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Item Permanent 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(Elsevier, 2020-06) Moore, Jeremy P.; Gallotti, Roberto; Shannon, Kevin M.; Pilcher, Thomas; Vinocur, Jeffrey M.; Cano, Óscar; Kean, Adam; Mondesert, Blandine; Nürnberg, Jan-Hendrik; Schaller, Robert D.; Sharma, Parikshit S.; Nishimura, Takuro; Tung, Roderick; Pediatrics, School of MedicineBackground Congenitally corrected transposition of the great arteries (CCTGA) is associated with spontaneous atrioventricular block and pacing-induced cardiomyopathy. Conduction system pacing is a potential alternative to conventional cardiac resynchronization therapy (CRT). Objective The purpose of this study was to determine the outcomes of conduction system pacing for CCTGA. Methods Retrospective data were collected from 10 international centers. Results His bundle (HBP) or left bundle branch pacing (LBBP) was attempted in 15 CCTGA patients (median age 23 years; 87% male). Previous surgery had been performed in 8 and chronic ventricular pacing in 7. Conduction system pacing (11 HBP, 2 LBBP 2; nonselective in 10, selective in 3) was acutely successful in 13 (86%) without complication. In 9 cases, electroanatomic mapping was available and identified the distal His bundle and proximal left bundle branches within the morphologic left ventricle below the pulmonary valve separate from the mitral annulus. Median implant HV interval was 42 ms (interquartile range [IQR] 35–48), R wave 6 mV (IQR 5–18), and threshold 0.5 V (IQR 0.5–1.2) at median 0.5 ms. QRSd was unchanged compared to junctional escape rhythm (124 vs 110 ms; P = .17) and decreased significantly compared to baseline ventricular pacing (112 vs 164 ms; P <.01). At a median of 8 months, all patients were alive without significant change in pacing threshold or lead dysfunction. New York Heart Association functional class improved in 5 patients. Conclusion Permanent conduction system pacing is feasible in CCTGA by either HBP or proximal LBBP. Narrow paced QRS and stable lead thresholds were observed at intermediate follow-up. Unique anatomic characteristics may favor this approach over conventional CRT.Item Significant Lead Migration of a Subcutaneous Implantable Cardioverter-Defibrillator in a Pediatric Patient(MediaSphere Medical, 2017-01-01) Srivastava, Nayan; Kean, Adam; Pediatrics, School of MedicineSince its introduction, the subcutaneous implantable cardioverter-defibrillator (S-ICD) has provided the benefit of reduced mortality from ventricular tachyarrythmias without the associated short- and long-term morbidity of transvenous or epicardial implantable cardioverter-defibrillator (ICD) leads. As its name implies, the S-ICD system is implanted in its entirety, including device and lead, just under the skin beginning along the anterior axillary line, with its lead tunneled to the left parasternum and then from the xiphoid to the manubrium–sternal junction. Dislocation of the lead due to migration of the parasternal lead has been described in a minority of patients. Here, we describe an unusual case of a significant lead migration in a pediatric patient.Item Subcutaneous Implantable Cardioverter-Defibrillators in Pediatrics and Congenital Heart Disease: A Pediatric and Congenital Electrophysiology Society Multicenter Review(Elsevier, 2020-12) von Alvensleben, Johannes C.; Dechert, Brynn; Bradley, David J.; Fish, Frank A.; Moore, Jeremy P.; Pilcher, Thomas A.; Escudero, Carolina; Ceresnak, Scott R.; Kwok, Sit Yee; Balaji, Seshadri; Aziz, Peter F.; Papagiannis, John; Cortez, Daniel; Garnreiter, Jason; Kean, Adam; Schäfer, Michal; Collins, Kathryn K.; Pediatrics, School of MedicineObjectives The primary goal of this study was to evaluate the implant experience and midterm results of subcutaneous implantable cardioverter-defibrillators (S-ICDs) in pediatric patients and those with congenital heart disease. Background The S-ICD was developed to avoid the lead-related complications associated with transvenous systems. The absence of intravascular or intracardiac components offers potential advantages to pediatric patients and those with congenital heart disease. Methods This international, multicenter, retrospective, standard-of-care study was conducted through the Pediatric & Congenital Electrophysiology Society. Complications at 30 and 360 days, inappropriate shocks, and delivery of appropriate therapy were assessed. Results The study included 115 patients with a median follow-up of 32 (19 to 52) months. Median age was 16.7 years (14.8 to 19.3 years), 29% were female, and 55% had a primary prevention indication. Underlying disease substrate was cardiomyopathy (40%), structural heart disease (32%), idiopathic ventricular fibrillation (16%), and channelopathy (13%). The complication rate was 7.8% at 30 days and 14.7% at 360 days. Overall, inappropriate shocks occurred in 15.6% of patients, with no single clinical characteristic reaching statistical significance. At implant, 97.9% of patients had successful first shock conversion with 96% requiring ≤65 J. Appropriate therapy was delivered to 11.2% of patients with an annual incidence of 3.9% and an acute first shock conversion success rate of 92.5%. Conclusions This study found that in a heterogeneous population of pediatric patients and those with congenital heart disease, the S-ICD had comparable rates of complications, inappropriate shocks, and conversion efficacy compared with previously published studies on transvenous systems in similar populations.