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.
Methods: Transthoracic echocardiography with speckle tracking analysis for RV global longitudinal strain (GLS) and 12-lead ECG were performed prospectively on 20 infants following rTOF. Three apical RV views were obtained using analogous imaging planes to the standard LV views to provide a comprehensive evaluation. Regional RV GLS patterns were categorized as synchronous, EMD, or indeterminate. EMD was defined as an early-terminated septal contraction opposed by early stretch and post-systolic peak contraction in the activation delayed RV free wall. An indeterminate pattern was defined as a lack of fully synchronous contraction of all segments but not meeting criteria for EMD. Pre-rTOF echocardiograms and ECGs were analyzed to confirm the presence of synchronous contraction and a normal QRS pattern and duration prior to surgery.
Results: Twenty TOF infants (median age 87 days; 8 days from surgery to post-rTOF evaluation) demonstrated QRSd prolongation following rTOF (pre-rTOF 58 ± 9 ms; post-rTOF 97 ± 14 ms; < 0.001) with new RBBB morphology in all but one patient. All pre-rTOF RV strain patterns were synchronous. Post-rTOF RV strain analysis showed EMD in 25% (5/20) and an indeterminate pattern in 40% (8/20) with the remaining 35% (7/20) maintaining a synchronous pattern, including the patient without RBBB. The EMD group had the lowest RV GLS following repair ( = 0.006).
Discussion: Acquisition of acute QRS prolongation in a RBBB pattern is near-universal following rTOF but without matched or identical patterns of dyssynchrony, suggesting that variations in the time from electrical to electromechanical dyssynchrony potentially caused by differences in right bundle branch anatomy and injury may be relevant to electromechanical outcomes.
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http://dx.doi.org/10.3389/fped.2024.1443924 | DOI Listing |
Cardiol Young
January 2025
Department of Medical Education and Clinical Sciences, Elson S Floyd College of Medicine, Washington State University, Spokane, WA, USA.
Sudden cardiac death poses a significant risk in patients with surgically repaired tetralogy of Fallot. Despite extensive research, risk stratification practices vary. This study surveyed the Pediatric and Adult Congenital Electrophysiology Society to identify these differences.
View Article and Find Full Text PDFBMC Med Genomics
January 2025
Department of Cardiovascular Surgery, Gansu Provincial Hospital, No. 204, Donggang West Road, Lanzhou City, Gansu Province, 730000, China.
Background: We did this study to better clarify the correlations of methylenetetrahydrofolate dehydrogenase 1 (MTHFD1)-G1958A (rs2236225) gene polymorphism with the risk of congenital heart diseases (CHD) and its subgroups.
Methods: Relevant articles were searched in PubMed, Web of Science, Cochrane Library, Embase, CNKI, VIP database and Wanfang DATA until October 2023. We will use odds ratios (ORs) and 95% confidence intervals (CIs) to examine the potential associations of MTHFD1- G1958A gene polymorphism with CHD and its subgroups.
Eur J Hum Genet
January 2025
Center for Medical Genetics, Ghent University Hospital, Ghent, Belgium.
The etiology of congenital heart disease (CHD) is complex, comprising both genetic and environmental factors. Despite documented familial occurrences, the genetic etiology remains largely elusive. Trio exome sequencing identified a heterozygous FLT4 splice site variant in two families with respectively tetralogy of Fallot (TOF), and variable CHD comprising both the TOF spectrum and aortic coarctation.
View Article and Find Full Text PDFInt J Cardiol
January 2025
Department of Cardiology, Cardiovascular Institute, Thorax Center, Erasmus MC, Rotterdam, the Netherlands; European Reference Network for Rare, Low Prevalence and Complex Diseases of the Heart (ERN GUARD-Heart), Amsterdam, the Netherlands.
Background: Little is known about the very long-term outcome in Tetralogy of Fallot (ToF) patients.
Objectives: To prospectively evaluate clinical outcome and quality-of-life after surgical repair of ToF.
Methods: Single-centre, longitudinal cohort-study evaluating every decade 144 ToF patients who underwent surgical repair <15 years of age between 1968 and 1980.
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.
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