Abstract
BACKGROUND: Right ventricular restrictive physiology (RVRP) is a common occurrence in repaired tetralogy of Fallot (rTOF). The relationship of RVRP with biventricular blood flow components and kinetic energy (KE) from 4-dimensional (4D) flow cardiovascular magnetic resonance (CMR) is unclear. OBJECTIVES: The purpose of this study was to investigate the association of 4D flow CMR parameters with RVRP in rTOF patients. METHODS: A total of 103 rTOF patients and 62 age and sex-matched healthy control subjects were prospectively recruited. All participants underwent CMR (cine, 2-dimensional phase-contrast, and 4D flow sequences), and cardiopulmonary exercise test in adult populations. RVRP was identified from pulmonary artery flow curve using 2-dimensional phase-contrast images. Biventricular flow components (direct flow, retained inflow, delayed ejection flow, and residual volume) and KE parameters normalized to end-diastolic volume (KEi(EDV)) were analyzed encompassing global, peak systolic, average systolic, average diastolic, peak E-wave, and peak A-wave. RESULTS: Compared with control subjects, rTOF patients had significantly lower RV direct flow and higher RV residual volume (both P < 0.001). All RV KEi(EDV) parameters, except peak A-wave, were higher in rTOF patients. In rTOF patients, 70 of 103 (68%) had RVRP, with increasing RV direct flow (27% vs 20%; P = 0.002) and RV peak E-wave KEi(EDV) (28.4 vs 20.7μJ/mL; P = 0.015) and decreasing RV residual volume (37% vs 42%; P = 0.039) than rTOF without RVRP. Exercise capacity was impaired in rTOF, although comparable between RVRP subgroups. Multivariable analysis revealed RV direct flow was an independent predictor of RVRP (OR: 1.158; 95% CI: 1.074-1.249; P < 0.001). CONCLUSIONS: RVRP is associated with dilated RV, higher pulmonary regurgitation, and higher RV direct flow. (Integrated Computational modeling of Right Heart Mechanics and Blood Flow Dynamics in Congenital Heart Disease; NCT03217240).