Background And Objective: The effect of chronic pulmonary regurgitation (PR) on right ventricular (RV) dysfunction in repaired Tetralogy of Fallot (RTOF) patients is well recognized by cardiac magnetic resonance (CMR). However, the link between RV wall motion, intracardiac flow and PR has not been established. Hemodynamic force (HDF) represents the global force exchanged between intracardiac blood volume and endocardium, measurable by 4D flow or by a novel mathematical model of wall motion. In our study, we used this novel methodology to derive HDF in a cohort of RTOF patients, exclusively using routine CMR imaging.
Methods: RTOF patients and controls with CMR imaging were retrospectively included. Three-dimensional (3D) models of RV were segmented, including RV outflow tract (RVOT). Feature-tracking software (QStrain 2.0, Medis Medical Imaging Systems, Leiden, Netherlands) captured endocardial contours from long/short-axis cine and used to reconstruct RV wall motion. A global HDF vector was computed from the moving surface, then decomposed into amplitude/impulse of three directional components based on reference (Apical-to-Basal, Septal-to-Free Wall and Diaphragm-to-RVOT direction). HDF were compared and correlated against CMR and exercise stress test parameters. A subset of RTOF patients had 4D flow that was used to derive vorticity (for correlation) and HDF (for comparison against cine method).
Results: 68 RTOF patients and 20 controls were included. RTOF patients had increased diastolic HDF amplitude in all three directions (<0.05). PR% correlated with Diaphragm-RVOT HDF amplitude/impulse ( = 0.578, <0.0001, = 0.508, < 0.0001, respectively). RV ejection fraction modestly correlated with global HDF amplitude ( = 0.2916, = 0.031). VO correlated with Septal-to-Free Wall HDF impulse ( = 0.536, = 0.007). Diaphragm-to-RVOT HDF correlated with RVOT vorticity ( = 0.4997, = 0.001). There was no significant measurement bias between Cine-derived HDF and 4D flow-derived HDF by Bland-Altman analysis.
Conclusion: RTOF patients have abnormal diastolic HDF that is correlated to PR, RV function, exercise capacity and vorticity. HDF can be derived from conventional cine, and is a potential link between RV wall motion and intracardiac flow from PR in RTOF patients.
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http://dx.doi.org/10.3389/fcvm.2022.929470 | DOI Listing |
JACC Clin Electrophysiol
January 2025
Montreal Heart Institute, Montreal, Québec, Canada.
Background: Ventricular tachycardia (VT) substrate characteristics before transcatheter pulmonary valve replacement (TPVR) in repaired tetralogy of Fallot (rTOF) are unknown.
Objectives: In this study, the authors sought to evaluate substrates for sustained monomorphic VT before TPVR in rTOF.
Methods: Retrospective (2017 to 2021) and prospective (commencing 2021) rTOF patients with native right ventricular outflow tract referred for electrophysiology study (EPS) before TPVR were included.
JACC Asia
December 2024
National Heart Research Institute Singapore, National Heart Centre Singapore, Singapore.
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.
Int J Cardiol Congenit Heart Dis
June 2024
Division of Cardiology, Department of Medicine, University of British Columbia, Vancouver, Canada.
Background: Surgically repaired Tetralogy of Fallot (rTOF) is associated with progressive right ventricular hypertrophy (RVH) and dilation (RVD). Accurate estimation of RVH/RVD is vital for the ongoing management of this patient population. The utility of the ECG in evaluating patients with rTOF with pre-existing right bundle branch block (RBBB) has not been studied.
View Article and Find Full Text PDFInt J Cardiol Congenit Heart Dis
September 2024
The Blalock-Taussig-Thomas Pediatric and Congenital Heart Center, Department of Pediatrics, Johns Hopkins School of Medicine, Johns Hopkins University, 600 N. Wolfe Street, 1389 Blalock, Baltimore, 21287, MD, USA.
Objective: Repaired Tetralogy of Fallot (rTOF), a complex congenital heart disease, exhibits substantial clinical heterogeneity. Accurate prediction of disease progression and tailored patient management remain elusive. We aimed to categorize rTOF patients into distinct phenotypes based on clinical variables and variables obtained from cardiac magnetic resonance (CMR) imaging.
View Article and Find Full Text PDFAnn Pediatr Cardiol
November 2024
Department of Pediatric Cardiology, Apollo Hospitals, Chennai, Tamil Nadu, India.
Transcatheter pulmonary valve implantation (TPVI) is the standard of care in patients with repaired tetralogy of Fallot (rTOF) presenting with right ventricular outflow tract (RVOT) dysfunction. However, the feasibility of TPVI is limited by the high cost and nonavailability of larger-sized valves for dilated native RVOT of rTOF patients. We report the first successful TPVI with a custom-made 35 mm balloon-expandable valve (Myval™) in a 30-year-old rTOF patient with severe pulmonary regurgitation and RV dysfunction.
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