Determinants and Prognostic Implications of Left-Heart Filling Pressures in Tetralogy of Fallot

Background: Elevated pulmonary artery wedge pressure (PAWP) is the hallmark of left-heart failure and is responsible for heart failure symptoms and mortality. Although PAWP typically correlates with right atrial pressure (RAP), it is primarily dependent on left-heart myocardial properties and volume status. As right-heart disease can occur in the absence of left-heart disease in patients with tetralogy of Fallot (TOF), we hypothesized that RAP was the primary determinant of PAWP in this population. Methods: A cohort study of adults with TOF that underwent right-heart catheterization at Mayo Clinic Rochester (1990 to 2017) to determine the relationship among RAP, PAWP, and mortality. Results: Among 213 patients (male 105; age 37 ± 14 years), the mean PAWP was 14 ± 5 mm Hg, and RAP was 11 ± 5 mm Hg. RAP was the strongest predictor of PAWP (β = 0.68, standard error = 0.06, P < 0.001), independent of left-heart disease and atherosclerotic cardiovascular risk factors. The patients with high PAWP also had normal tissue Doppler velocities, suggesting normal left-ventricular myocardial properties. PAWP was an independent predictor of death/transplant (hazard ration [HR] 1.11, 95% confidence interval [CI], 1.03-1.20, P = 0.004). However, when RAP was incorporated into the regression model, RAP (and not PAWP) became the independent predictor of outcomes (HR 1.14, 95% CI, 1.06-1.22, P = 0.001). Conclusions: The current study showed that RAP was the primary determinant of PAWP and accounts, to some extent, for the mortality in patients with TOF and high PAWP. The data provide new insight in the pathophysiology of disease progression for symptomatic patients with TOF.