Aims: In congenitally corrected transposition of the great arteries (CCTGA) the right ventricle (RV) is systemic. Atrioventricular block (AVB) and systolic dysfunction are frequently observed. Permanent pacing of the subpulmonary left ventricle (LV) may worsen RV dysfunction. The aim of this study was to seek out if LV conduction system pacing (LVCSP) guided by three-dimensional-electroanatomic mapping systems (3D-EAMs) can preserve RV systolic function in paediatric CCTGA patients with AVB.
Methods And Results: Retrospective analysis of CCTGA patients who underwent 3D-EAM-guided LVCSP. Three-dimensional-pacing map guided lead implantation towards septal sites with narrower paced QRS. Electrocardiograms (ECGs), echocardiograms, and lead parameters (threshold, sensing, and impedance) were compared at baseline (pre-implantation) and at 1-year follow-up. Right ventricle function was evaluated by 3D ejection fraction (EF), fractional area change (FAC), RV global longitudinal strain (GLS). Data are reported as median (25th-75th centiles). Seven CCTGA patients aged 15 (9-17) years, with complete/advanced AVB (4 with prior epicardial pacing), underwent 3D-guided LVCSP (5 DDD, 2 VVIR). Baseline echocardiographic parameters were impaired in most patients. No acute/chronic complications occurred. Ventricular pacing was >90%. At 1-year follow-up QRS duration showed no significant changes compared with baseline; however, QRS duration shortened in comparison with prior epicardial pacing. Lead parameters remained acceptable despite ventricular threshold increased. Systemic RV function was preserved: FAC and GLS improved significantly, and all patients showed normal RV EF (>45%).
Conclusion: Three-dimensional-EAM-guided LVCSP preserved RV systolic function in paediatric patients with CCTGA and AVB after short-term follow-up.
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http://dx.doi.org/10.1093/europace/euad026 | DOI Listing |
Heart Rhythm
January 2025
Children's Institute Department of Heart, Vascular & Thoracic, Division of Cardiology & Cardiovascular Medicine, Cleveland Clinic Children's, Cleveland, Ohio. Electronic address:
Background: There is limited data comparing arrhythmia burden amongst patients with congenitally corrected transposition of the great arteries (cc-TGA) undergoing anatomic repair (AR), physiologic repair (PR), and non-surgical management (NS).
Objective: To examine the difference in rate of brady- and tachyarrhythmias amongst patients with cc-TGA stratified by treatment pathway.
Methods: A retrospective cohort study was conducted including all patients with cc-TGA followed at Cleveland Clinic Children's (1995-2021).
Med Image Anal
December 2024
Division of Pediatric Cardiology, Department of Pediatrics, UT Southwestern Medical Center, Dallas, TX, USA. Electronic address:
Patients with congenitally corrected transposition of the great arteries (ccTGA) can be treated with a double switch operation (DSO) to restore the normal anatomical connection of the left ventricle (LV) to the systemic circulation and the right ventricle (RV) to the pulmonary circulation. The subpulmonary LV progressively deconditions over time due to its connection to the low pressure pulmonary circulation and needs to be retrained using a surgical pulmonary artery band (PAB) for 6-12 months prior to the DSO. The subsequent clinical follow-up, consisting of invasive cardiac pressure and non-invasive imaging data, evaluates LV preparedness for the DSO.
View Article and Find Full Text PDFJ Am Coll Cardiol
December 2024
Division of Pediatrics, Cardiothoracic Surgery, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht, the Netherlands.
Background: Congenitally corrected transposition of the great arteries (ccTGA) is a rare cardiac anomaly. The management strategy historically consisted of physiologic repair, leaving the morphologic right ventricle to support the systemic circulation. More recently, anatomic repair has been implemented to bring the left ventricle into the systemic circulation.
View Article and Find Full Text PDFJTCVS Open
October 2024
Department of Congenital and Pediatric Heart Surgery, Deutsches Herzzentrum der Charité, Berlin, Germany.
Objective: Patients with congenitally corrected transposition of the great arteries may require left ventricular training before the double switch operation. We evaluated the effects of combined pressure and volume loading.
Methods: We performed a retrospective study of patients with congenitally corrected transposition of the great arteries who underwent left ventricular training between 2012 and 2022.
Heart Rhythm
November 2024
Department of Pediatrics, National Taiwan University Hospital and Medical College, National Taiwan University, Taipei, Taiwan.
Background: Twin atrioventricular (AV) nodes (TWAVNs) are common in heterotaxy syndrome.
Objective: The purpose of this study was to investigate the presence and implications of TWAVNs and accessory pathways in congenital heart diseases (CHDs) with abnormal AV connections.
Methods: A retrospective study of a 1980-2022 cohort with sufficient electrocardiographic (ECG) data for review was conducted.
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