Thieben Mark J, Sandroni Paola, Sletten David M, Benrud-Larson Lisa M, Fealey Robert D, Vernino Steven, Lennon Vanda A, Shen Win-Kuang, Low Phillip A
Autonomic Disorders Center, Department of Neurology, Mayo Clinic College of Medicine, Rochester, Minn 55905, USA.
Mayo Clin Proc. 2007 Mar;82(3):308-13. doi: 10.4065/82.3.308.
To evaluate the prevalence and pathogenetic mechanisms of postural orthostatic tachycardia syndrome (POTS).
We reviewed the medical records of patients with POTS seen at the Mayo Clinic in Rochester, Minn, from January 1, 1993, through December 31, 2003. All patients were required to have had a full autonomic reflex screen. The results of the following additional tests were evaluated: thermoregulatory sweat test, plasma catecholamine measurement, serum ganglionic (a3) acetylcholine receptor antibody detection, and 24-hour urinary sodium measurement.
We identified 152 patients (86.8% female; mean +/- SD age, 30.2+/-10.3 years) with a mean duration of symptoms of 4.1 years. The mean orthostatic heart rate increment was 44 beats/min. Half the patients had sudomotor abnormalities (apparent on both the quantitative sudomotor axon reflex test and thermoregulatory sweat test), and 34.9% had significant adrenergic impairment, indicating that at least half of the patients had a neuropathic pattern of POTS. In 13.8% of patients, onset was subacute, and ganglionic acetylcholine receptor antibody was detected in 14.6%, suggesting an autoimmune origin in at least 1 in 7 patients. Hyperadrenergic status was documented in 29.0% of patients (standing plasma norepinephrine level 2600 pg/mL), and at least 28.9% were presumably hypovolemic (24-hour urinary sodium level <100 mEq/24h). The lack of correlation between urinary sodium and standing norepinephrine levels suggests that mechanisms other than hypovolemia accounted for the hyperadrenergic state.
Our findings suggest a neuropathic basis for at least half the cases of POTS and that a substantial percentage of cases may be autoimmune. Hyperadrenergic and hypovolemic correlates are likely compensatory or exacerbating.
评估体位性直立性心动过速综合征（POTS）的患病率及发病机制。
我们回顾了1993年1月1日至2003年12月31日期间在明尼苏达州罗切斯特市梅奥诊所就诊的POTS患者的病历。所有患者均需进行全面的自主神经反射检查。评估了以下额外检查的结果：体温调节汗液试验、血浆儿茶酚胺测量、血清神经节（α3）乙酰胆碱受体抗体检测以及24小时尿钠测量。
我们确定了152例患者（86.8%为女性；平均年龄±标准差为30.2±10.3岁），症状平均持续时间为4.1年。平均直立性心率增量为44次/分钟。一半的患者有汗腺功能异常（定量汗腺轴突反射试验和体温调节汗液试验均显示异常），34.9%的患者有明显的肾上腺素能损害，这表明至少一半的患者有神经性POTS模式。13.8%的患者起病为亚急性，14.6%的患者检测到神经节乙酰胆碱受体抗体，这表明至少七分之一的患者有自身免疫性病因。29.0%的患者记录有高肾上腺素能状态（站立时血浆去甲肾上腺素水平≥600 pg/mL），至少28.9%的患者可能存在血容量不足（24小时尿钠水平<100 mEq/24h）。尿钠与站立时去甲肾上腺素水平之间缺乏相关性，提示血容量不足以外的机制导致了高肾上腺素能状态。
我们的研究结果表明，至少一半的POTS病例有神经病变基础，且相当比例的病例可能是自身免疫性的。高肾上腺素能和血容量不足相关因素可能是代偿性的或会加重病情。
