Right Ventricular Function and Right-Heart Echocardiographic Response to Therapy Predict Long-term Outcome in Patients With Pulmonary Hypertension

Background: Right ventricular (RV) dysfunction in pulmonary hypertension (PH) is linked to adverse outcomes, but this response is considered heterogeneous because it can be associated with multiple factors. Methods: RV function of 51 PH patients was calculated by averaging peak speckle-tracking longitudinal strain from RV free-wall (RV-free), and the cutoff for RV dysfunction was predefined as RV-free <= 19%. Right-sided heart remodelling was assessed in terms of RV end-systolic area (RVESA) and right atrial (RA) area (RA-area). Midterm reverse remodelling was defined as a relative decrease in RVESA (DRVESA) and RA-area (DRA-area) of at least 15% at 5.7 +/- 4.0 months after introduction of pulmonary artery hypertension-specific drugs. Longterm outcome was tracked for 3.0 +/- 2.0 years. Results: Patients with midterm RV and RA reverse remodelling showed more favourable long-term outcomes than those without (P = 0.01, P = 0.047, respectively). Sequential Cox models showed that a model based on hemodynamic parameters (chi(2) = 0.3) was improved by the addition of RV-free (chi(2) = 6.4; P = 0.01), and further improved by addition of DRVESA and DRA-area (chi(2) = 28.2; P < 0.001). Furthermore, preservation of baseline RV function and midterm reverse remodelling in right-sided heart was associated with an optimal outcome: a survival rate of 100%. In contrast, absence of midterm reverse remodelling in the right-sided heart of patients with impaired baseline RV function was associated with significantly worse outcome with a survival rate of 33% (P = 0.01). Conclusions: RV function and echocardiographic right-heart reverse remodelling with therapy improves the prediction of long-term outcomes for PH patients over standard hemodynamic indices.