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Neurogenic orthostatic hypotension (nOH) is a sustained reduction in blood pressure (BP) upon standing that is caused by autonomic dysfunction and is common among patients with a variety of neurodegenerative disorders (eg, Parkinson's disease, multiple system atrophy, pure autonomic failure). A systolic BP drop of ≥20 mmHg (or ≥10 mmHg diastolic) upon standing with little or no compensatory increase in heart rate is consistent with nOH. Symptoms of nOH include light-headedness, dizziness, presyncope, and syncope; these symptoms can severely impact patients' activities of daily living and increase the likelihood of potentially dangerous falls. Because of their patient contact, nurses and nurse practitioners can play a key role in identifying and evaluating patients at risk for nOH. It is advisable to screen for nOH in patients presenting with one or more of the following characteristics: those who have disorders associated with autonomic failure, those with episodes of falls or syncope, those with symptoms upon standing, those who are elderly or frail, or those taking multiple medications. Initial evaluations should include questions about postural symptoms and measurement of orthostatic BP and heart rate. A review of medications for potential agents that can have hypotensive effects should be performed before initiating treatment. Treatment for nOH may include non-pharmacologic measures and pharmacologic therapy. Droxidopa and midodrine are approved by the US Food and Drug Administration for the treatment of symptomatic nOH and symptomatic OH, respectively. nOH is associated with the coexistence of supine hypertension, and the two disorders must be carefully managed. In conclusion, timely screening and diagnosis of patients with nOH can streamline the path to disease management and treatment, potentially improving patient outcomes.

A growing body of evidence suggests that postural orthostatic tachycardia syndrome (POTS) may be an autoimmune disorder. We have reported in a previous manuscript that 89% of POTS patients (n = 55) had elevations in G-protein-coupled adrenergic A1 receptor autoantibodies and 53% had elevations in muscarinic acetylcholine M4 receptor autoantibodies, as assessed by ELISA. Patients with autoimmune disorders have been reported with a variety of elevated cytokines and cytokines (such as rheumatoid arthritis), thus, we evaluated a limited number of cytokines/chemokines in POTS patients with elevated adrenergic and muscarinic receptor autoantibodies. We utilized the plasma of 34 patients from a previous study, all of the patients (100%) had autoantibodies against the A1 adrenergic receptor and 55.9% (19/34) had autoantibodies against the M4 muscarinic acetylcholine receptor. In particular, the plasma cytokine/chemokine levels were measured as biomarkers of inflammation by Quantibody® technology (Raybiotech, Peachtree Corners, GA, USA). We also evaluated the platelet dense granule numbers, as these patients frequently complain of symptoms related to platelet dysfunction. Patients were predominantly young females who displayed a multitude of co-morbidities but generally reported viral-like symptoms preceding episodes of syncope. Eighty five percent (29/34) had platelet storage pool deficiency. Patients had elevations in five of ten cytokine/chemokines biomarkers (IL1β, IL21, TNFα, INFγ, and CD30), whereas two biomarkers had decreased levels (CD40L and RANTES). Our observations demonstrate that POTS patients known to have autoantibodies against the G-protein-coupled adrenergic A1 receptor have abnormal plasma concentrations of inflammatory cytokines.

BACKGROUND Ivabradine is a selective If channel blocker that reduces heart rate without affecting other cardiovascular functions. In case reports and case series, it was shown to improve symptoms in patients with postural tachycardia syndrome (POTS). METHODOLOGY AND RESULTS This retrospective study examined patients who were diagnosed with POTS and received ivabradine as part of their treatment. Forty-nine patients (47 females, 95.9%) received ivabradine. The average age was 35.1 ± 10.35 years. The most common symptoms were palpitations and lightheadedness and both improved significantly, 88.4% and 76.1% response rate, respectively. A total of 38 patients reported improvement in their symptoms. In addition, ivabradine resulted in an objective decrease in sitting and standing heart rate (78.1 ± 10.7 vs 72.5 ± 7.6, P-value: 0.01) and (107.4 ± 14.1 vs 95.1 ± 13.7, P-value

Background Postural orthostatic tachycardia syndrome (POTS) is a constellation of signs and symptoms that occur when a patient is upright and relieved by recumbence. Currently, no drugs are labeled for the treatment for POTS. Droxidopa is an orally administered amino acid that is converted to norepinephrine and thought to improve both blood pressure and symptoms in patients with orthostatic intolerance. Study question To appraise the effect of Droxidopa in a clinical setting in patients with POTS refractory to other forms of treatment. Study design A retrospective study of patients with POTS at our Syncope and Autonomic Disorders Center. Three hundred fifty-two patients were screened, 54 of them were prescribed Droxidopa and found to be eligible to include in our study. Measures and outcome Symptoms of orthostatic intolerance, side effects of therapy and response to treatment. Statistical analyses were done using SPSS software. Thirty-seven patients were included in data analysis. Patients who failed to follow up, didn't obtain Droxidopa due to insurance and cost concerns, had hypertensive response to therapy or had allergic reaction were excluded from data analysis. Results The most frequently reported symptom was dizziness in 91.9% of patients, followed by syncope and fatigue in 70.3% and 67.6% of patients, respectively. Symptoms of dizziness, syncope and fatigue were reported less after treatment; 75.7%, 51.4% and 40.5%, respectively. There was no statistically significant difference in standing or sitting blood pressure before and after treatment. Despite the improvement in some symptoms. Only 27% of patients reported improved quality of life after treatment. Of total, 40.5% of patients stopped the treatment either due to side effects or ineffectiveness. Conclusion Droxidopa appears to improve some symptoms of orthostatic intolerance in patients with POTS but has diminutive impact on quality of life and blood pressure. Further assessment in large clinical trials is needed to evaluate its efficacy.

