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Hypovolemic and hemodynamic disorders，autonomic nervous dysfunction and hyperadrenergic state are recognized pathogenesis of postural tachycardia syndrome（POTS）. According to this，POTS can be divided into three types：hypovolemic POTS，neurogenic POTS and hyperadrenergic POTS. Autoimmune abnormalities，changes in hormone levels，and gene mutations may also cause POTS. In addition，some patients have joint overactivity syndrome，functional gastrointestinal disorders，Ehlers Danlos syndrome，mast cell activation disorders and other diseases.Therefore，POTS is a complex clinical syndrome that may involve multiple mechanisms.This article aims to offer clinicians an improved understanding of the pathogenesis of pediatric POTS through a comprehensive review of this disease.
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Postural tachycardia syndrome (POTS) is a common disease of the autonomic nervous system in childhood, characterized by sudden changes in posture or prolonged standing with abnormally rapid heart rate and chronic orthostatic intolerance such as headache, chest tightness or pain, fatigue, sighing, amaurosis, or syncope.The diagnostic criteria for POTS in children are different from those in adults, and the standing test and the head-up tilt test are still used as effective diagnostic tools.In recent years, it has been reported that significant progress has been made in the diagnosis of POTS in children, including characteristics of population age, changes of disease circadian rhythm, modify standing test applied in local hospitals, differential diagnosis of POTS by biological markers, etc.Early and rapid diagnosis of POTS in children can help children with timely intervention, which is conducive to the improvement of prognosis and quality of life.
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Vasovagal syncope (VVS) and postural orthostatic tachycardia syndrome (POTS) are the most common types of neurally mediated syncope, which is prevalent in school-age children and adolescents, seriously affecting their daily life and learning quality, and increasing the risk of accidental injury due to syncope.Because the clinical signs of these two diseases are very similar, diagnosis between VVS and POTS can easily be confused, and the pathogenesis of these two is not the same, the curative effect of empirical treatment is not very ideal.Therefore, finding a sensitive and reliable method for differential diagnosis and formulating individualized treatment plans have become an urgent clinical need in pediatrics syncope.In recent years, pediatricians utilizing biomarkers for study on differentiating VVS from POTS and predicting individualized treatment outcomes have made significant progress, thus promoting the precise treatment of syncope in children.
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A 25-year-old women was admitted to the department of Neurology in Affiliated 2nd Hospital of Hainan Medical University due to recurrent syncope for 8 years and return for 2 months. She had multiple episodes of syncope at onset. She presented with the feeling of weakness in both lower limbs, and fatigue in the past year. She experienced pain in the waist and limbs joint in recent three months. Physical examination showed joint hyperactivity in metacarpophalangeal joints of both upper limbs, increased skin elasticity. Active-standing transcranial Doppler (TCD) test showed that the average heart rate (HR) and the average middle cerebral artery (MCA) blood flow velocity in the supine position were 79 beats/min and 62 cm/s, respectively; while the average HR and the average MCA blood flow velocity in the standing position were 126 beats/min, 47 cm/s. Meanwhile,the blood pressure was normal during the test of supine-to-standing TCD. Genetic testing indicated LDB3 transgenation. The patient was diagnosed as postural tachycardia syndrome (joint-hypermobility-related), Ehlers-Danlos syndrome, and relieved by fluid infusion and rehabilitation therapy.
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Objective:To explore the significance of plasma homocysteine (Hcy) as a new biomarker for the differential diagnosis of postural tachycardia syndrome (POTS) and suspected myocarditis in children.Methods:A total of 24 children diagnosed with POTS and 21 children diagnosed with suspected myocarditis treated in the Pediatrics Department of the Peking University First Hospital from July to December 2016 were included in the study.Plasma Hcy levels were measured in each subject and compared between children with POTS and suspected myocarditis.The receiver operating characteristic (ROC) curves were depicted for assessing the diagnostic potential of Hcy in distinguishing POTS from suspected myocarditis.Results:Plasma Hcy level in the POTS group was significantly higher than that in the suspected myocarditis group [(14.25±8.09) μmol/L vs.(8.99±3.19) μmol/L], which was also significantly higher than that of the mean levels in Beijing children [(8.82±5.58) μmol/L] (all P<0.05). When the cut-off was 9.36 μmol/L, the area under the ROC curve was 0.76, and the sensitivity and specificity for distinguishing POTS from suspected myocarditis were 71% and 68%, respectively. Conclusions:Plasma Hcy levels are helpful in the differential diagnosis of POTS and suspected myocarditis in children.
