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.

키워드

참고문헌

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