Background The etiology of postural orthostatic tachycardia syndrome ( POTS ) is yet to be established. The disorder is often misdiagnosed as chronic anxiety or a panic disorder because the autonomic failure in these patients is not severe. A growing body of evidence suggests that POTS may be an autoimmune disorder. Antinuclear antibodies and elevations of ganglionic, adrenergic, and muscarinic acetylcholine receptor antibodies have all been reported. Methods and Results We collected detailed clinical symptoms of 55 patients diagnosed with POTS . We also evaluated serum levels of autoantibodies against 4 subtypes of G‐protein coupled adrenergic receptors and 5 subtypes of G‐protein coupled muscarinic acetylcholine receptors by ELISA . Our patients had a multitude of comorbidities, were predominantly young females, and reported viral‐like symptoms preceding episodes of syncope. We detected a significant number of patients with elevated levels of autoantibodies against the adrenergic alpha 1 receptor (89%) and against the muscarinic acetylcholine M 4 receptor (53%). Surprisingly, elevations of muscarinic receptor autoantibodies appeared to be dependent upon elevation of autoantibodies against the A 1 adrenergic receptor! Four patients had elevations of G‐protein coupled autoantibodies against all 9 receptor subtypes measured in our study. Five POTS patients had no elevation of any autoantibody; similarly, controls were also negative for autoantibody elevations. There was a weak correlation of clinical symptom severity with G‐protein coupled autoantibodies. Conclusions Our observations provide further evidence that, in most cases, POTS patients have at least 1 elevated G‐protein coupled adrenergic autoantibody and, in some instances, both adrenergic and muscarinic autoantibodies, supporting the hypothesis that POTS may be an autoimmune disorder.

Neurogenic orthostatic hypotension (nOH) is common in patients with neurodegenerative disorders such as Parkinson’s disease, multiple system atrophy, pure autonomic failure, dementia with Lewy bodies, and peripheral neuropathies including amyloid or diabetic neuropathy. Due to the frequency of nOH in the aging population, clinicians need to be well informed about its diagnosis and management. To date, studies of nOH have used different outcome measures and various methods of diagnosis, thereby preventing the generation of evidence-based guidelines to direct clinicians towards ‘best practices’ when treating patients with nOH and associated supine hypertension. To address these issues, the American Autonomic Society and the National Parkinson Foundation initiated a project to develop a statement of recommendations beginning with a consensus panel meeting in Boston on November 7, 2015, with continued communications and contributions to the recommendations through October of 2016. This paper summarizes the panel members’ discussions held during the initial meeting along with continued deliberations among the panel members and provides essential recommendations based upon best available evidence as well as expert opinion for the (1) screening, (2) diagnosis, (3) treatment of nOH, and (4) diagnosis and treatment of associated supine hypertension. Electronic supplementary material The online version of this article (doi:10.1007/s00415-016-8375-x) contains supplementary material, which is available to authorized users.

Ivabradine is a selective IThis retrospective study examined patients who were diagnosed with POTS and received ivabradine as part of their treatment. Forty-nine patients (47 females, 95.9%) received ivabradine. The average age was 35.1 ± 10.35 years. The most common symptoms were palpitations and lightheadedness and both improved significantly, 88.4% and 76.1% response rate, respectively. A total of 38 patients reported improvement in their symptoms. In addition, ivabradine resulted in an objective decrease in sitting and standing heart rate (78.1 ± 10.7 vs 72.5 ± 7.6, P-value: 0.01) and (107.4 ± 14.1 vs 95.1 ± 13.7, P-value: 0.001), respectively, with no significant change in blood pressure. The most common reported side effect was luminous phenomena/visual brightness occurring in nine patients. However, none of the patients stopped ivabradine due to side effects.Our study shows that ivabradine is likely to be effective in treating patients with POTS. Nearly 78% of our cohort reported a significant improvement in symptoms with no major adverse effects reported. A future randomized, placebo-controlled trial is warranted.

The postural tachycardia syndrome (POTS) is a heterogeneous group of disorders that results in symptoms of orthostatic intolerance. Excess blood pooling has been observed to cause low effective circulating volume in the central vasculature. Consequently, acute volume loading with IV saline has emerged as a potential strategy for clinical intervention. We evaluated the impact of acute volume loading on both the signs and symptoms of patients suffering from POTS. Fifty-seven subjects screened from our population of POTS patients and assenting to participation were administered the two surveys by telephone. Subjects completed each survey twice, before, and after initiating IV hydration therapy. The Orthostatic Hypotension Questionnaire (OHQ) was used to assess change in clinical symptomatology, while the short form 36 health survey (SF-36) was employed to assess the impact of IV saline infusion on quality of life. Fifty-seven patients were included in the analysis. The average number of medications trialed before referral for IV hydration was 3.6 ± 1.7 medications. Saline infusions occurred with mean frequency of 11.3 ± 8.5 days and at a mean volume of 1.5 ± 0.6 l per infusion. The mean change of the OHQ was 3.1 ± 0.3 (95% CI 2.6–3.7; P

Mechanisms have been postulated to explain postural orthostatic tachycardia syndrome (POTS), however, the etiology of this often debilitating disorder remains unknown. We conducted a retrospective case–control study of 181 POTS patients who exhibited/reported bleeding symptoms for a specific platelet (PL) dysfunction disorder, delta granule storage pool deficiency (δ-SPD). Patients were included only if results of blood tests for δ-SPD were available. Electron microscopy was utilized to diagnose δ-SPD. An ELISA assay was used to determine serotonin (5HT) concentration in PLs and medical record review was employed to collect patients’ clinical symptoms. The most common bleeding symptom was easy bruising (71%) but frequent nose bleeds, heavy menstrual bleeding, and a family history of bleeding were also commonly reported. Of the patients studied, 81% were diagnosed with δ-SPD. Our investigation of 5HT concentration extracted from PLs revealed significantly lower levels of 5HT in POTS patients when compared to that of control subjects. Our data suggest that patients with POTS have significant comorbidities including bleeding symptoms and/or family bleeding histories, and have diminished PL 5HT levels supporting the hypothesis that POTS is a low 5HT level disorder. While we describe a significant relationship with POTS and δ-SPD, this finding does not constitute an etiology for POTS. Our results establish an additional comorbidity frequently seen in POTS that could explain a number of disparate symptoms often affecting the severity of POTS.

Syncope is a common clinical problem accounting for 3% of all emergency room visits and 1% to 6% of all hospital admissions. Both a sign and a symptom, syncope can be caused by a wide variety of conditions. Syncope in the geriatric patient can be a particularly challenging problem because of the coexistence of multiple possible causative pathologic conditions in the same individual. This article reviews the causes, evaluation, and management of syncope in the elderly.

