PDF(Pubmed)
Abstract：
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.
CONCLUSIONS: 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.
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.
CONCLUSIONS: 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.
摘要：
背景：HypermobileEhlers-Danlos综合征（hEDS）是一种结缔组织疾病，其特征是关节过度活动和其他全身性表现，如心血管症状，肌肉骨骼疼痛，关节不稳定。心血管症状,如头晕和心悸，以及自主神经失调的类型，包括体位性心动过速综合征（POTS），经常在患有hEDS的成年人中报告，并已显示对生活质量（QoL）产生负面影响。
目的：本简要综述将概述POTS和hEDS中共同发生的症状，以告知潜在的心血管筛查程序。
结果：虽然许多hEDS患者报告心血管症状，很少有结构异常，这表明自主神经失调可能是这些症状的原因。一种有效的自主神经失调症状负担筛查方法是复合自主神经症状量表（COMPASS-31）。研究发现，成年人有POTS，hEDS,POTS和HEDS的COMPASS-31得分均高于普通人群，表明自主神经失调导致的高症状负担，这导致QoL受损。
结论：虽然研究已经检查了患有和不患有hEDS的成年人的心血管症状和自主神经失调的影响，关于小儿hEDS患者自主神经障碍的文献很少。因此,更多关于心血管症状和自主神经障碍的研究，因为它们与患有hEDS的儿科患者的生活质量有关，是需要的。这篇简短的综述总结了目前关于儿童和成人hEDS患者自主神经失调和心血管症状的文献。
目的：本简要综述将概述POTS和hEDS中共同发生的症状，以告知潜在的心血管筛查程序。
结果：虽然许多hEDS患者报告心血管症状，很少有结构异常，这表明自主神经失调可能是这些症状的原因。一种有效的自主神经失调症状负担筛查方法是复合自主神经症状量表（COMPASS-31）。研究发现，成年人有POTS，hEDS,POTS和HEDS的COMPASS-31得分均高于普通人群，表明自主神经失调导致的高症状负担，这导致QoL受损。
结论：虽然研究已经检查了患有和不患有hEDS的成年人的心血管症状和自主神经失调的影响，关于小儿hEDS患者自主神经障碍的文献很少。因此,更多关于心血管症状和自主神经障碍的研究，因为它们与患有hEDS的儿科患者的生活质量有关，是需要的。这篇简短的综述总结了目前关于儿童和成人hEDS患者自主神经失调和心血管症状的文献。
