Abstract

BackgroundFour-dimensional (4D) flow cardiovascular magnetic resonance (CMR) allows quantification of biventricular blood flow by flow components and kinetic energy (KE) analyses. However, it remains unclear whether 4D flow parameters can predict cardiopulmonary exercise testing (CPET) as a clinical outcome in repaired tetralogy of Fallot (rTOF). Current study aimed to (1) compare 4D flow CMR parameters in rTOF with age- and gender-matched healthy controls, (2) investigate associations of 4D flow parameters with functional and volumetric right ventricular (RV) remodelling markers, and CPET outcome.MethodsSixty-three rTOF patients (14 paediatric, 49 adult; 30 ± 15 years; 29 M) and 63 age- and gender-matched healthy controls (14 paediatric, 49 adult; 31 ± 15 years) were prospectively recruited at four centers. All underwent cine and 4D flow CMR, and all adults performed standardized CPET same day or within one week of CMR. RV remodelling index was calculated as the ratio of RV to left ventricular (LV) end-diastolic volumes. Four flow components were analyzed: direct flow, retained inflow, delayed ejection flow and residual volume. Additionally, three phasic KE parameters normalized to end-diastolic volume (KEiEDV), were analyzed for both LV and RV: peak systolic, average systolic and peak E-wave.ResultsIn comparisons of rTOF vs. healthy controls, median LV retained inflow (18% vs. 16%, P = 0.005) and median peak E-wave KEiEDV (34.9 µJ/ml vs. 29.2 µJ/ml, P = 0.006) were higher in rTOF; median RV direct flow was lower in rTOF (25% vs. 35%, P < 0.001); median RV delayed ejection flow (21% vs. 17%, P < 0.001) and residual volume (39% vs. 31%, P < 0.001) were both greater in rTOF. RV KEiEDV parameters were all higher in rTOF than healthy controls (all P < 0.001). On multivariate analysis, RV direct flow was an independent predictor of RV function and CPET outcome. RV direct flow and RV peak E-wave KEiEDV were independent predictors of RV remodelling index.ConclusionsIn this multi-scanner multicenter 4D flow CMR study, reduced RV direct flow was independently associated with RV dysfunction, remodelling and, to a lesser extent, exercise intolerance in rTOF patients. This supports its utility as an imaging parameter for monitoring disease progression and therapeutic response in rTOF.Clinical Trial Registrationhttps://www.clinicaltrials.gov. Unique identifier: NCT03217240.

Highlights

  • Tetralogy of Fallot (TOF) is the most common form of cyanotic congenital heart disease (CHD) accounting for approximately one in every 3600 livebirths and 5–7% of all CHD

  • Zhao et al Journal of Cardiovascular Magnetic Resonance (2022) 24:4. In this multi-scanner multicenter 4D flow cardiovascular magnetic resonance (CMR) study, reduced right ventricular (RV) direct flow was independently associ‐ ated with RV dysfunction, remodelling and, to a lesser extent, exercise intolerance in repaired tetralogy of Fallot (rTOF) patients

  • There were no significant differences in body surface area (BSA), heart rate, left ventricle (LV) mass index, Left ventricular end-diastolic volume (LVEDV) index, Left ventricular end-systolic volume (LVESV) index, LV stroke volume index, and LV ejection fraction (LVEF) between the two groups

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Summary

Introduction

Tetralogy of Fallot (TOF) is the most common form of cyanotic congenital heart disease (CHD) accounting for approximately one in every 3600 livebirths and 5–7% of all CHD. In a study of 81 paediatric rTOF patients, electrocardiographic (ECG) right bundle branch block-induced prolonged QRS duration, more than PR, was associated with reductions in RVEF and peak ­VO2 [10]. Four-dimensional (4D) flow cardiovascular magnetic resonance (CMR) allows quantification of biven‐ tricular blood flow by flow components and kinetic energy (KE) analyses It remains unclear whether 4D flow parameters can predict cardiopulmonary exercise testing (CPET) as a clinical outcome in repaired tetralogy of Fallot (rTOF). Current study aimed to (1) compare 4D flow CMR parameters in rTOF with age- and gender-matched healthy controls, (2) investigate associations of 4D flow parameters with functional and volumetric right ventricular (RV) remodelling markers, and CPET outcome

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