Infection is a well-recognized complication that can occur after the implantation of cardiac devices such as pacemakers and implantable cardioverter defibrillators (ICDs). Reported infection rates after new device implantation are reported to be around 1%, while infection rates after device generator replacements are higher with a reported average of up to 4-5% per year. Here we report our experience using a modified plastic surgical technique for cardiac device wound closure designed to both reduce infections and enhance cosmetic outcomes. Patients were recruited from among those individuals undergoing routine cardiac device implantation (either new or replacement) at our institution. A total of 124 patients were included in the study. There were 74 women and 48 men, mean age 58 ± 16 years. There were 74 new pacemaker implants and 27 pacemaker generator replacements. There were 17 new ICD generator implants and 6 ICD generator reimplants. Mean follow-up time was 15 ± 16 months. During the follow-up period, there have been no device infections nor any wound dehiscences observed. Each patient felt that the scar was cosmetically acceptable. Two patients developed mild rashes to the clear plastic adhesive that resolved after removal. The modified wound closure technique described above appears to minimize cardiac device wound infections while facilitating cosmetically acceptable wound scar formation.

There have been reports on the use of octreotide in patients with orthostatic hypotension, postural tachycardia syndrome, and orthostatic syncope. However, there are little if any data on the use of octreotide in patients who have failed multiple other medications. This study was a retrospective chart analysis and was approved by our Institutional Review Board. A total of 12 patients were identified for inclusion in this study. The diagnosis of orthostatic intolerance was based on patient history, physical examination, and response to Head Up Tilt Table testing. These patients had failed multiple medications and were ultimately treated with octreotide. In a retrospective chart review, we collected data, including demographic information, presenting symptoms, laboratory data, tilt-table response, standing heart rate, standing blood pressure before and after treatment (wherever available), and treatment outcomes. Twelve patients aged 33 6 18 years, eight (66.7%) females, were found to have symptoms of refractory orthostatic intolerance and failed multiple regimens of medication and were ultimately treated with octreotide administration. Five patients (41.7%) had demonstrated a postural tachycardia syndrome pattern, five (41.7%) a neurocardiogenic, and two (16.6%) a dysautonomic response on a Head Up Tilt Table. Symptoms of syncope and orthostatic palpitations improved in six (50%) of the patients. Standing heart rate was significantly reduced after octreotide administration (80 6 8 versus 108 6 13; P , 0.05). The standing systolic blood pressure was increased after octreotide administration (107 6 26 versus 116 6 22). Three patients (25%) reported complete elimination of syncope, whereas another three had reduction in the frequency of their syncope. However, symptoms of fatigue improved only in two (29%) of the seven patients. Octreotide may improve symptoms in some patients with refractory orthostatic intolerance.

Methylphenidate has been shown to be an effective therapy in patients with refractory neurocardiogenic syncope. However, the role of methylphenidate in patients suffering from postural orthostatic tachycardia (POTS) has not been reported. The study was approved by the institutional review board. A retrospective nonrandomized analysis was preformed on 24 patients evaluated at our autonomic center for POTS from 2003 to 2010. The diagnosis of POTS was based on patient history, physical examination, and response to head up tilt table testing. The mean follow-up period was 9 ± 3 months. The patients were included in the current study if they had a diagnosis of POTS with severe symptoms of orthostatic intolerance and were refractory to the commonly used medications. All of these patients were started on methylphenidate and the response to therapy was considered successful if it provided symptomatic relief. Twenty-four patients (age 28 ± 12, 20 women) met inclusion criterion for this study. The response to treatment was assed subjectively in each patient and was collected in a retrospective fashion from patient charts and physician communications. Four patients reported side effects in the form of nausea and 2 ultimately had to discontinue the treatment. Another 4 patients had a follow-up of less than 6 months. Thus, only 18 patients who received methylphenidate completed the follow-up of 6 months. Out of these 18 patients, 14 (77%) patients reported marked improvement in their symptoms. Nine out of 12 patients who had recurrent episodes of syncope reported no syncope at 6 months of follow-up. Fourteen (77%) patients reported marked improvement in their symptoms of fatigue and presyncope. Four patients continue to have symptoms of orthostatic intolerance and 3 continued to have recurrent episodes of syncope. Methylphenidate may be beneficial in patients with otherwise refractory postural tachycardia syndrome.

Many patients who suffer from orthostatic intolerance (OI) may also have severe fatigue and extreme exercise intolerance. In some of these patients, fatigue may be so severe that they are unable to maintain employment. In some, even the activities of the daily living may be compromised. We report on the use of modafinil in a subgroup of patients who failed therapy with commonly used medication for fatigue in patients with OI. The study was approved by the institutional review board. A retrospective nonrandomized analysis was preformed on 60 patients evaluated at our autonomic center for OI from 2003 to 2010. The diagnosis of OI was based on patient history, physical examination, and reponse to head up tilt table testing. All these patients had fatigue as their predominant symptom. Multiple trials of stimulants including methylphenidate, amphetamine, or dextroamphetamine failed to provide symptomatic relief of fatigue in these patients. Each patient received modafinil (100-200 mg daily). The mean follow-up period was 9 ± 3 months. A treatment was considered successful if it provided symptomatic relief from fatigue for the patient. Sixty patients, age 29 ± 15, 52 women were included in the analysis. Migraine (57%) and joint hypermobility syndrome (33%) were common comorbidities. Out of 60 patients, 40 patients reported initial improvement with initiation of modafinil therapy. Twenty patients reported no change in their symptoms of fatigue. Of the 40 patients who showed initial improvement in their symptoms 4 had eventual recurrence of fatigue after 3 months of modafinil therapy. Thirty-six patients continued to demonstrate symptom relief from fatigue for more than 6 months. In a selective group of patients of OI, modafinil may improve fatigue.

