Background There is limited information regarding the clinical use and effectiveness of IV sotalol in pediatric patients and patients with congenital heart disease, including those with severe myocardial dysfunction. A multicenter registry study was designed to evaluate the safety, efficacy, and dosing of IV sotalol. Methods and Results A total of 85 patients (age 1 day-36 years) received IV sotalol, of whom 45 (53%) had additional congenital cardiac diagnoses and 4 (5%) were greater than 18 years of age. In 79 patients (93%), IV sotalol was used to treat supraventricular tachycardia and 4 (5%) received it to treat ventricular arrhythmias. Severely decreased cardiac function by echocardiography was seen before IV sotalol in 7 (9%). The average dose was 1 mg/kg (range 0.5-1.8 mg/kg/dose) over a median of 60 minutes (range 30-300 minutes). Successful arrhythmia termination occurred in 31 patients (49%, 95% CI [37%-62%]) with improvement in rhythm control defined as rate reduction permitting overdrive pacing in an additional 18 patients (30%, 95% CI [19%-41%]). Eleven patients (16%) had significant QTc prolongation to >465 milliseconds after the infusion, with 3 (4%) to >500 milliseconds. There were 2 patients (2%) for whom the infusion was terminated early. Conclusions IV sotalol was safe and effective for termination or improvement of tachyarrhythmias in 79% of pediatric patients and patients with congenital heart disease, including those with severely depressed cardiac function. The most common dose, for both acute and maintenance dosing, was 1 mg/kg over ~60 minutes with rare serious complications.
Background: Previous studies suggest the relationship between activation time (AT) and action potential duration (APD) in the heart is dependent on electrotonic coupling, but this has not been directly tested. This study assessed whether acute changes in electrical coupling, or other determinants of conduction or repolarization, modulate APD heterogeneity.
Methods And Results: Langendorff-perfused guinea pig hearts were epicardially paced and optically mapped after treatment with the gap junction uncoupler carbenoxolone, ephaptic uncoupler mannitol, ephaptic enhancer dextran 2MDa, sodium channel inhibitor flecainide, or rapid component of the delayed rectifier potassium channel inhibitor E4031.
Infants requiring surgery for congenital heart disease (CHD) are at an increased risk of neurodevelopmental delay. Developmental Care Rounds (DCR) have been implemented widely to support the neurodevelopment of children with CHD while admitted to the hospital. This study aims to describe caregiver perceptions of DCR at a quaternary academic children's heart center.
Velocardiofacial syndrome (VCFS), also known as 22q11.2 deletion syndrome or DiGeorge syndrome, is a complex genetic disorder associated with a spectrum of phenotypic features, including craniofacial anomalies, congenital heart defects, and neurodevelopmental challenges. Among the more concerning, though under-recognized, presentation in VCFS is carotid artery medialization-a finding that places patients at substantial risk for vascular injury during pharyngeal surgeries.
The use of conduction system pacing (CSP) in adults with congenital heart disease (CHD) is growing, however data remain limited. In patients with congenitally corrected transposition of the great arteries following the double switch operation, existing CSP tools and techniques require modification to allow for the anterior displacement of the atrioventricular node and proximal conduction system in addition to navigating the tortuous route of the atrial redirection. We report the successful use of CSP focusing on the technique of delivery tool modification to allow stability on the basal septum for deployment to the area of the distal His bundle and proximal left bundle branch.