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Vasovagal syncope (VVS) and postural tachycardia syndrome (POTS) are the main forms of orthostatic intolerance in pediatrics and both are underlying causes of neurally-mediated syncope. In recent years, increasing attention has been paid to the management of VVS and POTS in children and adolescents. A number of potential mechanisms are involved in their pathophysiology, but the leading cause of symptoms varies among patients. A few studies thus have focused on the individualized treatment of VVS or POTS based on selected hemodynamic parameters or biomarkers that can predict the therapeutic effect of certain therapies and improve their effectiveness. This review summarizes the latest developments in individualized treatment of VVS and POTS in children and indicates directions for further research in this field.
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Objective To investigate the changes in ventricular late potentials (VLP) in children and adolescents with postural tachycardia syndrome (POTS).Methods One hundred and forty-four children and adolescents diagnosed as POTS by using the head-up tilt test (HUTT) in Second Xiangya Hospital of Central South University from January 2012 to March 2019 were selected as POTS group,their age ranged 4 to 16 years old [(11.03 ± 2.34) years old],there were 65 boys and 79 girls.Ninety-one healthy children and adolescents matched by age and gender [aged 4-16 (11.22 ± 1.68) years old,there were 50 boys and 41 girls] were selected as the healthy control group.VLP was measured by SR-1000A ECG automatic analyzer (Boai,Guangdong,China).Results Compared with the healthy control group,the prevalence of positive VLP in the POTS group increased [11.1％ (16/144 cases)vs.1.1％ (1/91 cases)],and the difference was statistically significant(x2=6.904,P ＜ 0.01);the heart rate in the POTS group increased compared with that in the healthy control group [82.0 (74.0-95.0) times/min vs.78.0 (71.0-86.0) times/min],and the difference was statistically significant (Z =-2.265,P ＜ 0.05);the low-amplitude signal (LAS40) in the POTS group prolonged compared with that in the healthy control group[23.0 (17.0-31.0) μV vs.19.0 (13.0-25.0) μV],and the difference was statistically significant (Z =-4.114,P ＜ 0.01);root mean square amplitude(RMS40) increased compared with that in the healthy control group [61.3 (34.4-79.1) ms vs.52.0 (38.8-64.5) ms],and the difference was statistically significant (Z =-2.469,P ＜ 0.05).There was no statistically significant difference in total QRS time (TQRS) between 2 groups [86.0 (76.0-97.5) ms vs.87.0 (81.0-94.0) ms,Z =-0.007,P ＞ 0.05].Conclusions The positive rate of VLP is increased of children and adolescents with POTS.It suggests that there is an abnormal cardiac electrical activity in children and adolescents with POTS.
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OBJECTIVE: To discuss the relationship between body mass index and postural tachycardia syndrome in children and adolescents. METHODS: The clinical data of 127 children and adolescents were retrospectively analyzed,who were diagnosed with postural tachycardia syndrome(POTS)in the Department of Pediatric Cardiovasology,Children's Medical Center,the Second Xiangya Hospital,Central South University,from August 2009 to June 2018,which included63 males and 64 females and their ages were from 4 to 17 years old(mean age 11.31±2.53)(POTS group). A total of 107 healthy children and adolescents including 64 males and 63 females were included as the control group,who had a health examination in the health care clinic in the hospital during the same period(aged 7 to 17,with a mean age of11.60±3.27). Body length and body mass were measured and body mass index(BMI)calculated. Statistical analysis was conducted with SPSS 22.0 software. RESULTS:(1)Duration and frequency of syncope:the duration of syncope was(8.13±13.76)months in POTS group and the frequency of syncope was(1.45±4.43)times.(2)BMI intergroup comparison:comparing POTS group with control group,there was no difference in age,length or body mass(P>0.05),and BMI was significantly lower[(17.32 ± 2.65)kg/m~2 vs.(18.17 ± 2.42)kg/m~2,t=2.655,P<0.01]in POTS group.(3)BMI classification:low body mass was higher in POTS group(69.29%,88/127)than in control group(56.69%,72/127);normal body mass was lower in POTS group(29.13%,37/127)than in control group(41.73%,53/127）,χ~2=4.444,P<0.05. CONCLUSION: BMI of POTS group decreases significantly in children and adolescents,and it is lower in girls than in boys.
