Continuous progression of orthostatic tachycardia as a further feature of the postural tachycardia syndrome

BACKGROUND: The clinical diagnosis of the postural tachycardia syndrome (POTS) includes the demonstration of an upright heart rate (HR) of at least 30 beats per minute (bpm) above supine HR. The dynamic behavior of HR during the course of standing has not yet been studied systematically in POTS. METHODS: HR and arterial blood pressure (ABP) were continuously monitored in 17 POTS patients and in 24 age-matched controls at rest and during an 11-minute phase of 80 degrees tilt. RESULTS: ABP values at different time intervals of the protocol did not differ between the subgroups with the exception of higher diastolic pressures in POTS after 5 and 10 minutes of tilt. POTS patients showed a higher resting HR (80.6 +/- 17.0 bpm vs 67.8 +/- 10.9 bpm in controls, P < 0.05) and there was a continuous HR acceleration in the course of the 11-minute tilt phase. In control subjects, the tilt-induced HR increase was nearly completed after 1 minute with only a minimal further rise between minute 1 and minute 10 (from 83.7 +/- 11.5 to 85.3 +/- 11.9 bpm vs from 106.1 +/- 15.6 to 120.1 +/- 13.8 bpm in POTS). CONCLUSIONS: Continuously progressing orthostatic tachycardia can serve as an additional criterion in the diagnosis of POTS. It may be related to the recently observed increased orthostatic capillary filtration rate in POTS.     

Inappropriate sinus tachycardia, postural orthostatic tachycardia syndrome, and overlapping syndromes

BACKGROUND: Inappropriate sinus tachycardia (IAST) and postural orthostatic tachycardia syndrome (POTS) are syndrome complexes with some distinctive features, overlapping clinical manifestations, and potential common mechanisms. Pathogenesis of these overlapping syndromes is poorly understood. Diagnostic and therapeutic approaches have not been standardized. PURPOSE: This article provides an overview of the definition, clinical presentation, and proposed mechanisms of IAST and other overlapping syndromes. A stepwise diagnostic approach is suggested. A multidisciplinary management scheme is outlined. METHODS: A MEDLINE search for English-language articles on IAST, POTS, and chronic orthostatic intolerance published up to 2005 was performed. Published data incorporated with our clinical experience were synthesized and presented in this review. RESULTS: The population of IAST is heterogeneous and underlying mechanisms are complex and likely multifactorial. Evidence suggests that both cardiac and extracardiac causes are plausible. Regional and limited autonomic neuropathies, at least in part, can provide a mechanism-based explanation of the cardiovascular indices and clinical symptoms in a significant number of patients with IAST. The regional abnormalities can be detected by autonomic testing. Among patients with IAST and evidence of autonomic dysregulation, an integrated autonomic, cardiovascular, and psychiatric management approach appears to be logical and rational when appropriate. Sinus node ablation could be considered in patients with persistent IAST in the absence of autonomic neuropathy and multisystem symptoms. Data from long-term outcomes are lacking. CONCLUSION: The current understanding of IAST mechanisms is incomplete and management approach is not adequate. Significant effort needed in clinical research to improve therapeutic outcome.     

Ivabradine in the treatment of postural tachycardia syndrome (POTS), a single center experience

1 Background

Ivabradine is a selective I_f 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).

2 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: < 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.

3 Conclusion

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.     

Pregnancy in postural orthostatic tachycardia syndrome

INTRODUCTION: Postural orthostatic tachycardia syndrome (POTS) is a rare disease characterized by syncope, sinus tachycardia, and orthostasis due to autonomic dysfunction. METHODS AND RESULTS: Two women aged 26 and 24 years with severe POTS became pregnant. Both women experienced hyperemesis gravidarum with subsequent marked improvement in their POTS symptoms until 6 months gestation, when their syncope and sinus tachycardia caused clinical decompensation. Both patients delivered healthy babies at 37 weeks by elective cesarean section. CONCLUSION: In long-term follow-up, both women reported improvement in their prepartum symptoms. We describe the first report, to our knowledge, of two successful pregnancy outcomes in severe POTS, including the first report of midodrine use in pregnant women.     

