Determinats and Prognostic Significance of Right Ventricular Reverse Remodeling in Idiopathic Pulmonary Arterial Hypertension Receiving Specific Medical Treatment

PurposeSurvival in idiopathic pulmonary arterial hyperthension (IPAH) is strongly associated to right ventricular function and right heart failure remains the primary cause of death. The aims of the present study were to determine the predictors of right ventricular reverse remodeling (RVRR) in IPAH patients receiving specific treatments and its impact on long-term prognosis.MethodsOne houndred-two therapy-naïve IPAH patients were enrolled in our centre and prospectively followed-up for the presence of clinical worsening (CW). Baseline evaluation included clinical and hemodynamic parameters. Echocardiographic evaluation was used for RVRR characterization at 1-year follow-up, defined as significant changes in RV end-diastolic area, right atrial area and left ventricular systolic eccentricity index by ROC analysis cut-points.ResultsAfter 360±16 days of follow-up 18 of 102 (17.6%) patients presented RVRR. Pulmonary vascular resistance (PVR) at mid-term follow-up and its reduction from baseline resulted the major determinants of RVRR. After 1-year evaluation, 81 event-free surviving patients were followed for 358±236 days; actuarial rates of CW were 79%, 56% and 44% at 1-2-3 years, respectively. RVRR and changes in WHO functional class significantly improved the power of the prognostic model based on traditional clinical and hemodynamic parameters (AUC 0.80 vs. 0.66, respectively; p=0.0004). The event-free survival rates were, respectively, 94% and 94% versus 65% and 43% after 1 and 2 years from mid-term re-evaluation, in patients with and without RVRR (p= 0.004).ConclusionRVRR is an independent predictor of prognosis in IPAH. Thus, as PVR decrease is the major determinant of RVRR, treatment strategies should be implemented to achive broad RV afterload reduction. PurposeSurvival in idiopathic pulmonary arterial hyperthension (IPAH) is strongly associated to right ventricular function and right heart failure remains the primary cause of death. The aims of the present study were to determine the predictors of right ventricular reverse remodeling (RVRR) in IPAH patients receiving specific treatments and its impact on long-term prognosis. Survival in idiopathic pulmonary arterial hyperthension (IPAH) is strongly associated to right ventricular function and right heart failure remains the primary cause of death. The aims of the present study were to determine the predictors of right ventricular reverse remodeling (RVRR) in IPAH patients receiving specific treatments and its impact on long-term prognosis. MethodsOne houndred-two therapy-naïve IPAH patients were enrolled in our centre and prospectively followed-up for the presence of clinical worsening (CW). Baseline evaluation included clinical and hemodynamic parameters. Echocardiographic evaluation was used for RVRR characterization at 1-year follow-up, defined as significant changes in RV end-diastolic area, right atrial area and left ventricular systolic eccentricity index by ROC analysis cut-points. One houndred-two therapy-naïve IPAH patients were enrolled in our centre and prospectively followed-up for the presence of clinical worsening (CW). Baseline evaluation included clinical and hemodynamic parameters. Echocardiographic evaluation was used for RVRR characterization at 1-year follow-up, defined as significant changes in RV end-diastolic area, right atrial area and left ventricular systolic eccentricity index by ROC analysis cut-points. ResultsAfter 360±16 days of follow-up 18 of 102 (17.6%) patients presented RVRR. Pulmonary vascular resistance (PVR) at mid-term follow-up and its reduction from baseline resulted the major determinants of RVRR. After 1-year evaluation, 81 event-free surviving patients were followed for 358±236 days; actuarial rates of CW were 79%, 56% and 44% at 1-2-3 years, respectively. RVRR and changes in WHO functional class significantly improved the power of the prognostic model based on traditional clinical and hemodynamic parameters (AUC 0.80 vs. 0.66, respectively; p=0.0004). The event-free survival rates were, respectively, 94% and 94% versus 65% and 43% after 1 and 2 years from mid-term re-evaluation, in patients with and without RVRR (p= 0.004). After 360±16 days of follow-up 18 of 102 (17.6%) patients presented RVRR. Pulmonary vascular resistance (PVR) at mid-term follow-up and its reduction from baseline resulted the major determinants of RVRR. After 1-year evaluation, 81 event-free surviving patients were followed for 358±236 days; actuarial rates of CW were 79%, 56% and 44% at 1-2-3 years, respectively. RVRR and changes in WHO functional class significantly improved the power of the prognostic model based on traditional clinical and hemodynamic parameters (AUC 0.80 vs. 0.66, respectively; p=0.0004). The event-free survival rates were, respectively, 94% and 94% versus 65% and 43% after 1 and 2 years from mid-term re-evaluation, in patients with and without RVRR (p= 0.004). ConclusionRVRR is an independent predictor of prognosis in IPAH. Thus, as PVR decrease is the major determinant of RVRR, treatment strategies should be implemented to achive broad RV afterload reduction. RVRR is an independent predictor of prognosis in IPAH. Thus, as PVR decrease is the major determinant of RVRR, treatment strategies should be implemented to achive broad RV afterload reduction.