高移动性埃勒斯-丹洛斯综合征成人和儿童患者的心血管症状、自主神经功能障碍和生活质量:简要回顾

IF 2.4 Q2 CARDIAC & CARDIOVASCULAR SYSTEMS Current Cardiology Reviews Pub Date : 2024-01-24 DOI:10.2174/011573403X271096231203164216
Amanda Hertel, William R Black, Lindsey Malloy Walton, Julie Martin, Jordan Jones
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引用次数: 0

摘要

背景:活动过度埃勒斯-丹洛斯综合征(hEDS)是一种结缔组织疾病,其特点是关节活动过度和其他全身表现,如心血管症状、肌肉骨骼疼痛和关节不稳定。头晕和心悸等心血管症状以及包括体位性正位性心动过速综合征(POTS)在内的各种类型的自律神经失调症,是成人 HEDS 患者的常见症状,已被证明会对生活质量(QoL)产生负面影响:本文将概述 POTS 和 hEDS 的并发症状,为潜在的心血管筛查程序提供参考:结果:虽然许多 hEDS 患者都有心血管症状,但很少有结构异常的患者,这表明这些症状很可能是由自主神经功能障碍引起的。综合自律神经症状量表 (COMPASS-31) 是一种有效的自律神经症状负担筛查方法。研究发现,患有 POTS、hEDS 以及同时患有 POTS 和 hEDS 的成年人的 COMPASS-31 得分高于普通人群,这表明自主神经失调导致了较高的症状负担,从而损害了 QoL:虽然已有研究探讨了成人和非成人 hEDS 患者的心血管症状和自主神经功能障碍的影响,但有关 hEDS 儿童患者自主神经功能障碍的文献却很少。因此,需要更多关于心血管症状和自主神经功能障碍的研究,因为它们与 hEDS 儿童患者的生活质量有关。这篇简短的综述总结了目前有关 hEDS 儿科和成人患者自律神经失调和心血管症状的文献。
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Cardiovascular Symptoms, Dysautonomia, and Quality of Life in Adult and Pediatric Patients with Hypermobile Ehlers-Danlos Syndrome: A Brief Review.

Background: Hypermobile Ehlers-Danlos Syndrome (hEDS) is a connective tissue disorder characterized by joint hypermobility and other systemic manifestations, such as cardiovascular symptoms, musculoskeletal pain, and joint instability. Cardiovascular symptoms, such as lightheadedness and palpitations, and types of dysautonomia, including postural orthostatic tachycardia syndrome (POTS), are frequently reported in adults with hEDS and have been shown to negatively impact quality of life (QoL).

Objective: This brief review will be an overview of co-occurring symptoms in POTS and hEDS to inform potential cardiovascular screening procedures.

Results: While many patients with hEDS report cardiovascular symptoms, few have structural abnormalities, suggesting that dysautonomia is likely responsible for these symptoms. One validated screening measure for dysautonomia symptom burden is the Composite Autonomic Symptom Scale (COMPASS-31). Studies have found that adults with POTS, hEDS, and both POTS and hEDS have higher COMPASS-31 scores than the general population, suggesting a high symptom burden due to dysautonomia, which leads to impaired QoL.

Conclusion: While studies have examined cardiovascular symptoms and the impact of dysautonomia in adults with and without hEDS, there is scant literature on dysautonomia in pediatric patients with hEDS. Therefore, more studies on cardiovascular symptoms and dysautonomia, as they relate to the quality of life in pediatric patients with hEDS, are needed. This brief review summarizes the current literature on dysautonomia and cardiovascular symptoms in pediatric and adult populations with hEDS.

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来源期刊
Current Cardiology Reviews
Current Cardiology Reviews CARDIAC & CARDIOVASCULAR SYSTEMS-
CiteScore
3.70
自引率
10.50%
发文量
117
期刊介绍: Current Cardiology Reviews publishes frontier reviews of high quality on all the latest advances on the practical and clinical approach to the diagnosis and treatment of cardiovascular disease. All relevant areas are covered by the journal including arrhythmia, congestive heart failure, cardiomyopathy, congenital heart disease, drugs, methodology, pacing, and preventive cardiology. The journal is essential reading for all researchers and clinicians in cardiology.
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