Abstract and Introduction
Risk stratification of patients with inherited arrhythmia syndromes (IASs) can be challenging. Recent guidelines acknowledge a place for considering the implantable loop recorder (ILR) to outrule malignant arrhythmia as a cause of syncope in certain inherited arrhythmia patients who are at low risk of sudden cardiac death. In this comprehensive literature review, we evaluate the available evidence for the use of the ILR in the IASs and in relatives of victims of sudden arrhythmic death syndrome.
The inherited arrhythmia syndromes (IASs) include the long QT syndrome (LQTS), Brugada syndrome (BrS), catecholaminergic polymorphic ventricular tachycardia (CPVT), short QT syndrome (SQTS), idiopathic ventricular fibrillation (IVF), and progressive cardiac conduction system disease (PCCD). While advances have been made in the last three decades, uncertainties remain regarding the risk stratification of patients with these conditions.
Recent guidelines acknowledge a place for considering the implantable loop recorder (ILR) as a means of rhythm-symptom correlation and to outrule malignant arrhythmia as a cause of syncope. However, case reports of life-threatening arrhythmias recorded on ILRs in patients with IASs serve as a reminder of the need for careful risk analysis before their insertion.[2,3]
Evidence on the use of the ILR in IASs is limited and comes mainly in the form of small, retrospective analyses. Such studies often consider a group of inherited arrhythmia patients, comprised of small numbers with each individual condition. This creates challenges in drawing conclusions for the conditions individually. There is an even greater paucity of published data on the role of the ILR in relatives of patients who suffer sudden arrhythmic death syndrome (SADS). Using conventional inherited cardiac condition evaluation protocols such families are found to have a potentially inherited cardiac disease in just under one-third of cases.
Our aim was to carry out a comprehensive literature review on the evidence for the use of the ILR in patients with IASs and in relatives of patients who had suffered SADS.
Europace. 2022;24(5):706-712. © 2022 Oxford University Press
Copyright 2007 European Heart Rhythm Association of the European Society of Cardiology (ESC). Published by Oxford University Press. All rights reserved.