In patients with repaired tetralogy of Fallot (rTOF), the regurgitant fraction (RF) in left pulmonary artery (LPA) and right pulmonary artery (RPA) is usually unequal. The morphometrics may play a crucial role in this RF discrepancy. Cardiovascular MR of 79 rTOF patients and 20 healthy controls were retrospectively enrolled. Forty-four from the 79 patients were matched in age, sex and body surface area to the 20 controls and were investigated for: (1) phase-contrast flow of main pulmonary artery (MPA), LPA, and RPA; (2) vascular angles: the angles between the thoracic anterior-posterior line (TAPL) with MPA (θ), MPA with RPA (θ), and MPA with LPA (θ); (3) cardiac angle, the angle between TAPL and the interventricular septum; (4) area ratio of bilateral lung and hemithorax regions. Compared with the 20 controls, the 44 rTOF patients exhibited wider θ, sharper θ angle, and a smaller θ/θ ratio. In the 79 rTOF patients, LPA showed lower forward, backward, and net flow, and greater RF as compared with RPA. Multivariate analysis showed that the RF of LPA was negatively associated with the θ/θ ratio and the age at surgery (R = 0.255). Conversely, the RF of RPA was negatively associated with the left lung/left hemithorax area ratio and cross-sectional area (CSA) of LPA, and positively associated with CSA of RPA and MPA (R = 0.366). In rTOF patients, the RF of LPA is more severe than that of RPA, which may be related to the vascular morphometrics. Different morphometric parameters are independently associated with the RF of LPA or RPA, which may offer potential insights for surgical strategies.
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http://dx.doi.org/10.1007/s10554-023-03035-1 | DOI Listing |
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.
View Article and Find Full Text PDFEuropace
December 2024
Department of Cardiology, Heart Lung Centre, Leiden University Medical Centre, P.O. Box 9600, 2300 RC Leiden, The Netherlands.
Aims: In repaired tetralogy of Fallot (rTOF), the septal anatomical isthmuses (AI), AI 3, between the ventricular septal defect (VSD) and pulmonary annulus, and AI 4, between the VSD and tricuspid annulus, are important ventricular tachycardia (VT) substrates when slow conducting. Our aim was to assess the influence of VSD characteristics, specifically the presence of muscular or fibrous tissue at its border, on the presence or absence of septal AIs, potentially related to VT.
Methods And Results: All consecutive rTOF patients who underwent electroanatomical mapping between January 2005 and March 2023 with an available surgical report providing VSD details (n = 91) were included.
BMC Med Imaging
December 2024
Division of Pediatric Cardiology, Department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol University, 2 Wanglang Road, Bangkoknoi, Bangkok, 10700, Thailand.
Background: Evaluating myocardial function using cardiac magnetic resonance (CMR) feature tracking provides a comprehensive cardiac assessment, particularly a detailed evaluation for patients with repaired tetralogy of Fallot (rTOF). This study aimed to identify factors associated with arrhythmias in rTOF patients utilizing conventional CMR techniques, including myocardial strain measurements.
Methods: This single-center, retrospective study included 245 rTOF patients who underwent CMR between 2017 and 2023.
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