Impaired Exercise Tolerance in Repaired Tetralogy of Fallot Is Associated With Impaired Biventricular Contractile Reserve: An Exercise-Stress Real-Time Cardiovascular Magnetic Resonance Study

Abstract

Background: Correction of tetralogy of Fallot (cTOF) often results in pulmonary valve pathology and right ventricular (RV) dysfunction. Reduced exercise capacity in cTOF patients cannot be explained by these findings alone. We aimed to explore why cTOF patients exhibit impaired exercise capacity with the aid of a comprehensive cardiopulmonary exercise testing (CPET) and real-time cardiovascular magnetic resonance exercise testing (CMR-ET) protocol. Methods: Thirty three cTOF patients and 35 matched healthy controls underwent CPET and CMR-ET in a prospective case-control study. Real-time steady-state free precession cine and phase-contrast sequences were obtained during incremental supine in-scanner cycling at 50, 70, and 90 W. RV and left ventricle (LV) volumes and pulmonary blood flow (Qp) were calculated. Differences of CPET and CMR-ET between cTOF versus controls and correlations between CPET and CMR-ET parameters in cTOF were evaluated statistically for all CMR exercise levels using Mann-Whitney U and Spearman rank-order correlation tests. Results: CPET capacity was significantly lower in cTOF than in controls. cTOF patients exhibited not only significantly reduced Qp and RV function but also lower LV function on CMR-ET. Higher CPET values in cTOF correlated with higher Qp (Qp 90 W versus carbon dioxide ventilatory equivalent %: R =−0.519, P <0.05), higher LV–end-diastolic volume indexed to body surface area (LV–end-diastolic volume indexed to body surface area at 50 W versus oxygen uptake in % at maximum exercise on CPET R =0.452, P <0.05), and change in LV ejection fraction (EF; LV-EF at 90 W versus Watt %: r =−0.463, P <0.05). No correlation was found with regard to RV-EF. Significant RV-LV interaction was observed during CMR-ET (RV-EF versus LV-EF at 50 W and 70 W: r =0.66, P <0.02 and r =0.52, P <0.05, respectively). Conclusions: Impaired exercise capacity in cTOF resulted from a reduction in not only RV, but also LV function. cTOF with good exercise capacity on CPET demonstrated higher LV reserve and pulmonary blood flow during incremental CMR-ET. Apart from RV parameters, CMR-ET–derived LV function could be a valuable tool to stratify cTOF patients for pulmonary valve replacement.