Philadelphia, PA, USA.
Department of Biostatistics, Epidemiology, and Informatics, The Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA.
Cardiol Young. 2020 Mar;30(3):388-394. doi: 10.1017/S1047951120000165. Epub 2020 Feb 3.
Postural tachycardia syndrome is more frequently being recognised in adolescents and adults. However, its pathophysiology remains undefined. We evaluated our database for patterns in family history of clinical symptoms and associated disorders in these patients.
Patients with postural tachycardia syndrome diagnosed in our clinic between 2014 and 2018 and who were less than 19 years at diagnosis were included. The history was reviewed for family members with postural tachycardia syndrome, dizziness and/or syncope, joint hypermobility with or without hypermobile Ehlers-Danlos syndrome, and autoimmune disorders. Statistical analysis assessed the entire cohort plus differences in gender, presence or absence of joint hypermobility, and presence or absence of familial autoimmune disease.
A total of 579 patients met inclusion criteria. We found that 14.2% of patients had a family member with postural tachycardia syndrome, with male patients more likely to have an affected family member (20% versus 12.7%, p = 0.04). If the patient also had joint hypermobility, male patients were more likely to have a family member with postural tachycardia syndrome (25% versus 12.6%, p = 0.017), more than one affected family member (7.1% versus 0.74%, p = 0.001), and a family member with joint hypermobility (37.5% versus 23.7%, p = 0.032). Autoimmune disease was seen in 45.1% of family members, but more likely in female patients with concurrent hypermobility (21.1% versus 8.9%, p = 0.035).
This in-depth analysis of associated familial disorders in patients with postural tachycardia syndrome offers further insight into the pathophysiology of the disorder, and informs further screening of family members in these patients.
体位性心动过速综合征在青少年和成年人中越来越常见。然而，其病理生理学仍未确定。我们评估了我们的数据库中这些患者的家族史中临床症状和相关疾病的模式。
纳入 2014 年至 2018 年在我院诊断为体位性心动过速综合征且诊断时年龄小于 19 岁的患者。回顾病史，了解家族成员是否存在体位性心动过速综合征、头晕和/或晕厥、伴或不伴易发性埃勒斯-当洛斯综合征的关节过度活动、自身免疫性疾病。统计分析评估了整个队列以及性别、是否存在关节过度活动、是否存在家族性自身免疫性疾病的差异。
共有 579 名患者符合纳入标准。我们发现 14.2%的患者有家族成员患有体位性心动过速综合征，男性患者更有可能有受影响的家族成员（20%比 12.7%，p = 0.04）。如果患者还存在关节过度活动，男性患者更有可能有家族成员患有体位性心动过速综合征（25%比 12.6%，p = 0.017）、不止一个受影响的家族成员（7.1%比 0.74%，p = 0.001）和有一个关节过度活动的家族成员（37.5%比 23.7%，p = 0.032）。家族成员中有 45.1%患有自身免疫性疾病，但在同时存在过度活动的女性患者中更为常见（21.1%比 8.9%，p = 0.035）。
本研究深入分析了体位性心动过速综合征患者相关家族疾病，进一步了解了该疾病的病理生理学，并为这些患者的家族成员进一步筛查提供了依据。
