RV adaptation to increased afterload in congenital heart disease and pulmonary hypertension

BACKGROUND: The various conditions causing a chronic increase of RV pressure greatly differ in the occurrence of RV failure, and in clinical outcome. To get a better understanding of the differences in outcome, RV remodeling, longitudinal function, and transverse function are compared between patients with pulmonary stenosis (PS), those with a systemic RV and those with pulmonary hypertension (PH). MATERIALS AND METHODS: This cross-sectional study prospectively enrolled subjects for cardiac magnetic resonance imaging (CMR), functional echocardiography and cardiopulmonary exercise testing. The study included: controls (n = 37), patients with PS (n = 15), systemic RV (n = 19) and PH (n = 20). Statistical analysis was performed using Analysis of Variance (ANOVA) with posthoc Bonferroni. RESULTS: PS patients had smaller RV volumes with higher RV ejection fraction (61.1±9.6%; p<0.05) compared to controls (53.8±4.8%). PH and systemic RV patients exhibited dilated RVs with lower RV ejection fraction (36.9±9.6% and 46.3±10.1%; p<0.01 versus controls). PH patients had lower RV stroke volume (p = 0.02), RV ejection fractions (p<0.01) and VO(2) peak/kg% (p<0.001) compared to systemic RV patients. Mean apical transverse RV free wall motion was lower and RV free wall shortening (p<0.001) was prolonged in PH patients–resulting in post-systolic shortening and intra-ventricular dyssynchrony. Apical transverse shortening and global longitudinal RV deformation showed the best correlation to RV ejection fraction (respectively r = 0.853, p<0.001 and r = 0.812, p<0.001). CONCLUSIONS: RV remodeling and function differed depending on the etiology of RV pressure overload. In contrast to the RV of patients with PS or a systemic RV, in whom sufficient stroke volumes are maintained, the RV of patients with PH seems unable to compensate for its increase in afterload completely. Key mediators of RV dysfunction observed in PH patients, were: prolonged RV free wall shortening, resulting in post-systolic shortening and intra-ventricular dyssynchrony, and decreased transverse function.