Background: The long-term efficacy of pyridostigmine, a reversible acetyl cholinesterase inhibitor, in the treatment of postural orthostatic tachycardia syndrome (POTS) patients remains unclear. We report our retrospective, single-center, long-term experience regarding the efficacy and adverse effect profile of pyridostigmine in the treatment of POTS patients. Methods: This retrospective study included an extensive review of electronic charts and data collection in regards to patient demographics, orthostatic parameters, side-effect profile, subjective response to therapy, as well as laboratory studies recorded at each follow-up visit to our institution’s Syncope and Autonomic Disorders Center. The response to pyridostigmine therapy was considered successful if patient had both symptom relief in addition to an objective response in orthostatic hemodynamic parameters (heart rate [HR] and blood pressure). Three hundred patients with POTS were screened for evaluation in this study. Of these 300, 203 patients with POTS who received pyridostigmine therapy were reviewed. Of these 203 patients, 168 were able to tolerate the medication after careful dose titration. The mean followup duration in this group of patients was 12 ± 3 (9‐15) months. Pyridostigmine improved symptoms of orthostatic intolerance in 88 of 203 (43%) of total patients or 88 of 172 (51%) who were able to tolerate the drug. The symptoms that improved the most included fatigue (55%), palpitations (60%), presyncope (60%), and syncope (48%). Symptom reduction correlated with a statistically significant improvement in upright HR and diastolic blood pressure after treatment with pyridostigmine as compared to their baseline hemodynamic parameters (standing HR 94 ± 19 vs 82 ± 16, P < 0.003, standing diastolic blood pressure 71 ± 11 vs 74 ± 12, P < 0.02). Gastrointestinal problems were the most common adverse effects (n = 39, 19%) reported. The overall efficacy of pyridostigmine in our study was seen in 42% of total patients or 52% of patients who could tolerate taking the drug. Conclusion: The subgroup of POTS patients who can tolerate oral pyridostigmine may demonstrate improvement in their standing HR, standing diastolic blood pressure, and clinical symptoms of orthostatic intolerance. (PACE 2011; 1‐6) pyridostigmine, orthostatic intolerance, postural orthostatic tachycardia syndrome

Introduction: There is anecdotal evidence that one or more forms of orthostatic intolerance (OI) subgroups may coexist in the same patients. However, there is a paucity of published data on the clinical featuresandmanagementofpatientswhosufferfromcoexistingfeaturesofposturaltachycardiasyndrome (POTS) and neurocardiogenic syncope (NCS). We herein present our experience of 18 patients who we found displayed evidence of coexisting NCS and POTS. Methods: We reviewed charts of 300 POTS patients seen at the University of Toledo Syncope and Autonomic Disorders Center from 2003 to 2010 and found 18 patients eligible for inclusion in this study. Patients were included in this study if they reported clinical symptoms consistent with both POTS and NCS and then demonstrated a typical POTS pattern (a rise in heart rate without change in blood pressure [BP]) on head up tilt table (HUTT) within the first 10 minutes of upright posture followed by a neurocardiogenic pattern (a sudden fall in heart rate and/or fall in blood pressure) reproducing symptoms that were similar to the patients spontaneous episodes. Results: We found 18 patients, mean age (30 ± 12), with 15 (84%) women and three (16%) men, who met the inclusion criterion for this study. Each of these 18 patients demonstrated a typical POTS pattern within the first 10 minutes on initial physical exam and on a HUTT. Continued tilting beyond 10 minutes resulted in a sudden decline in heart rate (which in some patients manifested as an asystole that lasted anywhere between 10 and 32 seconds [mean of 18 seconds]) and/or a fall in BP in each of these patients demonstrating apatternconsistent withneurocardiogenic subtypeofOI.Themean timetotheNCSpattern of a fall in BP and heart was 15 minutes with a range of 13‐20 minutes. This group of patients was highly symptomatic and reported frequent clinical symptoms that were suggestive of OI. Recurrent presyncope, syncope, orthostatic palpitations, exercise intolerance, and fatigue were the principal symptoms reported. Conclusion: NCS may coexist with POTS in a subgroup of patients suffering from OI. (PACE 2010; 1‐6)

Background Disturbances in autonomic nervous system function have been reported to occur in patients suffering from mitochondrial cytopathies. However, there is paucity of literature on the occurrence of orthostatic intolerance (OI) in these patients. We report on a series of patients diagnosed with mitochondrial cytopathy who developed features of autonomic dysfunction in the form of OI. Methods This was a single-center report on a series of 6 patients who were followed in our clinic for orthostatic intolerance. All of these patients had a diagnosis of mitochondrial cytopathy on the basis of muscle biopsy and were being followed at a center specializing in the treatment of mitochondrial disorders. This study was approved by our local institutional review board. Each of the patients had suffered from symptoms of fatigue, palpitations, near syncope, and syncope. The diagnosis of OI was confirmed by head-up tilt test. Collected data included demographic information, presenting symptoms, laboratory data, tilt-table response, and treatment outcomes. Results Six patients (3 females) were identified for inclusion in this report. The mean age of the group was 48 ± 8 years (range, 40–60 years). All of these patients underwent head-up tilt table testing and all had a positive response that reproduced their clinical symptoms. Among those having an abnormal tilt-table pattern, 1 had a neurocardiogenic response, 1 had a dysautonomic response, and 4 had a postural orthostatic tachycardia response. All but 1 patient reported marked symptom relief with pharmacotherapy. The patient who failed pharmacotherapy received a dual-chamber closed-loop pacemaker and subsequently reported marked improvement in her symptoms with elimination of her syncope. Conclusions Orthostatic intolerance might be a significant feature of autonomic nervous system dysfunction in patients suffering from mitochondrial cytopathy. Copyright © 2010 Wiley Periodicals, Inc. The authors have no funding, financial relationships, or conflicts of interest to disclose.

