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.

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Background/Introduction Implantable Loop Recorder (ILR) device monitoring is an established method for long term heart rhythm monitoring in patients with inherited cardiac conditions. Many present with a family history of Sudden Arrhythmic Death Syndrome (SADS). The value of ILR findings in the investigation of SADS relatives has not been well documented. Purpose We aimed to evaluate the impact of ILR monitoring on the management plans of patients with a family history of SADS. Methods We performed a retrospective analysis of the ILR reports and electronic patient records of all patients at the inherited cardiac disease clinic with a family history of SADS and an ILR implanted. Patient demographics, ILR implant indication and specific changes to management plans were recorded and analysed using descriptive statistics. Results All 135 patients with ILR monitoring at the inherited cardiac disease clinic were screened and 87 patients (57.6% female, 41.7±14.0 years) with SADS relatives were included in the study. The mean follow up period was 657.9±392.3 days from ILR implant. Indications for ILR implantation included syncope (n=31, 15.7%), presyncope (45, 22.7%), palpitations (44, 22.2%), chest pain (9, 4.5%), short term heart rhythm monitor findings (6, 3.0%), ECG findings (6, 3.0%), asymptomatic indications (10, (5.1%) including patients with more than one relative with SADS, a family history of conduction disease or family history of long QT syndrome), and atypical symptoms (2 (1%) including seizures and sleep paralysis). Some patients had more than one indication for ILR at the time of implant. As a direct result of ILR monitoring, 43 (49.4%) patients had a change to their management plan. 6 specific definitions for management changes were used: Permanent pacemaker implantation (2, 2.3%), subsequent electrophysiology study (3, 3.5%), medication change (7, 8.1%), arrhythmia excluded as a cause for patient symptoms (26, 29.9%), prompted ILR implant in first degree relative (11, 12.6%) and ILR re-implant for further monitoring for premature conduction disease (1, 1.2%). Patients whose indication for ILR implant was palpitations had the highest likelihood for change of management with 27 changes associated with this indication, of which exclusion of arrhythmia as a cause for symptoms (15) was the most frequent outcome. The indications, syncope and presyncope both yield 21 management changes each. Conclusion The use of ILR devices in family relatives of patients with SADS provides information that may directly impact on patient management, with syncope providing the highest yield and reassurance the most common outcome in our cohort. ILR monitoring helped guide a wide range of other management strategies which included changes to medications and the need for further cardiac procedures. This data represented clinical practice in a niche patient cohort who are at risk for inherited cardiac conditions and associated arrhythmias. FUNDunding Acknowledgement Type of funding sources: None. Indication for ILR vs management change Indications for ILR implant

Background There is limited published data on the use of implantable loop recorders (ILRs) in patients with long QT syndrome (LQTS). Purpose We sought to evaluate the utility of the ILR in patients who had a diagnosis of LQTS or who had a family history of LQTS. Methods We carried out a retrospective analysis of patients attending an Inherited Cardiac Conditions Clinic who had an active ILR and who had a diagnosis or family history of LQTS. Patient demographics, indications for ILR implantation and clinical outcomes were compiled. Results Nine patients with a diagnosis of LQTS and an active ILR were identified with an average age of 37 years. Genetic results were available for seven patients. Four patients had a confirmed pathogenic mutation (one KCNQ1 and three KCNH2), one patient had a variant of uncertain significance in SCN5A and two patients had negative genetic testing. Indications for implantation were syncope (n=4), recurrent altered consciousness episodes (n=1), assessment of occult arrhythmia or beta blocker complications (n=2), palpitations and dizziness (n=1) and nocturnal dyspnoea and palpitations (n=1). Mean follow-up was 706 days and average age at ILR implant was 35 years. The ILR for three patients was a second device, implanted after end of battery life of the initial ILR. ILR recordings led to a change in management in three patients: finding of paroxysmal atrial fibrillation (n=1), finding of nocturnal QTc of 511ms with a change in medications (n=1) and a pause leading to cessation of beta blocker (n=1). Eleven patients with a family history of LQTS and active ILR were identified. Nine patients were female, and the average age was 44.4 years. Genetic test results were available for five patients. Three patients had a pathogenic mutation in SCN5A, and two patients had negative genetic testing for common LQTS genes. Indications for implantation included syncope (n=5), lightheaded episodes (n=3) and risk assessment in gene carriers (n=3). The mean duration of ILR follow-up was 680 days and average age at ILR implant was 42.5 years. Five patients had no arrhythmia detected during ILR recording, two patients had supraventricular tachycardia identified, in two patients ILR data outruled a sinister cause for a symptomatic episode, one patient had documentation of SVT and brief NSVT which led to referral for electrophysiology study and one patient had no recurrence of symptoms and a second ILR was implanted. Conclusion ILRs were inserted in selected patients with a diagnosis of LQTS or in patients with a family history of LQTS or who were gene carriers. Rationale for ILR implantation included risk stratification, monitoring for occult arrhythmia and rhythm-symptom correlation in symptomatic patients. The ILR impacted management in almost 50% of patients and is considered an important tool in selected patients attending Inherited Cardiac Condition Clinics. Funding Acknowledgement Type of funding sources: None. Table 1

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This case report describes a young female Caucasian patient with newly presenting severe mitral stenosis at the peak of the coronavirus pandemic in the Republic of Ireland. The initial presumptive diagnosis was of severe coronavirus illness. This case report highlights the importance of keeping an open mind to alternative diagnoses and examines some of the challenges in the diagnosis and management of a rare condition in the pandemic environment. This patient gained 10 kg of weight within 6 weeks of percutaneous balloon mitral valvuloplasty, highlighting the contribution of cardiac cachexia to her low body weight and demonstrating the exceptional benefit that this treatment can offer to patients.