Pyridostigmine in the treatment of postural orthostatic tachycardia: a single-center experience

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 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, 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; 750–755)     

Neurocardiogenic syncope coexisting with postural orthostatic tachycardia syndrome in patients suffering from orthostatic intolerance: a combined form of autonomic dysfunction

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 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. 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 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. Conclusion: NCS may coexist with POTS in a subgroup of patients suffering from OI. (PACE 2011; 34:549–554)     

Reversible postural orthostatic tachycardia syndrome

Postural orthostatic tachycardia syndrome(POTS) is a relatively rare syndrome recognised since 1940. It is a heterogenous condition with orthostatic intolerance due to dysautonomia and is characterised by rise in heart rate above 30 bpm from base line or to more than 120 bpm within 5-10 min of standing with or without change in blood pressure which returns to base line on resuming supine position. This condition present with various disabling symptoms such as light headedness, near syncope, fatigue, nausea, vomiting, tremor, palpitations and mental clouding, etc. However there are no identifiable signs on clinical examination and patients are often diagnosed to have anxiety disorder. The condition predominantly affects young female between the ages of 15-50 but is rarely described in older people. We describe an older patient who developed POTS which recovered over 12 mo. Recognising this condition is important as there are treatment options available to alleviate the disabling symptoms.     

体位性心动过速综合征的年龄和性别差异

目的探讨体位性心动过速综合征(POTS)的年龄和性别差异。方法2001-01~2005-05在中南大学湘雅二医院从316例直立倾斜试验(HUTT)检查中诊断的POTS患者66例,分为成年(>18岁)和儿童(≤18岁)两组,分析其病程、诊断率、心率变化的年龄和性别差异。结果①病程及发病年龄:POTS病程在成年组1.00~11.33 a(平均5.22±4.33 a),男性略短于女性(3.00±4.24 a vs 5.71±4.44 a,P>0.05);儿童组0.08~4.00 a(平均0.73±1.13 a),男性长于女性(1.21±1.46 avs 0.46±0.81 a,P<0.05)。发病年龄在成年组为18~49岁(平均25.75±8.33岁),女性小于男性(P<0.05);儿童组为4~16岁(平均11.73±2.54岁),未见性别差异(P>0.05)。②诊断率及心率变化:POTS诊断率在年龄组间无差异(P>0.05),女性比例成年高于儿童;倾斜试验10 min内心率增加量在年龄组间及男女间未见差异(P>0.05)。结论POTS病程及心率增加量不存在年龄和性别差异,成年患者发病年龄女性<男性,诊断率无年龄差异,但成年女性比例高于儿童,这对临床上诊治POTS具有指导意义。     

The postural orthostatic tachycardia syndrome: a potentially treatable cause of chronic fatigue, exercise intolerance, and cognitive impairment in adolescents

Head upright tilt table testing has become an accepted method to measure an individual's predisposition to autonomically mediated periods of hypotension and bradycardia severe enough to cause frank syncope. At the same time it has become increasingly apparent that less profound falls in blood pressure, while not sufficient to result in loss of consciousness, may cause symptoms such as near syncope, vertigo, and dizziness. We describe a subgroup of adolescents that have a mild form of autonomic dysfunction that exhibit disabling symptoms such as postural tachycardia and palpitations, extreme fatigue, lightheadedness, exercise intolerance, and cognitive impairment. During baseline tilt table testing at a 70 degrees angle, these patients demonstrated a heart rate increase of > or = 30 beats/min (or a maximum heart rate of > or = 120 beats/min) within the first 10 minutes upright (not associated with profound hypotension), which reproduced their clinical symptom complex. Similar observations have been made in the adult population and has been termed the postural orthostatic tachycardia syndrome (POTS). We report that POTS may also occur in adolescents and represents a mild, potentially treatable form of autonomic dysfunction that can be readily identified during head upright tilt table testing.     

Asymptomatic Brugada Syndrome Associated with Postural Orthostatic Tachycardia Syndrome:

Autonomic imbalance may work as a modifying factor for initiating lethal arrhythmia in patients with Brugada syndrome. A 26-year-old man with episodes of near syncope was given a diagnosis of an autonomic disorder, postural orthostatic tachycardia syndrome (POTS). The patient spontaneously showed typical Brugada-type ECG, and ventricular fibrillation was induced by programmed electrical stimulation, which allowed the further diagnosis of Brugada syndrome. Although it seems that Brugada syndrome is asymptomatic, its uncommon association of POTS may increase the risk for future arrhythmic events in this patient.     

