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Postural tachycardia syndrome (POTS) is a chronic form of orthostatic intolerance associated with cognitive dysfunction. We hypothesized executive function and attention is impaired in POTS during active standing. Eighty-seven POTS participants and 39 healthy controls of similar age, sex, and education level completed executive function (Stroop word-color) and attention (CogState Identification) tests in supine and standing postures in a cross-sectional study. POTS participants had lower executive function (t-score: 48 ± 11 vs. 55 ± 10 control; p = 0.009) and worse attention (reaction speed: 2.78 ± 0.11 vs. 2.69 ± 0.06 control; p 0.001) during standing. These data provide new evidence that active standing impairs attention and executive functioning in POTS.

Objective To study the frequency of latent and overt involuntary emotional expression disorder (IEED) in postural tachycardia syndrome (PoTS). Methods We compared 51 PoTS patients with 15 neurally mediated hypotension (NMH) patients and 12 control subjects using the Center for Neurologic Study-Lability Scale (CNS-LS) for latent IEED and the PHQ-9 questionnaire for depression screening before head-up tilt (HUT). Overt IEED occurrence was observed during HUT. Results HUT precipitated overt IEED in 11 (22%) PoTS patients but not in NMH patients or controls. CNS-LS identified latent IEED in 23 (45%) PoTS patients, 5 (33%) NMH patients, and no control subjects. The CNS-LS tearfulness subscale was elevated in PoTS patients compared with controls (P = 0.0006). The PHQ-9 questionnaire scores were elevated in 69% of PoTS patients and 47% of NMH patients. The PHQ-9 score was higher in PoTS ( Conclusions The study demonstrated frequent occurrence of latent IEED in PoTS and NMH and overt IEED in PoTS without “structural” involvement. HUT was a specific trigger for overt IEED in PoTS. Depression was co-morbid with PoTS. Neurohumoral and cerebral blood flow changes in supine position along with reduced gray matter volume in the insula and cingulate gyrus may underlie susceptibility to IEED. During HUT, cerebral hypoperfusion, rising epinephrine levels, and augmented autonomic arousal provide underpinnings for overt IEED. Clinicians should recognize this entity and counsel PoTS patients and their families. Limiting the duration of orthostatic stress may reduce morbidity.

Postural Tachycardia Syndrome (POTS) is a multi-system disorder that can be associated with significant functional disability. Likely due to the multi-system nature of the disorder, it can be difficult for physicians caring for these patients with POTS to know enough about the management of problems that fall within so many different specialties. This special issue was written for these clinicians and their patients. We have brought together experts on POTS with different backgrounds to share their approach to many of the medical issues that confront patients with POTS. The result is this special POTS Issue of Autonomic Neurosciences that can serve as a “User's Guide to POTS”.

Orthostatic intolerance, including postural tachycardia syndrome, is often associated with gastrointestinal symptoms. In the vast majority of the cases, the gastrointestinal symptoms are not secondary to the orthostatic disorder, but rather just a comorbid condition. This concept is critical, since treatment aimed at the orthostatic condition will not improve the gastrointestinal symptoms. Only when the gastrointestinal symptoms develop in the upright position and improve or resolve in the supine position, they may be related to the orthostatic stress. The most common symptoms associated with orthostatic intolerance include nausea, dyspepsia, bloating and constipation. The majority of subjects do not have gastroparesis. The chapter discusses available treatments of these conditions.

Fatigue is common in POTS, and patients often report unrefreshing sleep. These symptoms are directly correlated with a reduced quality of life, however the treatment of sleep disorders in this population remains a challenge. This article will review the current literature on the prevalence of sleep disorders in POTS, their association with the underlying pathophysiology of POTS, and current treatment paradigms.

Fatigue is a significant symptom that is frequently reported by those with postural tachycardia syndrome (PoTS). There are a variety of reasons why those with PoTS might experience fatigue and as a consequence an individualised approach to management is most appropriate. In this chapter we will examine the prevalence of fatigue in those with PoTS, its overlap with conditions such as chronic fatigue syndrome and describe a clinical approach to the management of fatigue in those with PoTS.

The Benefit of Pacemaker Therapy in Patients with Presumed Neurally-Mediated Syncope and Documented Asystole is Greater when Tilt Test is Negative. An Analysis from the Third International Study on Syncope of Uncertain Etiology (ISSUE-3). Brignole M, Donateo P, Tomaino M, Massa R, Iori M, Beiras X, Moya A, Kus T, Deharo JC, Giuli S, Gentili A, Sutton R. (Lavagna, Italy) Circ Arrhythm Electrophysiol. 2014 Feb;7:10–16

Objective To test the validity and practicality of a simple, rapid autonomic cardiovascular evaluation (RACE). Introduction Assessment of the autonomic nervous system is costly and time consuming. Consequently, briefer measures are often utilized as evaluations of autonomic function. We therefore set out to assess the validity and practicality of a simple, rapid autonomic cardiovascular evaluation protocol which mimicked the bedside evaluations used in many healthcare centers. Design/methods Every eligible patient undergoing full autonomic testing (FAT) in our laboratory was enrolled. The protocol, performed blinded to FAT results, consisted of one breath in the supine position, and supine and standing (1 and 3 min) pressures and pulse. Results were scored for cardiac variation during the breath, a drop in pressure during the stand. Pulse increase was also scored. Aggregate RACE score was regressed against a similarly scored FAT. Results The single breath response correlated poorly with the standard response to deep breathing with convergent results in only 26/37 patients, with a specificity of 30%, and sensitivity of 85%. HR and BP changes standing showed little regression against matching values during FAT. Only 5/11 patients who met the criteria for postural tachycardia syndrome by FAT demonstrated a > 20 bpm increase in HR by 3 min standing. Finally, aggregate scores for FAT and RACE correlated poorly. Discussion The RACE does not satisfy the criteria to serve as a robust dysautonomia screen. Full autonomic testing provides more complete and accurate information than simple bedside assessment.