Introduction Placement of the Reveal implantable loop recorder (ILR; Medtronic Inc., Minneapolis, MN, USA) has previously involved preoperative cutaneous mapping to determine the optimal location. We describe an anatomic-based approach to ILR placement that does not require cutaneous mapping. Method A total of 63 patients (40 women, 23 men, mean age 38 ± 15 years) were included in the study. Each underwent implantation of a Reveal ILR in the left upper chest area midway between the supraclavicular notch and the left breast area. Thirty-two patients received a Medtronic Reveal DX ILR and 31 received Reveal XT device. Results In all 62 patients, adequate electrocardiographic tracings were obtained at implant without the need for preoperative cutaneous mapping, and all were followed for a period of 10 ± 4 months afterwards. The mean P wave amplitude was 0.12 ± 0.20 mV at implant and at follow-up (6-14 months postimplant); the amplitude was 0.11 ± 0.19 mV. The peak-to-peak QRS amplitude was 0.48 ± 0.15 mV at implant and 0.44 ± 0.16 mV at a follow-up of 6-14 months. The P waves were not detected in two patients at follow-up. In one patient, decreased amplitude of QRS complex resulted in the autoactivation of the device and in one other patient noise was inappropriately oversensed and recorded. Conclusion A simple anatomic approach can be used for reveal ILR placement.

Background Autonomic dysfunction presenting as inappropriate sinus tachycardia has been reported to occur following slow pathway ablation for atrioventricular node tachycardia. We report on a series of patients who developed new onset postural orthostatic tachycardia syndrome (POTS) following successful radiofrequency ablation of atrioventricular nodal reentrant tachycardia (AVNRT). Methods The study was a retrospective analysis that was approved by our Institutional Review Board. Patients were identified from those seen at our Syncope and Autonomic Disorders Clinic. A total of six patients were identified who were previously healthy except for supraventricular tachycardia. Each was found to have AVNRT during electrophysiology study and each underwent successful radiofrequency modification of the slow atrioventricular nodal pathway. Following ablation each patient developed the new onset of symptoms of orthostatic intolerance consistent with POTS. Results After aninitial symptom-free period(3‐6 weeks)post ablation each patient began to experience symptoms of orthostatic intolerance. All six patients began to experience progressive severe fatigue. Orthostatic tachycardia was reported by five patients, syncope by three patients, and presyncope by all six patients. Each patient reported the occurrence of symptom while upright that were relieved by becoming supine. Each patient had experienced symptoms for greater than 6 months prior to being seen at our center. Three patients reported such severe symptoms of orthostatic tachycardia that they underwent repeat electrophysiology study; however, none had evidence of AVNRT. Each patient demonstrated a POTS response within the first 10 min of upright tilt with reproduction of their clinical symptoms that had occurred post ablation. Conclusion POTS may be a complication of radiofrequency ablation of AVNRT.

Background: Autonomic dysfunction is common in patients with the joint hypermobility syndrome (JHS). However, there is a paucity of reported data on clinical features of Postural orthostatic tachycardia syndrome (POTS) in patients suffering from JHS.Methods: This retrospective study was approved by our local Institutional Review Board (IRB). Over a period of 10 years, 26 patients of POTS were identified for inclusion in this study. All these patients had features of Joint Hypermobility Syndrome (by Brighton criterion). A comparison group of 39 patients with other forms of POTS were also followed in the autonomic clinic during the same time. We present a descriptive report on the comparative clinical profile of the clinical features of Postural Orthostatic Tachycardia patients with and without Joint Hypermobility syndrome. The data is presented as a mean±SD and percentages wherever applicable.Results: Out of 65 patients, 26 patients (all females, 20 Caucasians) had POTS and JHS. The mean age at presentation of POTS was 24±13 (range 10-53 years) vs 41±12 (range 19-65 years), P=0.0001, Migraine was a common co morbidity 73 vs 29% p=0,001. In two patients POTS was precipitated by pregnancy, and in three by surgery, urinary tract infection and a viral syndrome respectively. The common clinical features were fatigue (58%), orthostatic palpitations (54%), presyncope (58%), and syncope (62%).Conclusion: Patients with POTS and JHS appear to become symptomatic at an earlier age compared to POTS patients without JHS. In addition patients with JHS had a greater incidence of migraine and syncope than their non JHS counterparts.

Background: Autonomic dysfunction is common in patients suffering from multiple sclerosis (MS) and orthostatic dizziness occurs in almost 50% of these patients. However, there have been no reports on postural orthostatic tachycardia syndrome (POTS) in patients suffering from MS. Methods: The patients were included for analysis in this study if they had POTS with either a prior history of MS or having developed MS while being followed for POTS. Postural orthostatic tachycardia (POTS) is defined as symptoms of orthostatic intolerance(>6months) accompanied by a heart rate increase of at least 30 beats/min (or a rate that exceeds 120 beats/min) that occurs in the first 10 minutes of upright posture or head up tilt test (HUTT) occurring in the absence of other chronic debilitating disorders. We identified nine patients with POTS who were suffering from MS as well. Each of these patients had been referred from various other centers for second opinions. Results: The mean age at the time of diagnosis of POTS was 49±9 years and eight of the 9 patients were women. Five patients (55%) had hyperlipidemia, 3 (33%) migraine and 2 (22%) patients had coronary artery disease and diabetes each. Fatigue and palpitations (on assuming upright posture) were the most common finding in our patients (9/9). All patients also had orthostatic dizziness. Syncope was seen in 5/9(55%) of patients. Four patients (44%), who did not have clear syncope, were having episodes of near syncope. The presence of POTS in our study population resulted in substantial limitation of daily activities. Following recognition and treatment of POTS, 6/9(66%), patients were able to resume daily activities of living. Their symptoms (especially fatigue and orthostatic intolerance) improved. The frequency and severity of syncope also improved. Three (33%) patients failed to show a good response to treatment. Conclusion: Patients suffering from MS may manifest autonomic dysfunction by developing POTS. Early recognition and proper management may help improve the symptoms of POTS.

We present herein an interesting tracing of a patient who suffered from recurrent episodes of transient loss of consciousness (TLOC) associated with convulsive activity thought to be due to epilepsy or conversion disorder.