重度颅脑外伤并发MODS的临床救治研究

目的：探讨重度颅脑外伤并发多器官功能障碍综合征（MODS）的发病机制与综合治疗方法。方法：回顾性分析1995-2000年来我院NICU中收治的重度颅脑外伤并发MODS患者43例的临床资料。结果：197例重度颅脑外伤患者救治过程中出现MODS43例（发生率为21.83%),其中死亡28例（病死率65.12%);发生肺功能障碍28例（发生率65.12%)。此外,广泛脑实质的损伤、脑疝的发生,入院时的低格拉斯哥昏迷评分是发生MODS的危险因素;脑干与下丘脑的损害是MODS发生的始动因素。结论：及时有效地缓解颅高压,恢复脑干功能是治疗的首要问题;而肺部并发症的合理治疗是控制MODS的重要环节;综合救治策略仍然是降低病死率的关键手段。     

体位性心动过速综合征

体位性心动过速综合征（ postural tachycardia syndrome , POTS）是直立不耐受的最常见表现之一,可有直立体位的大脑低灌注和交感神经过度兴奋的临床症状,包括轻度淡漠、视物模糊、认知障碍和虚弱、心悸、胸痛及震颤等,卧位可缓解,部分患者可有直立诱发的血管迷走性晕厥。 POTS表现多样,发病机制较复杂,可能与免疫、基因变异、神经系统传导和调节功能、精神心理因素等相关,治疗上需要根据具体类型和发病机制选择不同的方案。     

Idiopathic osteoporosis,Ehlers–Danlos syndrome,postural orthostatic tachycardia syndrome,and mast cell activation disorder in a 27‐year‐old male patient: A unique case presentation

A young male patient presents with widespread pain and varying chronic inflammatory symptoms for three years and idiopathic low bone density for more than ten years. Based on the patient’s clinical history, the patient has been diagnosed with an hypermobile Ehlers–Danlos syndrome, postural orthostatic tachycardia syndrome, and mast cell activation disorder trifecta with affiliated inflammation‐induced osteoporosis.     

Outcomes in Adolescents with Postural Orthostatic Tachycardia Syndrome Treated with Midodrine and β-Blockers

Background: Postural orthostatic tachycardia syndrome (POTS) is associated with debilitating fatigue, dizziness, and discomfort in previously healthy adolescents. The effects of medical therapy have not been well studied in this patient population. This study assessed the relative efficacy and impact of drug therapy on the functioning and quality of life in adolescents with POTS.
Methods: A retrospective, single center, chart review analysis with a follow-up written survey was conducted on a group of 121 adolescents who had undergone autonomic reflex screening at the Mayo Clinic from 2002 to 2005 as part of an evaluation for possible POTS.
Results: Of 121 surveys sent, 47 adolescents returned a completed survey. In this cohort of patients, the two most commonly prescribed drug therapies were midodrine (n = 13) and β-blockers (n = 14). Patients in the midodrine group were comparable to patients in the β-blocker group in gender, age, pretreatment postural heart rate changes, and months from initial evaluation to survey completion. More patients treated with a β-blocker reported improvement after visiting Mayo Clinic (100% vs 62%, P = 0.016) and more attributed their progress to medication (63.6% vs 36.4%, P = 0.011) than did those treated with midodrine.
Conclusion: Treatment with both midodrine and β-blockers was associated with overall improvement in POTS patients' general health; however, adolescents taking β-blockers were more likely than those taking midodrine to credit the role of medications in their improvement.     

Supraventricular tachycardia with a baseline ECG pattern of Brugada syndrome

The typical arrhythmias found in patients with the Brugada syndrome is either ventricular fibrillation or polymorphic ventricular tachycardia. We report the case of a patient who presented with supraventricular tachycardia accompanied by ECG features of the Brugada syndrome (BrS) during both tachyarrhythmia and sinus rhythm. This case lends support to the recently reported association between supraventricular tachyarrhythmia and BrS and the hypothesis that the arrhythmogenic substrate in BrS is not limited to the ventricular level.     

Brugada syndrome: a case report of monomorphic ventricular tachycardia

A 56-year-old woman without structural heart disease had an ECG typical of Brugada syndrome. Syncope occurred due to monomorphic VT with left bundle branch block (LBBB) morphology. At electrophysiological study, VT with the same morphology was inducible.     

