Inhaled ilroprost can improve clinical status and symptoms in Eisenmenger syndrome (ES). Right ventricular (RV) function is important prognostic factor of pulmonary arterial hypertension. But there has been insufficient data in echocardiographic parameters related to RV function after long term iloprost therapy. So we evaluated effect of long term iloprost treatment on RV function and other parameters measured by echocardiography in this study.Design and Method:
Eleven consecutive patients with ES associated with congenital heart disease underwent echocardiographic measurements at baseline and 48 weeks after iloprost therapy. Echocardiographic measurements included conventional parameters and mean pulmonary arterial pressure (mPAP), pulmonary arterial systolic pressure (PASP), pulmonary vascular resistance (PVR), RV fractional change, myocardial performance index (MPI) of RV, Tricuspid annular plane systolic excursion (TAPSE), RV long-axis motions assessed by strain analysis. In addition, World Health organization (WHO) functional class, 6-minite walk distance (6MWD), systemic arterial oxygen saturation (SaO2), and laboratory test such as hemoglobin, serum creatinine and N-terminal pro-B natriuretic peptide were also recorded.Results:
After 48 weeks of ilorprost therapy mPAP, PASP and PVR were significantly decreased (all p's < 0.05). And there was statistical significant improvement in RV MPI (0.78 ± 0.32 to 0.55 ± 0.10, p = 0.003), TAPSE (15.5 ± 2.9 mm to 18.2 ± 2.9 mm, p = 0.003) and RV longitudinal strain (−15.7 ± 1.6% to −18.1 ± 1.5%, p = 0.003). In clinical assessment, WHO functional class (P = 0.006), 6MWD (p = 0.003) and SaO2 (p = 0.024) were significantly improved. In laboratory test, uric acid and NT-proBNP were improved and another aggravation was not reported after the 48 weeks of iloprost treatment.Conclusions:
There was significant improvement in RV echocardiographic parameters, WHO functional class, 6MWD, and SaO2 after 48 weeks of iloprost therapy. This result may provide insight on the efficacy of long term iloprost treatment to RV functional improvement that has been known as the prognostic factors in ES patient.