Background: Autonomic dysfunction is common in patients suffering from multiple sclerosis (MS) and orthostatic dizziness occurs in almost 50% of these patients. However, there have been no reports on postural orthostatic tachycardia syndrome (POTS) in patients suffering from MS. Methods: The patients were included for analysis in this study if they had POTS with either a prior history of MS or having developed MS while being followed for POTS. Postural orthostatic tachycardia (POTS) is defined as symptoms of orthostatic intolerance(>6months) accompanied by a heart rate increase of at least 30 beats/min (or a rate that exceeds 120 beats/min) that occurs in the first 10 minutes of upright posture or head up tilt test (HUTT) occurring in the absence of other chronic debilitating disorders. We identified nine patients with POTS who were suffering from MS as well. Each of these patients had been referred from various other centers for second opinions. Results: The mean age at the time of diagnosis of POTS was 49±9 years and eight of the 9 patients were women. Five patients (55%) had hyperlipidemia, 3 (33%) migraine and 2 (22%) patients had coronary artery disease and diabetes each. Fatigue and palpitations (on assuming upright posture) were the most common finding in our patients (9/9). All patients also had orthostatic dizziness. Syncope was seen in 5/9(55%) of patients. Four patients (44%), who did not have clear syncope, were having episodes of near syncope. The presence of POTS in our study population resulted in substantial limitation of daily activities. Following recognition and treatment of POTS, 6/9(66%), patients were able to resume daily activities of living. Their symptoms (especially fatigue and orthostatic intolerance) improved. The frequency and severity of syncope also improved. Three (33%) patients failed to show a good response to treatment. Conclusion: Patients suffering from MS may manifest autonomic dysfunction by developing POTS. Early recognition and proper management may help improve the symptoms of POTS.

In many patients with recurrent neurocardiogenic syncope (NCS), a significant fall in blood pressure precedes any appreciable decline in heart rate. Closed-loop pacing (CLS) employs a sensing system that measures myocardial contractility, thereby providing a potential way to detect the onset of NCS at a much earlier point in time than that provided by standard pacing systems.Patients were included in the study if they suffered from recurrent NCS and met all of the following criteria: (1) They had suffered at least two syncopal episodes in the preceding 6 months. (2) Patients were refractory to (or intolerant of) all conventional, non-pharmacological, or pharmacological treatments. (3) They had evidence of asystole (10 s) or severe bradycardia (heart rate30 bpm) on implantable loop recorder or during head-up tilt test (HUTT).Thirty five patients meeting the above criterion received 44 devices. Twelve patients received a standard unit (with rate drop or rate hysterisis response) and 32 patients received a CLS unit (Cylos, Biotronik). The pacemaker implantation was termed successful if there was no recurrence of syncope, if the syncope burden decreased byor =50%, if only presyncope occurred, or if the syncope occurred but with significant warning symptoms. Thirty-five patients, 29 females and six males, age 41 +/- 11, with refractory NCS underwent pacemaker implantation. Mean follow-up was 9 +/- 3 months. Out of 32 patients who received CLS, nine had a conventional pacemaker implanted in the past. Recurrence (59% vs 83%), reduction in syncope burden and pacemaker success (84% vs 25%, P = 0.002), and occurrence prodrome/warning signs (40% vs 16%) were much better in the closed-loop group.These preliminary observations suggest that dual-chamber CLS pacing may be promising therapy for refractory NCS. Further randomized trials will be needed to better determine the role of this therapy in refractory NCS.

Background: Postural orthostatic tachycardia syndrome (POTS) occurs more commonly in women than in men and often affects women of childbearing age. Many of these women wish to have children, yet there are little reported data on the outcomes of pregnancy in patients with POTS. To date there has been one report of two patients with POTS who successfully completed pregnancy. We report the outcomes of 22 women with preexisting POTS who became pregnant. Objective: To assess the outcome of pregnancy in patients with preexisting POTS. Methods and Results:Twenty-two patients, age 30 ±7 years, with POTS became pregnant. Migraine was the common comorbidity found in 40% of patients. Medications used were β-blockers (18%), midiodrine (31%), selective serotonin reuptake inhibitors (31%), fludrocortisone (13%), combination (40%), and none (18%). During pregnancy, symptoms of POTS remained unchanged in three (13%), improved in 12 (55%), and worsened in seven (31%) patients. One patient who had recurrent episodes of syncope without aura was found to have complete heart block and received a cardiac pacemaker. All patients completed pregnancy successfully. There were no stillbirths. One patient developed hyperemesis. Eighteen patients had vaginal delivery and four patients delivered by cesarian section. No other complications of pregnancy were encountered. Congenital abnormalities were encountered in the form of one atrial septal defect, one ventricular septal defect, and one Down’s syndrome. Postpartum symptoms of POTS remained stable in 15 (69%) patients and worsened in seven (31%) patients. Conclusion: Based on our observation, patients with POTS can safely complete pregnancy if they desire to do so. POTS should not be considered a contraindication to pregnancy per se. (PACE 2009; 32:1000‐1003)

Introduction: Not all convulsive episodes are due to epilepsy and a number of these have a cardiovascular cause. Failure to identify these patients delays the provision of adequate therapy while at the same time exposes the individual to the risk of injury or death. Methods: We report on three patients who suffered from recurrent convulsive episodes, thought to be epileptic in origin, who were refractory to antiseizure therapy. Although each patient had undergone extensive evaluation, no other potential cause of his or her seizure like episodes had been uncovered. In each patient placement of an implantable loop recorder (ILR) demonstrated that their convulsive episodes were due to prolonged periods of cardiac asystole and/or complete heart block. In all patients their convulsive episodes were eliminated by permanent pacemaker implantation. Conclusion: In patients with refractory “seizure' like episodes of convulsive activity of unknown etiology a potential cardiac rhythm disturbance should be considered and can be easily evaluated by ILR placement.

Background: Implantable loop recorders (ILR) have been found to be useful in the diagnosis and management of syncope of unclear etiology. The clinical symptoms of abnormalities seen during ILR monitoring have not been adequately studied. Aim: The aim of this retrospective study was to determine the clinical symptoms which were the best predictors of asystolic or bradycardic responses during ILR monitoring. Methods: Patients with either asystole or bradycardia recorder during ILR monitoring were analyzed from our database. The clinical characteristics of these patients were compared to the patients with ILR's who did not have recorded bradycardic episodes. The episodes were characterized as being convulsive or nonconvulsive, brief ( 5 minutes), and having had a prodrome or no prodrome. Results: Eleven patients (4 males and 7 females; age 39 ±11years) had asystole or bradycardia on ILR monitoring. Eleven patients (2 males and 9 females; age 46±23) had no bradycardiac events. Palpitations, convulsive syncope, prolonged episode, and prodrome were present in 37% vs. 74% (P = 0.125), 62% vs. 0% (P = 0.002), 87% vs. 0% (P=0), and 73% vs. 13% (P=0.009) patients, respectively, in the asystole/bradycardia and non-bradycardia groups. In the asystole/bradycardia group, four patients had complete atrioventricular block and six had long sinus pauses. One patient in the non-bradycardia group had ventricular tachycardia and atrial fibrillation each and two patients had supraventricular tachycardia. Conclusion: Convulsive syncope, prolonged loss of consciousness during syncopal episode, and absence of prodrome or aura are clinical predictors of asystole or bradycardia on ILR monitoring.

