Heightened Dependence of Left-Heart Filling Pressures on Right-Heart Failure in Congenital Heart Disease

Abstract

BackgroundPulmonary artery wedge pressure (PAWP) is often elevated in patients with right-sided congenital heart disease (CHD), raising the possibility of coexisting left-heart disease, but pressure-volume relationships in the left and right sides of the heart influence one another through interdependence, which may be amplified in patients with CHD. MethodsWe hypothesized that increases in PAWP in patients with CHD would be more strongly related to ventricular interdependence compared with patients who have isolated left-heart disease such as heart failure with preserved ejection fraction (HFpEF). Ventricular interdependence was assessed by the relationship between PAWP and right-atrial pressure (RAP), RAP/PAWP ratio, and the left-ventricular (LV) eccentricity index. ResultsPAWP was elevated (≥15 mm Hg) in 49% of patients with CHD (n = 449). There was a very strong correlation between RAP and PAWP in CHD (r = 0.81, P < 0.001) that greatly exceeded the respective correlation in HFpEF (n = 160; r = 0.58, P < 0.001; P < 0.001 between groups). RAP/PAWP ratio and LV eccentricity index were higher in CHD than HFpEF (1.26 ± 0.18 vs 1.05 ± 0.14, P = 0.007) and (0.80 ± 0.21 vs 0.59 ± 0.19, P < 0.001), respectively. RAP (but not PAWP) was an independent predictor of death/transplant (hazard ratio 1.86 per 5 mm Hg, 95% confidence interval, 1.39-2.45, P = 0.002). ConclusionsLeft-heart filling pressures are commonly elevated in right-sided CHD, but this is related predominantly to right-heart failure and enhanced ventricular interdependence rather than left-heart disease. These data provide new insight into the basis of abnormal left-heart hemodynamics in patients with CHD and reinforce the importance of therapeutic interventions targeted to the right heart.