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Background: Permanent cardiac pacemakers require invasive procedures with complications often related to long pacemaker leads. We are developing a percutaneous pacemaker for implantation of an entire pacing system into the pericardial space., Methods: Percutaneous micropacemaker implantations were performed in 6 pigs (27.4-34.1 kg) using subxyphoid access to the pericardial space. Modifications in the implantation methods and hardware were made after each experiment as the insertion method was optimized. In the first 5 animals, nonfunctional pacemaker devices were studied. In the final animal, a functional pacemaker was implanted., Results: Successful placement of the entire nonfunctional pacing system into the pericardial space was demonstrated in 2 of the first 5 animals, and successful implantation and capture was achieved using a functional system in the last animal. A sheath was developed that allows retractable features to secure positioning within the pericardial space. In addition, a miniaturized camera with fiberoptic illumination allowed visualization of the implantation site before electrode insertion into myocardium. All animals studied during follow-up survived without symptoms after the initial postoperative period., Conclusions: A novel micropacemaker system allows cardiac pacing without entering the vascular space or surgical exposure of the heart. This pericardial pacemaker system may be an option for a large number of patients currently requiring transvenous pacemakers but is particularly relevant for patients with restricted vascular access, young children, or those with congenital heart disease who require epicardial access., (© 2018 American Heart Association, Inc.)

Background: Although retrograde decremental accessory pathways (DAPs) are thought to typically present as permanent junctional reciprocating tachycardia (permanent junctional reciprocating tachycardia), they may also be diagnosed unexpectedly during electrophysiology study. We aimed to compare the clinical and electrophysiological characteristics of patients with DAPs to an age-matched cohort with nondecremental accessory pathways., Methods and Results: We retrospectively studied pediatric patients (<21 years of age) with retrograde DAPs and an age-matched control population with nondecremental accessory pathways who underwent electrophysiology study between 2005 and 2014. Decrement was defined as rate-dependent prolongation of the local ventriculo-atrial time by >30 ms. Twenty-six patients with DAPs were compared with 73 controls (mean age at electrophysiology study 9.8±5.7 and 10.3±5.2 years, respectively [P=nonsignificant]). Compared with controls, patients with DAPs had more frequent syncope (5/26 [19%] versus 3/73 [4%]; P=0.02) and ventricular dysfunction (6/26 [23%] versus 4/73 [6%]; P=0.04). Only 11 (42%) DAP patients manifested clinical permanent junctional reciprocating tachycardia, and these patients had more syncope (5/11 [45%] versus 0/15 [0%]; P<0.01), slower orthodromic reciprocating tachycardia (176±44 beats per minute versus 229±31 beats per minute; P=0.001), and longer ventriculo-atrial times (mean maximum ventriculo-atrial times of 283±116 ms versus 208±42 ms; P=0.02) compared with those with DAPs without clinical permanent junctional reciprocating tachycardia. DAPs and controls had similar rates of acute ablation success (23/26 [89%] versus 67/73 [92%]; P=nonsignificant) and recurrences (1/23 [4%] versus 2/67 [3%]; P=nonsignificant)., Conclusions: The majority of pediatric patients with DAPs do not present with clinical permanent junctional reciprocating tachycardia. DAPs are associated with more severe symptoms, but ablation outcomes are similar to those of age-matched controls., (© 2016 American Heart Association, Inc.)

