Life expectancy of patients with rToF has considerably improved due to refined surgical interventions. Monomorphic fast VTs are frequently encountered in adult patients with rToF. The dominant substrate of VT is anatomical isthmuses bordered by surgical incisions, patch material and valve annuli. Substrate based ablation strategies aim to transect all slow conducting anatomical isthmuses (SCAI) as identified by electroanatomical mapping. Procedural success is defined as non-inducibility of VT and confirmed conduction block over the SCAI resulting in long-term VT free survival in most patients. The identification of SCAIs in rToF may have important implications for risk stratification and preventive treatment.
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http://dx.doi.org/10.1016/j.ccep.2017.02.010 | DOI Listing |
Front Pediatr
January 2025
Division of Pediatric Cardiology, Division of Pediatrics, Children's Mercy Hospital, University of Missouri-Kansas City, Kansas City, MO, United States.
Background: In adolescents and adults with tetralogy of Fallot (TOF), right ventricle (RV) electromechanical dyssynchrony (EMD) due to right bundle branch block (RBBB) is associated with reduced exercise capacity and RV dysfunction. While the development of RBBB following surgical repair of tetralogy of Fallot (rTOF) is a frequent sequela, it is not known whether EMD is present in every patient immediately following rTOF. The specific timing of the onset of RBBB following rTOF therefore provides an opportunity to assess whether acute RBBB is associated with the simultaneous acquisition of EMD.
View Article and Find Full Text PDFJACC 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.
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