伴多器官功能障碍的急性肾损伤患者危险因素及预后分析

目的 观察并比较合并多器官功能障碍（multiple organ Ddysfunction syndrome,MODS）的急性肾损伤（acute kidney injury,AKI）患者的临床及预后特点,并分析AKI患者合并MODS的危险因素.方法 回顾性分析上海瑞金医院1997～2010年间住院患者中合并MODS的130例AKI患者的临床病例资料,并与同期收集的910例未合并MODS的AKI患者进行比较分析.结果 合并MODS的患者占同期AKI患者的12.5％,和NonMODS组相比,年龄高于NonMODS组（55.57±20.16 vs.49.57±19.90,P=0.001）,性别比例无明显差异;RIFLE分级有显著统计学差异,偏向更高严重等级（x2=24.193,P＜0.001）,接受RRT 的比例明显高于对照组,有显著统计学差异（x2=52.237,P＜0.001）;MODS-AKI患者肾功能恢复率为28.5％,明显低于NonMODS组（x2=94.271,P＜0.001）,死亡率达70％,和对照组相比有明显统计学意义（x2=256.152,P＜0.001）,但预后和RIFLE分级及RRT情况无明显相关,和MODS数目呈正相关（r=0.406,P＜0.001）;并发MODS危险度最高的因素为机械通气（P＜0.001,OR=14.966）,其余依次为低血压（P＜0.001,OR=5.422）、急性胰腺炎（P=0.004,OR=3.995）、充血心力衰竭（P＜0.001,OR=3.527）、败血症（P=0.018,OR=3.357）、发热（P=0.001,OR=2.477）、CKD （P＜0.001,OR=0.355）,但CKD是否在该过程中扮演保护性角色尚需进一步研究来证实.结论 AKI患者中并发MODS的人群年龄偏高于NonMODS者,性别比例无特殊,RIFLE标准在评估MODS-AKI的预后方面有局限性,RRT在改善MODS-AKI的预后方面的作用本研究未能证实.合并MODS的AKI患者在临床上有其自身特点,要区别于普通AKI而对待.当AKI患者进行机械通气或合并血流动力学改变、感染及急性胰腺炎等急性损伤因素时MODS的发生率增高,因此要注意严密监测上述情况相关指标预防MODS的发生以改善AKI患者预后.     

Postural Orthostatic Tachycardia Syndrome: A Rare Complication Following Electrical Injury

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)     

体位干预防治剖宫产术产妇仰卧位低血压综合征的效果

目的 研究体位干预防治剖宫产产妇仰卧位低血压综合征(SHS)的效果.方法 回顾性分析2018年12月至2019年12月收治的86例剖宫产产妇的临床资料,按照护理方法的不同将其分为参照组和体位干预组,每组43例.麻醉后,参照组采取将手术床朝向左侧并倾斜30°的体位进行护理,体位干预组采取将孕妇右侧臀部垫起4～5 cm的体...     

Post-cardiac injury syndrome: an atypical case following percutaneous coronary intervention

Post-cardiac injury syndrome (PCIS) is a syndrome characterized by pericardial and/or pleural effusion, triggered by a cardiac injury, usually a myocardial infarction or cardiac surgery, rarely a minor cardiovascular percutaneous procedure. Nowadays, the post-cardiac injury syndrome, is regaining importance and interest as an emerging cause of pericarditis, especially in developed countries, due to a great and continuous increase in the number and complexity of percutaneous cardiologic procedures. The etiopathogenesis seems mediated by the immunitary system producing immune complexes, which deposit in the pericardium and pleura and trigger an inflammatory response. We present the atypical case of a 76-year-old man presenting with a hydro-pneumothorax, low-grade fever and elevated inflammation markers, after two complex percutaneous coronary interventions, executed 30 and 75 days prior. The clinical features of our case are consistent with the diagnostic criteria of PCIS: prior injury of the pericardium and/or myocardium, fever, leucocytosis, elevated inflammatory markers, remarkable steroid responsiveness and latency period. Only one element does not fit with this diagnosis and does not find any further explanation: the air accompanying the pleural effusion, determining a hydro-pneumothorax and requiring a pleural drainage catheter positioning.