Background: Catecholaminergic polymorphic ventricular tachycardia is an uncommon, potentially lethal, ion channelopathy. Standard therapies have high failure rates and little is known about treatment in children. Newer options such as flecainide and left cardiac sympathetic denervation are not well validated. We sought to define treatment outcomes in children with catecholaminergic polymorphic ventricular tachycardia., Methods and Results: This is a Pediatric and Congenital Electrophysiology Society multicenter, retrospective cohort study of catecholaminergic polymorphic ventricular tachycardia patients diagnosed before 19 years of age. The cohort included 226 patients, including 170 probands and 56 relatives. Symptomatic presentation was reported in 176 (78%). Symptom onset occurred at 10.8 (interquartile range, 6.8-13.2) years with a delay to diagnosis of 0.5 (0-2.6) years. Syncope (P<0.001), cardiac arrest (P<0.001), and treatment failure (P=0.008) occurred more often in probands. β-Blockers were prescribed in 205 of 211 patients (97%) on medication, and 25% experienced at least 1 treatment failure event. Implantable cardioverter defibrillators were placed in 121 (54%) and was associated with electrical storm in 22 (18%). Flecainide was used in 24% and left cardiac sympathetic denervation in 8%. Six deaths (3%) occurred during a cumulative follow-up of 788 patient-years., Conclusions: This study demonstrates a malignant phenotype and lengthy delay to diagnosis in catecholaminergic polymorphic ventricular tachycardia. Probands were typically severely affected. β-Blockers were almost universally initiated; however, treatment failure, noncompliance and subtherapeutic dosing were often reported. Implantable cardioverter defibrillators were common despite numerous device-related complications. Treatment failure was rare in the quarter of patients on flecainide. Left cardiac sympathetic denervation was not uncommon although the indication was variable., (© 2015 American Heart Association, Inc.)

Background: Supraventricular tachycardia (SVT) is one of the most common conditions requiring emergent cardiac care in children, yet its management has never been subjected to a randomized controlled clinical trial. The purpose of this study was to compare the efficacy and safety of the 2 most commonly used medications for antiarrhythmic prophylaxis of SVT in infants: digoxin and propranolol., Methods and Results: This was a randomized, double-blind, multicenter study of infants <4 months with SVT (atrioventricular reciprocating tachycardia or atrioventricular nodal reentrant tachycardia), excluding Wolff-Parkinson-White, comparing digoxin with propranolol. The primary end point was recurrence of SVT requiring medical intervention. Time to recurrence and adverse events were secondary outcomes. Sixty-one patients completed the study, 27 randomized to digoxin and 34 to propranolol. SVT recurred in 19% of patients on digoxin and 31% of patients on propranolol (P=0.25). No first recurrence occurred after 110 days of treatment. The 6-month recurrence-free status was 79% for patients on digoxin and 67% for patients on propranolol (P=0.34), and there were no first recurrences in either group between 6 and 12 months. There were no deaths and no serious adverse events related to study medication., Conclusions: There was no difference in SVT recurrence in infants treated with digoxin versus propranolol. The current standard practice may be treating infants longer than required and indicates the need for a placebo-controlled trial. Clinical Trial Registration Information- http://clinicaltrials.gov; NCT-00390546.

Background: Intravenous amiodarone and procainamide are both used as therapies for refractory supraventricular tachycardia (SVT). However, no studies have compared the efficacy and safety of these agents in pediatric patients., Methods and Results: All patients treated with intravenous amiodarone or procainamide during 25 consecutive months for the following mechanisms of SVT were included: orthodromic reciprocating tachycardia, intra-atrial reentrant tachycardia, and ectopic atrial tachycardia; junctional ectopic tachycardia was excluded. Treatment response was categorized as full success, partial success, or failure. Partial success was defined as clinical improvement and/or arrhythmia control but not meeting full success criteria. Adverse events were classified as major (requiring resuscitation) or minor (management changes). There were 40 episodes of SVT in 37 patients (median age, 34 days; 24 with congenital heart disease). Amiodarone was the initial therapy in 26 cases and procainamide in 14 cases. If partial and full success are combined, procainamide was successful in 71% of cases compared with 34% for amiodarone (P=0.046). If partial success is considered a treatment failure, procainamide was successful in 50% compared with 15% for amiodarone (P=0.029). Ten patients received the second medication after the first failed. Success was achieved in 5 of 8 amiodarone-to-procainamide crossovers compared with 1 of 2 procainamide-to-amiodarone crossovers. One major and 10 minor adverse events occurred in amiodarone patients versus 6 minor adverse events in procainamide patients (P=NS)., Conclusions: In this cohort, procainamide achieved greater success compared with amiodarone in the management of recurrent SVT without statistically significant differences in adverse event frequency.