Introduction: Not all convulsive episodes are due to epilepsy and a number of these have a cardiovascular cause. Failure to identify these patients delays the provision of adequate therapy while at the same time exposes the individual to the risk of injury or death. Methods: We report on three patients who suffered from recurrent convulsive episodes, thought to be epileptic in origin, who were refractory to antiseizure therapy. Although each patient had undergone extensive evaluation, no other potential cause of his or her seizure like episodes had been uncovered. In each patient placement of an implantable loop recorder (ILR) demonstrated that their convulsive episodes were due to prolonged periods of cardiac asystole and/or complete heart block. In all patients their convulsive episodes were elimi- nated by permanent pacemaker implantation. Conclusion: In patients with refractory "seizure' like episodes of convulsive activity of un- known etiology a potential cardiac rhythm disturbance should be considered and can be easily evaluated by ILR placement.

We report on two previously healthy patients who developed severe form of postural orthostatic tachycardia syndrome (POTS) following an electric injury. Both the patients developed symptoms of orthostatic intolerance in the form of dizziness, fatigue, lightheadedness, and palpitations, weeks to months after electrical injury. Orthostatic intolerance produced considerable functional impairment in these patients. Early recognition of POTS when it occurs after an electrical injury allows for prompt evaluation and management to occur. (PACE 2010; 33:e59–e61)

Syncope is a common clinical problem accounting for 3% of all emergency room visits and 1% to 6% of all hospital admissions. Both a sign and a symptom, syncope can be caused by a wide variety of conditions. Syncope in the geriatric patient can be a particularly challenging problem because of the coexistence of multiple possible causative pathologic conditions in the same individual. This article reviews the causes, evaluation, and management of syncope in the elderly.

The evolution of upright posture is usually considered an advantage in humans. For people with postural tachycardia syndrome (PoTS) it can present a daily challenge. Although orthostatic intolerance is often associated with older people, PoTS tends to affect young women who present with multiple, non-specific symptoms and significant functional impairment.1 PoTS was characterised in 1993,2 but previously existed under various names including irritable heart, soldier's heart, and idiopathic orthostatic intolerance. It is a heterogeneous group of disorders sharing similar characteristics as a consequence of abnormal autonomic nervous system response to assuming upright posture. When humans stand up, approximately 500 ml of blood descends from the thorax into the abdominal cavity and limbs. A normal autonomic nervous system responds with immediate peripheral vasoconstriction, increase in heart rate of 10–20 beats per minute (bpm), and minimal change in blood pressure.1 In patients with PoTS this mechanism does not respond appropriately; the exact pathophysiology remains unclear.3 A likely mechanism is inadequate vasoconstriction on standing, resulting in pooling of blood in splanchnic and peripheral vasculature. Heart rate and catecholamine levels increase further to compensate. Symptoms of cerebral hypoperfusion including dizziness and syncope occur, often in the presence of normal blood pressure. Ironically, some patients with PoTS have a hypertensive response to standing.1 Diagnosis is usually made following a tilt table test or 10-minute stand test. The definition is arbitrary, but agreed by consensus (Box 1).4 Heart rate increases but, by definition, blood pressure does not necessarily drop. However, there is overlap with neurally mediated hypotension and some …

Background: We present our single center experience of 27 patients of hyperadrenergic postural orthostatic tachycardia syndrome (POTS). Methods: In a retrospective analysis, we reviewed the charts of 300 POTS patients being followed at our autonomic center from 2003 to 2010, and found 27 patients eligible for inclusion in this study. POTS was defined as symptoms of orthostatic intolerance (of greater than six monthsi duration) accompanied by a heart rate increase of at least 30 bpm (or a rate that exceeds 120 bpm) that occurs in the first 10 min of upright posture or head up tilt test (HUTT) occurring in the absence of other chronic debilitating disorders. Patients were diagnosed as having the hyperadrenergic form based on an increase in their systolic blood pressure of ‡ 10 mm Hg during the HUTT (2) with concomitant tachycardia or their serum catecholamine levels (serum norepinephnrine level ‡ 600 pg/mL) upon standing. Results: Twenty seven patients, aged 39 ‐ 11 years, 24, (89%) of them female and 22 (82%) Caucasian were included in this study. Most of these patients were refractory to most of the first and second line treatments, and all were on multiple combinations of medications. Conclusions: Hyperadrenergic POTS should be identified and differentiated from neuropathic POTS. These patients are usually difficult to treat and there are no standardized treatment protocols known at this time for patients with hyperadrenergic POTS. (Cardiol J 2011; 18, x: xxnxx)

It is our belief that a well-designed cardiac device management program should include end-of-life patient and family planning, addressing potential decisions regarding withdrawal of pacemaker and/or implantable cardioverter defibrillator therapy. Guided by the basic ethical and legal principles outlined in the article, it is the responsibility of the electrophysiologist and other involved health care providers to introduce this topic to patients, provide guidance and resources for decision making, and honor both patient and family requests.

