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Objectives: Comorbidities associated with venous origin chronic pelvic pain (VO-CPP) were evaluated pre and post venous treatment to assess change., Materials and Methods: 45 women with VO-CPP were treated with venous stenting and/or embolization. Four surveys assessed symptoms pre- and post-treatment: IPPS (chronic pelvic pain), PUF (interstitial cystitis), OHQ (dysautonomia), and modified ROME III (IBS). Prevalence of joint hypermobility was investigated., Results: Ages were 18-65. Pretreatment, 64% and 49% of women were in the severe range for PUF and OHQ, respectively. 40% and 56% met criteria for IBS and Ehlers-Danlos syndrome/Hypermobility Spectrum Disorder (EDS/HSD), respectively. 17eceived an iliac stent, 5 pelvic embolization, and 23 both. Post-treatment, average scores improved: IPPS (by 55%), PUF (34%), and OHQ (49%). Rome III improved only slightly., Conclusion: Pelvic pain, interstitial cystitis, and dysautonomia were frequently found with VO-CPP and improved after venous treatment. EDS/HSD and IBS were common in these women., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Individuals with Chiari malformation can present with symptoms of fatigue, lightheadedness, and syncope-the cardinal features of orthostatic intolerance. Similar orthostatic symptoms can complicate the clinical course following Chiari decompression. The presence of orthostatic intolerance in patients with Chiari malformation is not surprising given the location of the major circulatory control centers and their pathways in the brainstem. This article reviews the normal physiologic response to upright posture and the common forms of orthostatic intolerance encountered in clinical practice. The authors describe the relationship between orthostatic intolerance and Chiari malformation and provide suggestions regarding the evaluation and management of these disorders., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Postural orthostatic tachycardia syndrome (POTS) is a chronic and often disabling disorder characterized by orthostatic intolerance with excessive heart rate increase without hypotension during upright posture. Patients often experience a constellation of other typical symptoms including fatigue, exercise intolerance and gastrointestinal distress. A typical patient with POTS is a female of child-bearing age, who often first displays symptoms in adolescence. The onset of POTS may be precipitated by immunological stressors such as a viral infection. A variety of pathophysiologies are involved in the abnormal postural tachycardia response; however, the pathophysiology of the syndrome is incompletely understood and undoubtedly multifaceted. Clinicians and researchers focused on POTS convened at the National Institutes of Health in July 2019 to discuss the current state of understanding of the pathophysiology of POTS and to identify priorities for POTS research. This article, the first of two articles summarizing the information discussed at this meeting, summarizes the current understanding of this disorder and best practices for clinical care. The evaluation of a patient with suspected POTS should seek to establish the diagnosis, identify co-morbid conditions, and exclude conditions that could cause or mimic the syndrome. Once diagnosed, management typically begins with patient education and non-pharmacologic treatment options. Various medications are often used to address specific symptoms, but there are currently no FDA-approved medications for the treatment of POTS, and evidence for many of the medications used to treat POTS is not robust., (Copyright © 2021 The Authors. Published by Elsevier B.V. All rights reserved.)

The National Institutes of Health hosted a workshop in 2019 to build consensus around the current state of understanding of the pathophysiology of postural orthostatic tachycardia syndrome (POTS) and to identify knowledge gaps that must be addressed to enhance clinical care of POTS patients through research. This second (of two) articles summarizes current knowledge gaps, and outlines the clinical and research priorities for POTS. POTS is a complex, multi-system, chronic disorder of the autonomic nervous system characterized by orthostatic intolerance and orthostatic tachycardia without hypotension. Patients often experience a host of other related disabling symptoms. The functional and economic impacts of this disorder are significant. The pathophysiology remains incompletely understood. Beyond the significant gaps in understanding the disorder itself, there is a paucity of evidence to guide treatment which can contribute to suboptimal care for this patient population. The vast majority of physicians have minimal to no familiarity or training in the assessment and management of POTS. Funding for POTS research remains very low relative to the size of the patient population and impact of the syndrome. In addition to efforts to improve awareness and physician education, an investment in research infrastructure including the development of standardized disease-specific evaluation tools and outcome measures is needed to facilitate effective collaborative research. A national POTS research consortium could facilitate well-controlled multidisciplinary clinical research studies and therapeutic trials. These priorities will require a substantial increase in the number of research investigators and the amount of research funding in this area., (Copyright © 2021 The Authors. Published by Elsevier B.V. All rights reserved.)

