ABSTRACT
BACKGROUND: Implantable loop recorders (ILRs) are conventionally utilized to elucidate the mechanism of atypical syncope. The objective of this study was to assess the impact of these devices on management of pediatric patients with known or suspected inherited arrhythmia syndromes. METHODS AND RESULTS: A retrospective chart review was undertaken of all pediatric patients with known or suspected inherited arrhythmia syndromes in whom an ILR was implanted from 2008 to 2015. Captured data included categorization of diagnosis, treatment, transmitted tracings, and the impact of ILR tracings on management. Transmissions were categorized as symptomatic, autotriggered, or routine. Actionable transmissions were abnormal tracings that directly resulted in a change of medical or device therapy. A total of 20 patients met the stated inclusion criteria (long QT syndrome, n=8, catecholaminergic polymorphic ventricular tachycardia,n=9, Brugada syndrome, n=1, arrhythmogenic right ventricular cardiomyopathy, n=2), with 60% of patients being genotype positive. Primary indication for implantation of ILR included ongoing monitoring +/- symptoms (n=15, 75%), suspicion of noncompliance (n=1, 5%), and liberalization of recommended activity restrictions (n=4, 25%). A total of 172 transmissions were received in patients with inherited arrhythmia syndromes, with 7% yielding actionable data. The majority (52%) of symptom events were documented in the long QT syndrome population, with only 1 tracing (5%) yielding actionable data. Automatic transmissions were mostly seen in the catecholaminergic polymorphic ventricular tachycardia cohort (81%), with 21% yielding actionable data. There was no actionable data in routine transmissions. CONCLUSIONS: ILRs in patients with suspected or confirmed inherited arrhythmia syndromes may be useful for guiding management. Findings escalated therapies in 30% of subjects. As importantly, in this high-risk population, the majority of symptom events represented normal or benign rhythms, reassuring patients and physicians that no further intervention was required.
Subject(s)
Arrhythmias, Cardiac/therapy , Prostheses and Implants , Adolescent , Arrhythmias, Cardiac/diagnosis , Arrhythmias, Cardiac/genetics , Child , Child, Preschool , Death, Sudden, Cardiac/prevention & control , Female , Humans , Male , Monitoring, Ambulatory/instrumentation , Retrospective Studies , Treatment OutcomeSubject(s)
Diagnostic Errors , Heart Septal Defects, Atrial/diagnosis , Heart Septal Defects, Atrial/surgery , Pulmonary Veins/abnormalities , Pulmonary Veins/surgery , Vascular Surgical Procedures/adverse effects , Child , Humans , Middle Aged , Postoperative Complications/diagnosis , Postoperative Complications/etiology , Time FactorsABSTRACT
A 9-year-old boy was found to have ruptured sinus of Valsalva aneurysm (RSVA) and aortic coarctation. Following relief of aortic coarctation by balloon angioplasty, transcatheter coil occlusion of the RSVA was performed successfully under transesophageal echocardiographic and fluoroscopic monitoring; bioptome-assisted delivery of 0.052" Gianturco coil was undertaken via a 7 Fr sheath stabilized by an 0.035" guidewire passing through the RSVA and the sheath. This report details the technique of occlusion.
