The Importance of Echocardiographic Screening for Pulmonary Arterial Hypertension in Korean Patients with Systemic Sclerosis

Recognition of pulmonary arterial hypertension (PAH) associated with systemic sclerosis (SSc) (SSc-PAH) is very important, because SSc-PAH is highly prevalent and is a leading cause of mortality in patients with SSc. Despite the use of novel therapeutic strategies, response to PAH-targeted therapy is still insufficient in SSc-PAH, which is associated with poor prognosis in SSc-PAH. Indeed, it has been reported that PAH develops in 12% of patients with SSc, and about 50% of SSc patients die within three years of SSc-PAH diagnosis. Initially, there have been argues that early diagnosis and management of SSc-PAH can improve patients’ outcomes, but recent clinical trials and cohort studies have clearly demonstrated the beneficial effect of early treatment for SSc-PAH. Therefore, current guideline recommends annual echocardiographic screening for the detection of SSc-PAH. Right heart catheterization (RHC) is required for patients in whom the results of echocardiography are suggestive of PAH, to confirm diagnosis. In this issue of the Journal of Cardiovascular Ultrasound, Yoo et al. performed echocardiography and RHC for the evaluation of PAH in 37 adult Korean patients with SSc. The authors showed that the prevalence of SSc-PAH in study patients was 21.6% in echocardiography and 13.5% in RHC. This SSc-PAH prevalence is similar to prior studies performed in other countries. The result of this study is meaningful and deserves clinical attention, because this is the first study primarily focused on the prevalence of SSc-PAH in Korea using RHC. Despite of clinical importance of SSc-PAH, there pISSN 1975-4612/ eISSN 2005-9655 Copyright © 2016 Korean Society of Echocardiography www.kse-jcu.org https://doi.org/10.4250/jcu.2016.24.4.276