Survival rates in newly diagnosed PAH patients after the development of PAH-specific therapies were 86.3% and 61.2% at 1 and 5 years, respectively (2). should be needed to improve the survival of PAH patients. The BREATHE-5 trial, first placebo-controlled trial in patients with ES, demonstrated a significant improvement of hemodynamics and exercise capacity without adversely affecting systemic arterial oxygen saturation on bosentan-treated patients (3). Other recent randomized controlled trials in ES with phosphodiesterase type-5 inhibitors have shown improvements in exercise capacity and hemodynamics (4,5). In common with other world registries, our group also reports the importance of specialized therapies to improve the survival of PAH Etravirine ( R165335, TMC125) patients in Korean Registry of pulmonary arterial hypertension (KORPAH) (the control group 831 70.064???Use of iNOa (h)11.2 4.525 6.80.031Plasma BNPb (pg/mL)98 46265 920.008Mechanical ventilation time (h)10.1 12.541.1 46.10.018ICU stay (h)39.4 26.490.3 60.80.005Chest tube use (h)63.9 22.789.3 42.80.039Inotropic support (h)103.8 88.274.5 56.00.246Drug used (g/kg/h)???Milrinone0.375C0.50.375C0.5???Dopamine5C105C10 Open in a separate window a, administered Etravirine ( R165335, TMC125) via an endotracheal tube before weaning when clinically necessary for the immediate postoperative period; b, Checked on the 7th postoperative day. The recent study of the Etravirine ( R165335, TMC125) German National Resister for Etravirine ( R165335, TMC125) congenital heart defects (GNR-CHD) contains a nation-wide data with a large population of ES patients in the community (14). Among 153 patients with ES, 57.5% of patients were treated with PAH-specific medical therapies. Of those, 17.6% of patients received combination therapy; 76.1% of patients on monotherapy were on bosentan and 44.4% of patients treated primarily with Sildenafil were also on this drug as a second line. The GNR-CHD is a well-designed study and recruited Cspg4 a large number of patients representing the community-based population. The result of this study may be valuable data on advanced targeted therapy. However, as the intrinsic drawback of registry, the lack of uniform treatment strategy couldnt explain effective and timely treatments in PAH-targeted therapy. This limitation was shown as no outcome difference between monotherapy and dual targeted therapy because of the long escalation time. Nevertheless, the strongest message from GNR-CHD is the better clinical outcome in the large volume centers than remaining centers that means the need of expert care from centers of excellence. In summary, the GNR-CHD demonstrated better survival of advanced targeted therapy based on the real world as well as tertiary referrals in the Germany. This modern real world registry data reinforce the reason why specialized medical therapies in PAH expert center should be considered in ES patients. Acknowledgements This research was partly supported by the Gachon University Gil Medical Center (Grant number: 2015-02) and the Next-generation Medical Device Development Program for Newly-Created Market of the National Research Foundation (NRF) funded by the Korean government, MSIP (No. 2015M3D5A1066043). Footnotes This is an invited Editorial commissioned by the Section Editor Haiyun Yuan (Department of Cardiovascular Surgery, Guangdong Provincial Cardiovascular Institute, Guangdong General Hospital, Guangzhou, China). The authors have no conflicts of interest to declare..
