The utility of the implantable loop recorder in patients with Ehlers-Danlos syndrome and hypermobility spectrum disorder.

Abstract

Context: Patients with Ehlers-Danlos Syndrome (EDS) and hypermobility spectrum disorder (HSD) often complain of palpitations, presyncope/syncope, attributable to postural orthostatic tachycardia syndrome (POTS). Occasionally, the etiology of these complaints is not positional, unclear, and may require further cardiac evaluation, including cardiac monitoring. To our knowledge, the utility of implantable loop recorders (ILRs) has yet to be explored in this population.

Objectives: This study aimed to evaluate the utility of the ILRs in diagnosing and/or excluding arrhythmias not attributable to POTS in symptomatic patients with EDS and HSD.

Methods: Patients with EDS and HSD were referred from the New York Institute of Technology (NYIT) Ehlers-Danlos Syndrome/Hypermobility Treatment Center to the Long Island Heart Rhythm Center (LIHRC) for cardiac evaluation between January 2019 and November 2023. A retrospective analysis of observational de-identified data from the LIHRC was permitted by the NYIT College of Osteopathic Medicine Institutional Review Board (BHS-1465). Patients were monitored monthly, and the utility of the ILRs was assessed based on a correlation of symptoms to an arrhythmia, the ability of the device to monitor therapy efficacy, and/or to rule out an arrhythmia as the explanation of symptoms. Data is reported as percentages and mean ± standard deviation (SD).

Results: A total of 116 hypermobile patients (81 EDS/35 HSD) were evaluated. Among these patients, 31 (26.7 %) received an ILR (length of follow-up, 27.3 months ± 14.0 months), 29 females/2 males, 28 patients had EDS, 3 patients had HSD, age 34.1 ± 11.4 years. Symptomatic sinus tachycardia (ST) occurred in 16 patients (51.6 %), and ST helped diagnose POTS and/or monitor therapy in 15 (48.4 %). Symptomatic premature ventricular complexes (PVCs) occurred in 8 patients (25.8 %), supraventricular tachycardia (SVT) in 6 (19.4 %), and ventricular tachycardia (VT) in 1 (3.2 %). In 2 patients, ILR findings led to further interventions, including PVC ablation and an implantable cardioverter-defibrillator (ICD) for symptomatic VT. The ILR demonstrated utility in all patients.

Conclusions: This study demonstrated the utility of the ILR in identifying symptomatic arrhythmias in patients with EDS and HSD. ILR monitoring also aided in solidifying a POTS diagnosis and guiding patient management/treatment efficacy. Further evaluation in a larger cohort is needed to further understand the impact of ILR monitoring in hypermobile patients.