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Objective@#To investigate the changes in ventricular late potentials (VLP) in children and adolescents with postural tachycardia syndrome (POTS).@*Methods@#One hundred and forty-four children and adolescents diagnosed as POTS by using the head-up tilt test (HUTT) in Second Xiangya Hospital of Central South University from January 2012 to March 2019 were selected as POTS group, their age ranged 4 to 16 years old[(11.03±2.34)years old], there were 65 boys and 79 girls.Ninety-one healthy children and adolescents matched by age and gender[aged 4-16 (11.22±1.68) years old, there were 50 boys and 41 girls]were selected as the healthy control group.VLP was measured by SR-1000A ECG automatic analyzer (Boai, Guangdong, China).@*Results@#Compared with the healthy control group, the prevalence of positive VLP in the POTS group increased [ 11.1%(16/144 cases)vs.1.1%(1/91cases)], and the difference was statistically significant(χ2= 6.904, P<0.01); the heart rate in the POTS group increased compared with that in the healthy control group[82.0 (74.0-95.0) times/min vs.78.0 (71.0-86.0) times/min], and the difference was statistically significant(Z=-2.265, P<0.05); the low-amplitude signal(LAS40) in the POTS group prolonged compared with that in the healthy control group[23.0 (17.0-31.0) μV vs.19.0 (13.0-25.0) μV], and the difference was statistically significant(Z=-4.114, P<0.01); root mean square amplitude(RMS40) increased compared with that in the healthy control group[61.3 (34.4-79.1) ms vs.52.0 (38.8-64.5) ms], and the difference was statistically significant (Z=-2.469, P<0.05). There was no statistically significant difference in total QRS time (TQRS) between 2 groups [86.0 (76.0-97.5) ms vs.87.0 (81.0-94.0) ms, Z=-0.007, P>0.05].@*Conclusions@#The positive rate of VLP is increased of children and adolescents with POTS.It suggests that there is an abnormal cardiac electrical activity in children and adolescents with POTS.
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<p><b>Background</b>The pathogenesis of postural tachycardia syndrome (POTS) remains unclear. This study aimed to explore the changes and significance of sulfur dioxide (SO) in patients with POTS.</p><p><b>Methods</b>The study included 31 children with POTS and 27 healthy children from Peking University First Hospital between December 2013 and October 2015. A detailed medical history, physical examination results, and demographic characteristics were collected. Hemodynamics was recorded and the plasma SOwas determined.</p><p><b>Results</b>The plasma SOwas significantly higher in POTS children compared to healthy children (64.0 ± 20.8 μmol/L vs. 27.2 ± 9.6 μmol/L, respectively, P < 0.05). The symptom scores in POTS were positively correlated with plasma SOlevels (r = 0.398, P < 0.05). In all the study participants, the maximum heart rate (HR) was positively correlated with plasma levels of SO(r = 0.679, P < 0.01). The change in systolic blood pressure from the supine to upright (ΔSBP) in POTS group was smaller than that in the control group (P < 0.05). The ΔSBP was negatively correlated with baseline plasma SOlevels in all participants (r = -0.28, P < 0.05). In the control group, ΔSBP was positively correlated with the plasma levels of SO(r = 0.487, P < 0.01). The change in HR from the supine to upright in POTS was obvious compared to that of the control group. The area under curve was 0.967 (95% confidence interval: 0.928-1.000), and the cutoff value of plasma SOlevel >38.17 μmol/L yielded a sensitivity of 90.3% and a specificity of 92.6% for predicting the diagnosis of POTS.</p><p><b>Conclusions</b>Increased endogenous SOlevels might be involved in the pathogenesis of POTS.</p>
