Background: Non-invasive imaging markers in patients with repaired tetralogy of Fallot (rTOF) are still being investigated to inform clinical decision making. Atrial function is a prognostic indicator in many acquired and congenital heart diseases. We sought to examine the relationship between cardiac MRI (CMR)-derived indices of left atrial (LA) function, native left ventricular (LV) T1 values, biventricular systolic function, and exercise capacity in rTOF.
Methods: Sixty-six patients with rTOF without prior pulmonary valve replacement who underwent CMR (median age 18.5 years) were identified. Twenty-one adult rTOF patients (age range 19-32 years) were compared with 20 age-matched healthy volunteers (age range 19-34 years). LA reservoir, conduit, and pump global longitudinal strain (GLS) and strain rate (SR) were determined by tissue tracking. Native LV T1 values were measured on rTOF patients. Pearson correlations were performed to determine bivariate associations.
Results: Adult rTOF patients had higher pump GLS, pump:conduit, and pump:reservoir GLS ratios, and lower conduit:reservoir GLS ratio, LV ejection fraction (EF), and right ventricular EF compared to controls (p < 0.001 for each comparison). LA conduit:reservoir GLS and pump:reservoir GLS had correlations to native LV T1 (ρ = 0.26, p = 0.03 and ρ = - 0.26, p = 0.03, respectively). LA reservoir SR had positive correlation to RV EF (ρ = 0.27, p = 0.03). There were no statistically significant correlations between LA function and exercise capacity.
Conclusions: LA function is altered in adolescent and young adult patients with rTOF indicating worse diastolic function and relates to increasing native LV T1 values. Future studies are indicated to investigate the progression of adverse atrial-ventricular interactions and poor outcomes in this population.
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http://dx.doi.org/10.1007/s00246-021-02587-0 | 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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