-
Cardiac Electrophysiology Clinics Jun 2024The following case series presents three different pediatric patients with SCN5A-related disease. In addition, family members are presented to demonstrate the variable... (Review)
Review
The following case series presents three different pediatric patients with SCN5A-related disease. In addition, family members are presented to demonstrate the variable penetrance that is commonly seen. Identifying features of this disease is important, because even in the very young, SCN5A disorders can cause lethal arrhythmias and sudden death.
Topics: Humans; NAV1.5 Voltage-Gated Sodium Channel; Long QT Syndrome; Male; Female; Arrhythmias, Cardiac; Child; Electrocardiography; Child, Preschool; Adolescent; Infant
PubMed: 38749643
DOI: 10.1016/j.ccep.2023.10.007 -
Journal of Cardiovascular... Feb 2024Severe transitory episodes of bradycardia with subsequent syncope in children are common, and generally portend a benign prognosis. Rarely, patients may experience...
INTRODUCTION
Severe transitory episodes of bradycardia with subsequent syncope in children are common, and generally portend a benign prognosis. Rarely, patients may experience prolonged asystolic episodes secondary to significant sinus pauses (SP) or paroxysmal atrioventricular block (AVB). Cardioneuroablation (CNA) is a catheter-based intervention, used to identify and ablate the epicardial ganglionated plexi (GP), which results in disruption of the vagal-mediated parasympathetic input to the sinus and atrioventricular node.
OBJECTIVE
Describe the methodology and role of CNA for treatment of pediatric patients with functional AVB or SP.
METHODS
This is a single-center, case series study. Patients with SP or AVB, 21 years of age or younger, who underwent CNA between 2015 and 2021 were included. CNA was performed via anatomically guided and high-frequency stimulation methods.
RESULTS
Six patients were included. The median age was 18.9 years (range 12.3-20.9 years), 33% female. Two patients had prolonged SP, two had paroxysmal AVB, and two had both SP and AVB. Four patients had prior syncope. The median longest pause was 8.9 s (range 3.9-16.8) with 11 total documented pauses (range 2-231) during the 6 months pre-CNA. Post-CNA, the median longest pause was 1.3 s (range 0.8-2.2) with one documented SP after termination of atrial tachycardia at the 3-month follow-up. At 6 months, the median longest pause was 1.1 s (0.8-1.3) with 0 documented pauses. No patients had syncope post-CNA.
CONCLUSION
CNA may be an effective alternative to pacemaker implantation in pediatric patients with syncope or significant symptoms secondary to functional SP or AVB.
Topics: Humans; Female; Child; Adolescent; Young Adult; Adult; Male; Atrioventricular Block; Sick Sinus Syndrome; Atrioventricular Node; Syncope; Heart Atria; Heart Block; Cardiomyopathies; Genetic Diseases, Inborn
PubMed: 38038245
DOI: 10.1111/jce.16145