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Pulmonary arterial hypertension (PAH) is a life-threatening disorder with a poor prognosis and causes pulmonary vascular remodeling accompanied with increased pulmonary arterial medial wall thickness and fibrosis, which leads to vascular and right ventricular (RV) dysfunction. Despite treatment with prostacyclin, endothelin antagonist, and phosphodiesterase-5 inhibitors the 1-year mortality rate of PAH still remains high. Recent registries, clinical trials, and basic researches have been increasing the knowledge of PAH and it would contribute to potential therapeutic strategies and better clinical outcome.

Korean Registry of Pulmonary Arterial Hypertension (KORPAH) is the first modern PAH registries in Asian ethnicity. Total 39 centers participated and 625 patients were enrolled. This study evaluated the incidence, prevalence, epidemiology, therapeutic modalities and survival data of Korean patients with PAH.

“Gachon experiences” was to characterize the clinical outcomes and evaluate the factors influencing survival time of the PAH patients in Korean. This study compared the cumulative survival of total 43 PAH patients who received targeted or conventional therapy.

PAH Ilopost BMPR-2 gene in Korea IIT Multi-institutional (PILGRIM) is a prospective, investigator-initiative, and multi-institutional clinical trials. This study was recently completed in March by 7 institutes, and aimed to investigate (1) the prevalence of BMPR-2 gene mutations in the Korean PAH patients and (2) the effect of iloprost inhalation solution on hemodynamic response, and exercise echocardiography.

PAH basic research focuses on two major themes: (1) Systematic comparison of the effects of adipose tissue, bone marrow and umbilical cord blood-derived mesenchymal stem cell transplantation on MCT-induced PAH in rats and (2) investigation of the effect of human UCB-derived MSC (hUCB-MSC) transplantation combined with apelin-13 administration on MCT-induced PAH in rats. Data suggests that, although the survival and therapeutic potential of all three MSCs were similar with regards to the attenuation of MCT-induced PAH progression in rats, each MSCs may act differently via distinct molecular mechanisms. Transplantation of hUCB-MSCs combined with apelin-13 administration improved the survival of transplanted cells in PAH rat lungs, and also improved the ability of hUCB-MSCs to protect from RV dysfunction and medial wall thickening.

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