The long-term efficacy of pyridostigmine, a reversible acetyl cholinesterase inhibitor, in the treatment of postural orthostatic tachycardia syndrome (POTS) patients remains unclear. We report our retrospective, single-center, long-term experience regarding the efficacy and adverse effect profile of pyridostigmine in the treatment of POTS patients.This retrospective study included an extensive review of electronic charts and data collection in regards to patient demographics, orthostatic parameters, side-effect profile, subjective response to therapy, as well as laboratory studies recorded at each follow-up visit to our institution's Syncope and Autonomic Disorders Center. The response to pyridostigmine therapy was considered successful if patient had both symptom relief in addition to an objective response in orthostatic hemodynamic parameters (heart rate [HR] and blood pressure). Three hundred patients with POTS were screened for evaluation in this study. Of these 300, 203 patients with POTS who received pyridostigmine therapy were reviewed. Of these 203 patients, 168 were able to tolerate the medication after careful dose titration. The mean follow-up duration in this group of patients was 12 ± 3 (9-15) months. Pyridostigmine improved symptoms of orthostatic intolerance in 88 of 203 (43%) of total patients or 88 of 172 (51%) who were able to tolerate the drug. The symptoms that improved the most included fatigue (55%), palpitations (60%), presyncope (60%), and syncope (48%). Symptom reduction correlated with a statistically significant improvement in upright HR and diastolic blood pressure after treatment with pyridostigmine as compared to their baseline hemodynamic parameters (standing HR 94 ± 19 vs 82 ± 16, P0.003, standing diastolic blood pressure 71 ± 11 vs 74 ± 12, P0.02). Gastrointestinal problems were the most common adverse effects (n = 39, 19%) reported. The overall efficacy of pyridostigmine in our study was seen in 42% of total patients or 52% of patients who could tolerate taking the drug.The subgroup of POTS patients who can tolerate oral pyridostigmine may demonstrate improvement in their standing HR, standing diastolic blood pressure, and clinical symptoms of orthostatic intolerance.

A subgroup of patients suffering from Lyme disease (LD) may initially respond to antibiotics only to later develop a syndrome of fatigue, joint pain and cognitive dysfunction referred to as 'post treatment LD syndrome'. We report on a series of patients who developed autonomic dysfunction in the form of postural orthostatic tachycardia syndrome (POTS).All of the patients in this report had suffered from LD in the past and were successfully treated with antibiotics. All patients were apparently well, until years later when they presented with fatigue, cognitive dysfunction and orthostatic intolerance. These patients were diagnosed with POTS on the basis of clinical features and results of the tilt table (HUTT) testing.Five patients (all women), aged 22-44 years, were identified for inclusion in this study. These patients developed symptoms of fatigue, cognitive dysfunction, orthostatic palpitations and either near syncope or frank syncope. The debilitating nature of these symptoms had resulted in lost of the employment or inability to attend school. Three patients were also suffering from migraine, two from anxiety and depression and one from hypertension. All patients demonstrated a good response to the employed treatment. Four of the five were able to engage in their activities of daily living and either resumed employment or returned to school.In an appropriate clinical setting, evaluation for POTS in patients suffering from post LD syndrome may lead to early recognition and treatment, with subsequent improvement in symptoms of orthostatic intolerance.

There is anecdotal evidence that one or more forms of orthostatic intolerance (OI) subgroups may coexist in the same patients. However, there is a paucity of published data on the clinical features and management of patients who suffer from coexisting features of postural tachycardia syndrome (POTS) and neurocardiogenic syncope (NCS). We herein present our experience of 18 patients who we found displayed evidence of coexisting NCS and POTS.We reviewed charts of 300 POTS patients seen at the University of Toledo Syncope and Autonomic Disorders Center from 2003 to 2010 and found 18 patients eligible for inclusion in this study. Patients were included in this study if they reported clinical symptoms consistent with both POTS and NCS and then demonstrated a typical POTS pattern (a rise in heart rate without change in blood pressure [BP]) on head up tilt table (HUTT) within the first 10 minutes of upright posture followed by a neurocardiogenic pattern (a sudden fall in heart rate and/or fall in blood pressure) reproducing symptoms that were similar to the patients spontaneous episodes.We found 18 patients, mean age (30 ± 12), with 15 (84%) women and three (16%) men, who met the inclusion criterion for this study. Each of these 18 patients demonstrated a typical POTS pattern within the first 10 minutes on initial physical exam and on a HUTT. Continued tilting beyond 10 minutes resulted in a sudden decline in heart rate (which in some patients manifested as an asystole that lasted anywhere between 10 and 32 seconds [mean of 18 seconds]) and/or a fall in BP in each of these patients demonstrating a pattern consistent with neurocardiogenic subtype of OI. The mean time to the NCS pattern of a fall in BP and heart was 15 minutes with a range of 13-20 minutes. This group of patients was highly symptomatic and reported frequent clinical symptoms that were suggestive of OI. Recurrent presyncope, syncope, orthostatic palpitations, exercise intolerance, and fatigue were the principal symptoms reported.NCS may coexist with POTS in a subgroup of patients suffering from OI.

Autonomic dysregulation (also called diencephalic epilepsy) has been reported following traumatic brain injuries (TBI). However, until now, postural tachycardia syndrome (POTS) has not been reported as a long-term complication in patients who have suffered a TBI. We report on a series of patients who developed POTS after suffering TBI.Eight patients who were referred to our center had suffered TBI and developed features of orthostatic intolerance following head trauma. The patients' neurological, neurosurgical and autonomic data (charts and/or physician letters) were then carefully reviewed for demographic characteristics, comorbid conditions, symptoms of orthostatic intolerance, medications and response to medication. These patients were diagnosed as having POTS, primarily based on their clinical features and findings from the head-up tilt test (HUTT). The data presented is observational and descriptive (percentages or means).Eight patients (seven of them women) aged 21-41 years had suffered from TBI and had developed features of POTS. All had been normal with no symptoms prior to their TBI. All patients experienced orthostatic dizziness, fatigue, palpitations and near syncope. Six patients suffered from frank syncope. Six patients developed significant cognitive dysfunction, and three developed a chronic pain syndrome following trauma. All of the patients reported severe limitations to their daily activities and had been unable to keep their jobs, and two were housebound. Six patients demonstrated a good response to therapy with various combinations of medication. The symptoms of orthostatic intolerance and syncope improved with the initiation of medical therapy, as well as their reported quality of life. Two patients failed to show any improvement with various combinations of medications and tilt training, and continued to experience orthostatic difficulties.Postural tachycardia syndrome may, in some cases, be a late complication of traumatic brain injury.