Differences in right ventricular-pulmonary vascular coupling and clinical indices between repaired standard tetralogy of Fallot and repaired tetralogy of Fallot with pulmonary atresia

Purpose: The purpose of this study was to compare ventricular vascular coupling ratio (VVCR) between patients with repaired standard tetralogy of Fallot (TOF) and those with repaired TOF-pulmonary atresia (TOF-PA) using cardiovascular magnetic resonance (CMR). Materials and methods: Patients with repaired TOF aged > 6 years were prospectively enrolled for same day CMR, echocardiography, and exercise stress test following a standardized protocol. Sanz's method was used to calculate VVCR as right ventricle (RV) end-systolic volume/pulmonary artery stroke volume. Regression analysis was used to examine associations with exercise test parameters, New York Heart Association (NYHA) class, RV size and biventricular systolic function. Results: A total of 248 subjects were included; of these 222 had repaired TOF (group I, 129 males; mean age, 15.9 ± 4.7 [SD] years [range: 8–29 years]) and 26 had repaired TOF-PA (group II, 14 males; mean age, 17.0 ± 6.3 [SD] years [range: 8–29 years]). Mean VVCR for all subjects was 1.54 ± 0.64 [SD] (range: 0.43–3.80). Mean VVCR was significantly greater in the TOF-PA group (1.81 ± 0.75 [SD]; range: 0.78–3.20) than in the standard TOF group (1.51 ± 0.72 [SD]; range: 0.43–3.80) (P = 0.03). VVCR was greater in the 68 NYHA class II subjects (1.79 ± 0.66 [SD]; range: 0.75–3.26) compared to the 179 NYHA class I subjects (1.46 ± 0.61 [SD]; range: 0.43–3.80) (P < 0.001). Conclusion: Non-invasive determination of VVCR using CMR is feasible in children and adolescents. VVCR showed association with NYHA class, and was worse in subjects with repaired TOF-PA compared to those with repaired standard TOF. VVCR shows promise as an indicator of pulmonary artery compliance and cardiovascular performance in this cohort.