Low Phillip A, Sandroni Paola, Joyner Michael, Shen Win-Kuang
Department of Neurology, Mayo Clinic, Rochester, Minnesota 55905, USA.
J Cardiovasc Electrophysiol. 2009 Mar;20(3):352-8. doi: 10.1111/j.1540-8167.2008.01407.x. Epub 2009 Jan 16.
POTS is defined as the development of orthostatic symptoms associated with a heart rate (HR) increment >or=30, usually to >or=120 bpm without orthostatic hypotension. Symptoms of orthostatic intolerance are those due to brain hypoperfusion and those due to sympathetic overaction.
We provide a review of POTS based primarily on work from the Mayo Clinic.
Females predominate over males by 5:1. Mean age of onset in adults is about 30 years and most patients are between the ages of 20-40 years. Pathophysiologic mechanisms (not mutually exclusive) include peripheral denervation, hypovolemia, venous pooling, beta-receptor supersensitivity, psychologic mechanisms, and presumed impairment of brain stem regulation. Prolonged deconditioning may also interact with these mechanisms to exacerbate symptoms. The evaluation of POTS requires a focused history and examination, followed by tests that should include HUT, some estimation of volume status and preferably some evaluation of peripheral denervation and hyperadrenergic state. All patients with POTS require a high salt diet, copious fluids, and postural training. Many require beta-receptor antagonists in small doses and low-dose vasoconstrictors. Somatic hypervigilance and psychologic factors are involved in a significant proportion of patients.
POTS is heterogeneous in presentation and mechanisms. Major mechanisms are denervation, hypovolemia, deconditioning, and hyperadrenergic state. Most patients can benefit from a pathophysiologically based regimen of management.
直立性心动过速综合征(POTS)的定义为,与心率(HR)增加≥30次/分钟相关的直立症状的出现,通常心率增加至≥120次/分钟,且无直立性低血压。直立不耐受症状包括因脑灌注不足引起的症状和因交感神经过度兴奋引起的症状。
我们主要基于梅奥诊所的研究成果对POTS进行综述。
女性与男性的患病比例为5:1。成年人的平均发病年龄约为30岁,大多数患者年龄在20至40岁之间。病理生理机制(并非相互排斥)包括外周去神经支配、血容量不足、静脉淤积、β受体超敏反应、心理机制以及推测的脑干调节受损。长期缺乏运动也可能与这些机制相互作用,加重症状。对POTS的评估需要详细的病史询问和体格检查,随后进行的检查应包括直立倾斜试验(HUT)、血容量状态的某种评估,最好还包括外周去神经支配和高肾上腺素能状态的一些评估。所有POTS患者都需要高盐饮食、大量饮水以及体位训练。许多患者需要小剂量的β受体拮抗剂和低剂量的血管收缩剂。相当一部分患者存在躯体过度警觉和心理因素。
POTS在临床表现和发病机制上具有异质性。主要机制包括去神经支配、血容量不足、缺乏运动和高肾上腺素能状态。大多数患者可从基于病理生理学的治疗方案中获益。