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Objective To investigate the changes of amplitude of T wave and ST segment between the supine and orthostatic electrocardiogram in children and adolescents with postural tachycardia syndrome (POTS). Methods A total of 74 children and adolescents diagnosed as POTS by head-up tilt test (HUTT),who complained of unexplained dizziness,headache,syncope or other symptoms,were chosen as POTS group. A total of 64 children and adolescents who did the health check in our child health clinic were selected as control group by age and sex in the same period. All the cases in this study came from the syncope specialized clinic or inpatient department of the Second Xiangya Hospital,Central South University,from August 2013 to January 2016. The heart rate and the 12-lead T-wave and ST-segment amplitude of the supine and orthostatic electrocardiogram were measured by software and manual intervention. Results (1) Com-pared with supine electrocardiogram,the heart rate of orthostatic electrocardiogram of POTS group increased [(105. 2 ± 16. 3) times/ min vs. (83. 8 ± 18. 0) times/ min,t = - 7. 598,P < 0. 01],the T-wave amplitude decreased in lead Ⅰ[(0. 28 ± 0. 14) mV vs. (0. 33 ± 0. 11) mV,t = 2. 598,P < 0. 05],Ⅱ[(0. 28 ± 0. 12) mV vs. (0. 39 ± 0. 13) mV,t = 5. 340,P < 0. 01],Ⅲ[(0. 00 ± 0. 19) mV vs. (0. 07 ± 0. 11) mV,t = 3. 041, P < 0. 01],aVF[(0. 14 ± 0. 13) mV vs. (0. 23 ± 0. 11) mV,t = 4. 505,P < 0. 01],V4 [(0. 33 ± 0. 21) mV vs. (0. 51 ± 0. 23) mV,t = 4. 938,P < 0. 01],V5 [(0. 37 ± 0. 10) mV vs. (0. 50 ± 0. 15) mV,t = 7. 764,P <0. 01] and V6 [(0. 25 ± 0. 10) mV vs. (0. 37 ± 0. 10) mV,t = 7. 538,P < 0. 01],the T-wave amplitude in-creased in lead aVR[( - 0. 27 ± 0. 11) mV vs. ( - 0. 36 ± 0. 10) mV,t = - 5. 023,P < 0. 01],and the ST-segment amplitude increased in lead V 5 [(0. 07 ± 0. 04) mV vs. (0. 06 ± 0. 04) mV,t = - 2. 309,P < 0. 05]. (2) Compared with control group,the difference of heart rate of POTS group increased [( - 21. 4 ± 14. 0) times/ min vs. ( - 10. 7 ± 11. 4)times/ min,t = 4. 875,P < 0. 01],and the difference of T-wave amplitude in-creased in lead Ⅰ[(0. 05 ± 0. 10) mV vs. ( - 0. 01 ± 0. 15) mV,t = - 3. 161,P < 0. 01],Ⅱ[(0. 11 ± 0. 12) mV vs. (0. 07 ± 0. 12) mV,t = - 2. 243,P < 0. 05],V4 [(0. 18 ± 0. 18) mV vs. (0. 07 ± 0. 20) mV,t =- 3. 282,P < 0. 01],V5 [(0. 18 ± 0. 11) mV vs. (0. 14 ± 0. 13) mV,t = - 2. 013,P < 0. 05] and V6 [(0. 13 ± 0. 08) mV vs. (0. 08 ± 0. 10) mV,t = - 3. 364,P < 0. 01],and the difference of T-wave amplitude in-creased significant in lead aVR[( - 0. 09 ± 0. 08) mV vs. ( - 0. 03 ± 0. 08) mV,t = 4. 109,P < 0. 01]. (3) Logistic regression analysis:the difference of heart rate and T-wave amplitude in lead V5 and V6 on supine and orthostatic electrocardiogram was statistically valuable for the diagnosis of POTS (P < 0. 05). (4) Diag-nostic test evaluation:when the difference of heart rate ≥15 times/ min,of T-wave amplitude in lead V5 and lead V 6≥ 0. 10 mV separately,on supine and orthostatic electrocardiogram at the same time,the sensitivity of the diagnosis of POTS was 37. 8％ and the specificity was 81. 3％ . (5)The follow-up of children and adoles-cents with POTS showed no significant difference of T-wave amplitude and ST-segment amplitude on ortho-static and supine ECG comparing to the initial diagnosis,regardless of the type of HUTT reaction remained or turned negative (P >0. 05). Conclusion The difference of heart rate and of T-wave amplitude in lead V5 and V6 are valuable for the diagnosis of POTS in children and adolescents.
