Boris Jeffrey R
Division of Cardiology, Department of Pediatrics, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania 19104, United States of America.
Cardiol Young. 2010 Dec;20 Suppl 3:135-9. doi: 10.1017/S1047951110001198.
Dysfunction of the autonomic nervous system, or dysautonomia, is an uncommon disease. Postural orthostatic tachycardia syndrome is one of the several types of dysautonomia. Postural orthostatic tachycardia syndrome, also known as chronic orthostatic intolerance, is the most common but least severe of the dysautonomic disorders; it will serve as the model for evaluation and management of the other dysautonomias. Overall, these patients can have variable dysfunction of the autonomic nervous system that is more severe than that observed in typical neurocardiogenic syncope. Frequently, providers are not familiar with either the evaluation or the management of this syndrome, or are just not interested in doing so. This article attempts to describe strategies for evaluation and management of postural orthostatic tachycardia syndrome. The diagnosis of postural orthostatic tachycardia syndrome is made by the finding of orthostatic intolerance associated with a pulse greater than 120 beats per minute in the first 10 minutes of upright position or an elevation in pulse greater than 30 beats per minute in the first 10 minutes of upright position. Overall, these patients can have variable dysfunction of the autonomic nervous system that is more severe than that seen in typical neurocardiogenic syncope. A wide variety of associated symptoms may exist and these symptoms can have tremendous impact on the lives of the patients and their families. Management of these patients can be difficult as well as rewarding. It is helpful to perform an extensive education up front with these patients and their families. Interventions for patients with postural orthostatic tachycardia syndrome typically fall into two broad categories: non-pharmacological and pharmacological. Non-pharmacological therapies are varied, but are based primarily on ensuring adequate status of intravascular fluid. Polypharmacy may be required to control symptoms associated with postural orthostatic tachycardia syndrome. On account of the severity of their symptoms, these patients frequently have difficulty in completing their school assignments. The physician may need to help support the attempts of the family to work with the school to help the patient stay in school. As postural orthostatic tachycardia syndrome is underdiagnosed and poorly understood, it is a disease that provides an excellent opportunity to perform research. The most important studies would be those that aim to elucidate an aetiology and a pathophysiology of postural orthostatic tachycardia syndrome. In the final analysis, the role of the cardiologist in the evaluation and management of a patient with dysautonomia is to help a patient with severe disability to feel as if they are normal, or much closer to it.
自主神经系统功能障碍，即自主神经失调，是一种罕见疾病。体位性直立性心动过速综合征是自主神经失调的几种类型之一。体位性直立性心动过速综合征，也称为慢性直立不耐受，是自主神经失调性疾病中最常见但最不严重的一种；它将作为评估和管理其他自主神经失调的模型。总体而言，这些患者的自主神经系统功能障碍程度各异，且比典型的神经心源性晕厥更为严重。通常，医疗服务提供者对该综合征的评估或管理并不熟悉，或者根本不感兴趣。本文试图描述体位性直立性心动过速综合征的评估和管理策略。体位性直立性心动过速综合征的诊断依据是，在直立位的前10分钟内发现直立不耐受且脉搏大于每分钟120次，或在直立位的前10分钟内脉搏升高大于每分钟30次。总体而言，这些患者的自主神经系统功能障碍程度各异，且比典型的神经心源性晕厥更为严重。可能存在各种各样的相关症状，这些症状会对患者及其家人的生活产生巨大影响。对这些患者的管理既困难又有意义。预先对这些患者及其家人进行广泛的教育会有所帮助。体位性直立性心动过速综合征患者的干预措施通常分为两大类：非药物治疗和药物治疗。非药物治疗方法多种多样，但主要基于确保血管内液体状态充足。可能需要联合用药来控制与体位性直立性心动过速综合征相关的症状。由于症状严重，这些患者在完成学校作业时经常遇到困难。医生可能需要帮助支持家庭与学校合作，以帮助患者继续留在学校。由于体位性直立性心动过速综合征诊断不足且了解甚少，它是一种进行研究的绝佳疾病。最重要的研究将是那些旨在阐明体位性直立性心动过速综合征病因和病理生理学的研究。归根结底，心脏病专家在自主神经失调患者评估和管理中的作用是帮助严重残疾的患者感觉自己正常，或者更接近正常。
