Pediatric and Congenital EPPermanent conduction system pacing for congenitally corrected transposition of the great arteries: A Pediatric and Congenital Electrophysiology Society (PACES)/International Society for Adult Congenital Heart Disease (ISACHD) Collaborative Study
Introduction
Congenitally corrected transposition of the great arteries (CCTGA), also known as l-transposition of the great arteries, is a cardiac disorder characterized by ventricular inversion, coexisting structural abnormalities, and a 2% annual risk for spontaneous atrioventricular (AV) block.1 Approximately 25% of patients with isolated CCTGA and up to 67% of those with concomitant structural heart disease develop right ventricular (RV) failure by age 45 years2 due to maladaptation of a morphologic RV to its role as the systemic ventricle. Patients with CCTGA who undergo pacemaker placement for AV block may experience an accelerated progression to RV failure that can be reversed with cardiac resynchronization therapy (CRT).3,4 Unfortunately, due to significant anatomic variants, conventional CRT through the coronary venous system may be challenging or impossible to accomplish.5,6 Alternate methods to ensure and optimize cardiac resynchronization in this patient population are needed in order to preserve ventricular function.
Although isolated case reports have described the feasibility of cardiac conduction system pacing for CCTGA anatomy,7, 8, 9 the optimal technical approach, relevant anatomic considerations, and mid-term outcomes have not been reported. The aim of the present cohort study is to report the results and findings related to conduction system pacing for CCTGA from an international group of centers experienced in the treatment of complex congenital heart disease.
Section snippets
Methods
After local ethics approval was approved, patients undergoing attempted conduction system pacing between August 2016 and October 2019 were retrospectively identified from 10 participating centers, with data transfer to the coordinating center at UCLA through REDCap (Research Electronic Data Capture). Patients were eligible for inclusion if the underlying cardiac anatomy was CCTGA, as defined by AV and ventriculoarterial discordance. Patients in whom anatomic repair of CCTGA had been performed
Patient characteristics
Fifteen patients with CCTGA and attempted HBP were identified from the 10 participating centers (median 23 years; IQR 15–36; 87% male). Coexisting congenital heart disease lesions were present in 11, and cardiac surgery had been performed in 8. Patient characteristics are given in Table 1. The pacing indication was complete heart block in 10, high-grade AV block in 1, and intermittent AV block in 3. One patient did not have AV block, but HBP was pursued as a prophylactic measure during
Discussion
The major findings of the present study are as follows. (1) Permanent conduction system pacing is feasible among patients with CCTGA anatomy and is associated with favorable mid-term pacing characteristics. (2) Conduction system pacing for CCTGA anatomy can be achieved from the distal His bundle, reproducibly located at the upper septum. (3) Proximal left bundle pacing may also serve as an alternative lead fixation target for CCTGA anatomy.
Given the increased rate of spontaneous AV block in the
Conclusion
Permanent conduction system pacing is feasible in patients with CCTGA. Unique congenital characteristics may favor this approach over conventional CRT given the superficial location of the distal His bundle and left bundle branches, both accessible from a venous approach. Narrow paced QRS and stable lead thresholds were observed at mid-term follow-up. Further study of this strategy in comparison with conventional CRT should be pursued.
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Cited by (28)
Conduction System Pacing for Patients with Congenital Heart Disease
2023, Cardiac Electrophysiology ClinicsConduction System Pacing Versus Conventional Cardiac Resynchronization Therapy in Congenital Heart Disease
2023, JACC: Clinical ElectrophysiologyUse of adjunctive 3-dimensional echocardiogram for His bundle pacing in pediatric patients
2023, HeartRhythm Case ReportsCitation Excerpt :No complications were observed with any patients. Reports of HBP in pediatric and congenital heart disease patients2,3 suggest that the technique is safe, though experience is limited in pediatric patients. Takemoto and colleagues3 first reported a pediatric patient with L-TGA undergoing HBP with a simultaneously placed permanent pacing lead into the subpulmonary, morphological left ventricle.
Reduction of fluoroscopy in conduction system pacing guided by electroanatomical mapping in pediatrics and congenital heart disease
2022, Indian Pacing and Electrophysiology JournalCitation Excerpt :Recently, our group reported feasibility, safety and encouraging outcomes of this type of physiologic pacing in pediatric patients with and without congenital heart disease [9]. Several studies have reported positive outcomes using a minimal fluoroscopy technique for conductive system pacing in the last few years [12], [-17] however, data about the results of this approach in pediatrics and patients with CHD are rather scarce [15,17–20]. In our experience, His bundle and left bundle pacing guided by electroanatomic mapping is a feasible and safe procedure in pediatric patients with and without congenital heart disease.