Tale Ermin, Robinson Grace, Edward Justin, Kaushal Riya, Riley Bernadette, Cohen Todd J
43984 New York Institute of Technology College of Osteopathic Medicine , Old Westbury, NY, USA.
Jacobi Medical Center/Albert Einstein College of Medicine, Department of Medicine, Bronx, NY, USA.
J Osteopath Med. 2025 Jun 19. doi: 10.1515/jom-2025-0036.
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.
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.
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).
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.
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.
患有埃勒斯-当洛综合征（EDS）和高活动度谱系障碍（HSD）的患者经常抱怨心悸、先兆晕厥/晕厥，这归因于体位性直立性心动过速综合征（POTS）。偶尔，这些症状的病因并非体位性，尚不清楚，可能需要进一步的心脏评估，包括心脏监测。据我们所知，植入式环路记录仪（ILR）在该人群中的效用尚未得到探索。
本研究旨在评估ILR在诊断和/或排除有症状的EDS和HSD患者中并非由POTS引起的心律失常方面的效用。
2019年1月至2023年11月期间，患有EDS和HSD的患者从纽约理工学院（NYIT）埃勒斯-当洛综合征/高活动度治疗中心转诊至长岛心律中心（LIHRC）进行心脏评估。纽约理工学院骨科医学院机构审查委员会（BHS-1465）允许对LIHRC的观察性去识别数据进行回顾性分析。每月对患者进行监测，并根据症状与心律失常的相关性、设备监测治疗效果的能力和/或排除心律失常作为症状解释的能力来评估ILR的效用。数据以百分比和平均值±标准差（SD）报告。
共评估了116名高活动度患者（81例EDS/35例HSD）。在这些患者中，31例（26.7%）接受了ILR（随访时间为27.3个月±14.0个月），29名女性/2名男性，28例患者患有EDS，3例患者患有HSD，年龄34.1±11.4岁。16例患者（51.6%）出现有症状的窦性心动过速（ST），其中15例（48.4%）的ST有助于诊断POTS和/或监测治疗。8例患者（25.8%）出现有症状的室性早搏（PVC），6例（19.4%）出现室上性心动过速（SVT），1例（3.2%）出现室性心动过速（VT）。在2例患者中，ILR的检查结果导致了进一步的干预措施，包括PVC消融和为有症状的VT植入植入式心脏复律除颤器（ICD）。ILR在所有患者中均显示出效用。
本研究证明了ILR在识别EDS和HSD患者有症状的心律失常方面的效用。ILR监测也有助于巩固POTS的诊断并指导患者管理/治疗效果。需要在更大的队列中进行进一步评估，以进一步了解ILR监测对高活动度患者的影响。