Background: Orthostatic intolerance (OI) is a frequent finding in individuals with myalgic encephalomyelitis /chronic fatigue syndrome (ME/CFS). Published studies have proposed that deconditioning is an important pathophysiological mechanism in various forms of OI, including postural orthostatic tachycardia syndrome (POTS), however conflicting opinions exist. Deconditioning can be classified objectively using the predicted peak oxygen consumption (VO 2 ) values from cardiopulmonary exercise testing (CPET). Therefore, if deconditioning is an important contributor to OI symptomatology, one would expect a relation between the degree of reduction in peak VO 2 during CPET and the degree of reduction in CBF during head-up tilt testing (HUT)., Methods and Results: In 22 healthy controls and 199 ME/CFS patients were included. Deconditioning was classified by the CPET response as follows: %peak VO 2  ≥ 85% = no deconditioning, %peak VO 2 65-85% = mild deconditioning, and %peak VO 2  < 65% = severe deconditioning. HC had higher oxygen consumption at the ventilatory threshold and at peak exercise as compared to ME/CFS patients (p ranging between 0.001 and < 0.0001). Although ME/CFS patients had significantly greater CBF reduction than HC (p < 0.0001), there were no differences in CBF reduction among ME/CFS patients with no, mild, or severe deconditioning. We classified the hemodynamic response to HUT into three categories: those with a normal heart rate and blood pressure response, postural orthostatic tachycardia syndrome, or orthostatic hypotension. No difference in the degree of CBF reduction was shown in those three groups., Conclusion: This study shows that in ME/CFS patients orthostatic intolerance is not caused by deconditioning as defined on cardiopulmonary exercise testing. An abnormal high decline in cerebral blood flow during orthostatic stress was present in all ME/CFS patients regardless of their %peak VO 2 results on cardiopulmonary exercise testing.

Following concussion, children often experience nonspecific symptoms that overlap with those of other common pediatric conditions, including orthostatic intolerance (OI). The primary goal of this study was to evaluate OI in youth presenting for clinical care for concussion and reporting symptoms frequently observed in OI. Eighty-two of 114 patients aged 10 to 22 years endorsed symptoms based on 8 screening questions, and 24 of those 82 patients met criteria for OI based on an active standing test. No screening question generated clinically useful likelihood ratios for predicting OI. The prevalence of OI in this cohort is considerably higher than estimates in the general pediatric population, suggesting a link between concussion and OI. Future work is needed to evaluate the best method of testing for OI, the natural history of OI symptoms in youth with concussion, and the response to intervention in children with both concussion and OI.

Unlabelled: INTRODUCTION/RESULTS: In 17 patients, chronic idiopathic nausea was associated with orthostatic intolerance (OI) by abnormal tilt table tests (88%) or gastric dysrhythmias (71%). After fludrocortisone treatment, there was >26% nausea improvement in 71%, 1-25% in 6%, and no improvement in 24%. In six subjects, EGGs repeated after >50% nausea improvement all remained to be abnormal, suggesting nausea is independent of gastric dysrhythmias., Conclusion: Association of EGG abnormalities and OI in this subset of nausea patients suggests a generalized disturbance of autonomic regulation.

Orthostatic intolerance (OI) refers to a group of clinical conditions, including postural orthostatic tachycardia syndrome (POTS) and neurally mediated hypotension (NMH), in which symptoms worsen with upright posture and are ameliorated by recumbence. The main symptoms of chronic orthostatic intolerance syndromes include light-headedness, syncope or near syncope, blurring of vision, headaches, problems with short-term memory and concentration, fatigue, intolerance of low impact exercise, palpitations, chest pain, diaphoresis, tremulousness, dyspnea or air hunger, nausea, and vomiting. This review discusses what is known about the pathophysiology of this disorder, potential treatments, and understanding its role in the patient with chronic headache pain., (Copyright 2010 Elsevier Inc. All rights reserved.)