Diagnostic Criteria for Postural Tachycardia Syndrome: Consideration of the Clinical Features Differentiating PoTS from

A diagnosis of Postural Tachycardia Syndrome (PoTS) requires a sustained increase in heart rate of 30 beats per minute or more (40 beats per minute if age 12–19 years) within 10 minutes of assuming upright posture, and in the absence of orthostatic hypote

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iations ADHD Attention deficit hyperactivity disorder HR Heart Rate IOH Initial Orthostatic Hypotension IST Inappropriate Sinus Tachycardia OH Orthostatic Hypotension PoTS Postural Tachycardia Syndrome NET Norepinephrine transporter SNRI Serotonin-norepinephrine reuptake inhibitor

Background The current definition of Postural Tachycardia Syndrome (PoTS) was developed in 1993 by Ron Schondorf and Phillip Low in an effort to provide a standardized profile for this disorder [1]. Prior to this, accounts of PoTS in the literature only referred to small or single sample cases [1]. Through reviewing a set of patients age 20–51  years who demonstrated orthostatic tachycardia during testing at the Mayo Autonomic Reflex Laboratory, a PoTS diagnosis

K. M. Bourne · M. G. Lloyd · S. R. Raj (*)  University of Calgary, Calgary, AB, Canada e-mail: [email protected] S. R. Raj  Vanderbilt University Medical Center, Nashville, TN, USA

was made if the heart rate (HR) increase was 2 standard deviations above the mean increase for a sex-matched control population [1]. Importantly, these data excluded children and adolescents.

Diagnostic Criteria PoTS is a chronic form of orthostatic intolerance marked by excessive orthostatic tachycardia and associated symptoms. Specifically, a PoTS diagnosis is made when, upon assumption of upright posture, a sustained HR increase of 30 beats per minute (bpm) or more is observed in association with symptoms of orthostatic intolerance [2, 3]. In youth under 19 years of age, this HR increase should be 40 bpm or more. Diagnosing PoTS in pediatric patients will be discussed later in this chapter. The duration of the increased HR should be sustained – in other words, seen on at least 2 consecutive recordings. This orthostatic tachycardia should develop within 10 minutes of upright posture [2]. It is not unusual, but not necessary for diagnosis, for the HR to exceed 120 bpm [2, 3]. Orthostatic symptoms should improve with recumbence, and may include lightheadedness, blurry vision, tremulousness, and weakness [2, 3]. The observed postural tachycardia should also be in the absence of orthostatic hypotension (>20/10 mmHg decrease in blood pressure [BP]) [3], and symptoms should

© Springer Nature Switzerland AG 2021 N. Gall et al. (eds.), Postural Tachycardia Syndrome, https://doi.org/10.1007/978-3-030-54165-1_4

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be chronic (lasting at least 3–6 months) [4]. The critical concern is that PoTS patients must have both excessive orthostatic tachycardia and symptoms of orthostatic intolerance in order to meet the criteria for this disorder. The diagnostic criteria are summarized in Table 1.

Clinical Evaluation of a Patient with Suspected PoTS While seemingly simple, there are many caveats to the PoTS diagnosis that are important when differentiating between this disorder and other causes of orthostatic intolerance. All diagnostic criteria must be fulfilled for a diagnosis of PoTS. A more in-depth look at each of the diagnostic criteria is discussed below:

Orthostatic Ta