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Postural tachycardia syndrome (POTS) is one type of orthostatic intolerance.The treatment for POTS including non-drug treatment and medications,such as α-receptor agonists,β-recepter blockers and oral rehydration salts.The prognostic meaning of biomarkers and hemodynamic parameters in the POTS children treated with midodrine hydrochloride are discussed in this paper.
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BACKGROUND AND PURPOSE: Postural tachycardia syndrome (POTS) is a syndrome of orthostatic intolerance in the setting of excessive tachycardia with orthostatic challenge, and these symptoms are relieved when recumbent. Apart from symptoms of orthostatic intolerance, there are many other comorbid conditions such as chronic headache, fibromyalgia, gastrointestinal disorders, and sleep disturbances. Dermatological manifestations of POTS are also common and range widely from livedo reticularis to Raynaud's phenomenon. METHODS: Questionnaires were distributed to 26 patients with POTS who presented to the neurology clinic. They were asked to report on various characteristics of dermatological symptoms, with their answers recorded on a Likert rating scale. Symptoms were considered positive if patients answered with "strongly agree" or "agree", and negative if they answered with "neutral", "strongly disagree", or "disagree". RESULTS: The most commonly reported symptom was rash (77%). Raynaud's phenomenon was reported by over half of the patients, and about a quarter of patients reported livedo reticularis. The rash was most commonly found on the arms, legs, and trunk. Some patients reported that the rash could spread, and was likely to be pruritic or painful. Very few reported worsening of symptoms on standing. CONCLUSIONS: The results suggest that dermatological manifestations in POTS vary but are highly prevalent, and are therefore of important diagnostic and therapeutic significance for physicians and patients alike to gain a better understanding thereof. Further research exploring the underlying pathophysiology, incidence, and treatment strategies is necessary.
Subject(s)
Humans , Arm , Exanthema , Fibromyalgia , Headache Disorders , Incidence , Leg , Livedo Reticularis , Neurology , Orthostatic Intolerance , Postural Orthostatic Tachycardia Syndrome , Primary Dysautonomias , Tachycardia , Varicose VeinsABSTRACT
Postural tachycardia syndrome (POTS)is one common type of orthostatic intolerance (OI)in chil-dren.POTS is characterized by OI symptoms and excessively increased heart rate from supine to upright position.Main treatments for POTS are physical training,automatic function exercise and medications including α-receptor agonists,β-receptor blockers and oral rehydration salts.This review aimed to summarize the biomarkers in the diagnosis and treatment of POTS.
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Currently we are still unclear in the aspect of etiology and mechanisms,and lack of medical advice based on evidence-based medicine on postural tachycardia syndrome,inappropriate sinus tachycardia and vasovagal syncope.It also prompts clinical research direction of the three special arrhythmia in future.Based on this,it is very necessary to interpret the management consensus of these three types of arrhythmia.
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Objective To analyze the spectrum of underlying diseases in children with transient loss of consciousness (TLOC) through a multi-center and large sample clinical research.Methods Nine hundred and thirty-seven children with TLOC who came from Beijing,Hunan province,Hubei province and Shanghai of China from Aug 1999 to Apr 2011 were recruited in the present study,and then the spectrum of underlying diseases in children with TLOC was analyzed.Results In 937 children with TLOC,903 cases (96.4％ )were children with syncope,34 cases (3.6％) were non-syncope.And in 903 children with syncope,213 cases (23.6％) had vasovagal syncope (VVS) with vasoinhibitory response,46 cases (5.1％ ) had VVS with cardioinhibitory response,112 cases ( 12.4％ ) had VVS with mixed response,268 cases (29.7％ ) had postural tachycardia syndrome,22 cases (2.4％) had orthostatic hypotension,19 cases (2.1％ ) had situational syncope,21 cases (2.3％ ) had cardiogenic syncope,and 202 cases (22.4％ ) had unexplained syncope.Conclusion In children with TLOC,syncope was the most common underlying disease.And in children with syncope,the most common was VVS,followed by postural tachycardia syndrome.In three different hemodynamic patterns of VVS,the most common pattern was VVS vasoinhibitory pattern.