title: Effects of long-term iloprost treatment on right ventricular function in patients with Eisenmenger syndrome.
abstract: Right ventricular (RV) function is an important prognostic factor of pulmonary arterial hypertension (PAH), but there is insufficient data regarding RV function after long-term inhaled iloprost treatment. We evaluated the effect of long-term iloprost treatment on RV function in patients with Eisenmenger syndrome (ES).
  
  
  
  Eleven consecutive patients with ES associated with congenital heart disease underwent echocardiographic measurements at baseline and 48 weeks after iloprost therapy. In addition, we recorded World Health Organization (WHO) functional class, 6-minute walk distance (6MWD), systemic arterial oxygen saturation (SaO2), and laboratory values such as hemoglobin, serum creatinine, and N-terminal pro-B natriuretic peptide.
  
  
  
  After 48 weeks of iloprost therapy, mean pulmonary arterial pressure (mPAP), pulmonary arterial systolic pressure (PASP), and pulmonary vascular resistance (PVR) were significantly decreased [mPAP, 42.5 (38.5-61.0) to 36.5 (29.1-40.0)mmHg; PASP, 92.6±19.9 to 74.5±23.8mmHg; PVR, 23.4 (19.8-26.0) to 23.4 (19.8-26.0)Wood unit respectively, all p<0.05]. There was also significant improvement in RV myocardial performance index [0.68 (0.61-0.80) to 0.52 (0.51-0.62), p=0.003] and RV longitudinal strain (-15.7±1.6 to -18.1±1.5%, p<0.001). In clinical assessment, WHO functional class (p=0.006), 6MWD (310.6±44.7 to 399.7±80.8m, p<0.001), and SaO2(90.9±6.0% to 92.5±6.0%, p=0.022) were significantly improved.
  
  
  
  The improvement in echocardiographic parameters of the RV function after 48 weeks of iloprost therapy may provide insight on the efficacy of long-term iloprost treatment for RV functional improvement, which is a prognostic factor in patients with ES.
authors: Department of Cardiology, Pusan National University Yangsan Hospital, Yangsan, South Korea.
authors: Min Ku
authors: MK
authors: Chon
authors: Department of Internal Medicine, Kosin University School of Medicine, Busan, South Korea. Electronic address: kyoungim74@gmail.com.
authors: Kyoung Im
authors: KI
authors: Cho
authors: Department of Cardiology and Medical Research Institute, Pusan National University Hospital, Busan, South Korea.
authors: Kwang Soo
authors: KS
authors: Cha
authors: Department of Internal Medicine, Busan Paik Hospital, University of Inje College of Medicine, Busan, South Korea.
authors: Jeong Sook
authors: JS
authors: Seo
authors: Department of Internal Medicine, Busan Paik Hospital, University of Inje College of Medicine, Busan, South Korea.
authors: Dong Soo
authors: DS
authors: Kim
mesh_terms: D000280:Administration, Inhalation; D000328:Adult; D004452:Echocardiography; D004541:Eisenmenger Complex / Q000188:drug therapy*; D005080:Exercise Test; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy; D016285:Iloprost / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D010100:Oxygen / Q000097:blood; D011446:Prospective Studies; D014655:Vascular Resistance / Q000187:drug effects; D014665:Vasodilator Agents / Q000627:therapeutic use*; D018497:Ventricular Dysfunction, Right / Q000188:drug therapy*
chemical_list: D014665:Vasodilator Agents; D016285:Iloprost; D010100:Oxygen
keywords: Eisenmenger syndrome; Iloprost; Pulmonary arterial hypertension; Right ventricular function