Pediatric-Onset Postural Orthostatic Tachycardia Syndrome in a Single Tertiary Care Center

Abstract

Aim: We characterize the pediatric postural orthostatic tachycardia syndrome (POTS) population seen at a single tertiary care referral center. Method: Retrospective chart review of all pediatric POTS patients seen in our center between 2015 and 2017. Pediatric POTS was defined as chronic, at least 3 months, symptoms of orthostatic intolerance associated with excessive orthostatic tachycardia as determined by tilt table testing with orthostatic heart rate increment of ≥40 bpm within 5 minutes of head-up tilt or absolute orthostatic heart rate ≥130 bpm for patients 13 years old and younger and ≥120 bpm for those 14 years and older. We looked at demographics, presenting symptoms, comorbidities, examination findings, investigation findings, treatment, and patient reported outcomes. Outcome measures were separated by patient report and group comparisons were made using 2-sample t tests or Mann-Whitney U tests for continuous variables and Fisher exact tests for categorical variables. Results: One hundred thirty-four patients with pediatric onset POTS were identified. The mean age was 15 years. Seventy-nine percent of patients were female and 90% were white. The most common presenting symptoms included dizziness/lightheadedness (88%), syncope (54%), and palpitations (40%). Many patients had significant comorbidities attributable to numerous bodily systems, most commonly headache syndromes (migraine 43%, nonspecific headache 22%, chronic daily headache 14%, and new daily persistent headache 5%) and chronic fatigue (60%). Low vitamin D and insufficient iron stores were commonly seen. The majority of patients improved or had resolution of symptoms following treatment (70%). When separated by outcome, statistically significant differences were found for glucose (patients whose symptoms resolved had higher median glucose), palpitations (patients whose symptoms resolved were less likely to have palpitations), constipation (patients whose symptoms were stable/worsened were more likely to have constipation), and unexplained pain (patients whose symptoms were stable/worsened were more likely to have unexplained pain). Conclusions: Pediatric POTS is a chronic condition with a fairly good prognosis following appropriate treatment. It is associated with numerous comorbidities that necessitate multidisciplinary expert care.

The Application of Head-Up Tilt Test to Diagnose Hemodynamic Type of Orthostatic Intolerance in Children Aged Between 3 and 5 Years

“…The results suggested that headaches and syncope were the most frequent symptoms (46 and 29%, respectively) (4). In a single center, they reported that dizziness was the most common symptom in pediatric patients with POTS (age 5-18 years) (12). In our study, the most frequent symptoms were syncope (28.7%) and dizziness (22.6%), and 5.5% of cases had headache symptoms.…”

Symptom Score: A New Instrument to Assess Orthostatic Intolerance in Children and Adolescents

“…Plash et al 25 suggested that the optimal cut-off value of orthostatic heart rate increment to discriminate postural tachycardia syndrome at 10 minutes was 38 beats/min in the head-up tilt test and 29 beats/min in the standing test for adults. Similarly, Staples et al 8 reported that the mean orthostatic heart rate increment was 29 beats/min in the standing test and 49 beats/min in the head-up tilt test for children and adolescents. And the symptom score in our study combines presence and frequency of symptoms, rather than the single presence of symptoms.…”

“…We showed that dizziness and syncope were most common symptoms, followed by blurred vision and fatigue, which is consistent with clinical experience and prior reports. 7,8 In addition, we found that prevalence of dizziness, headache, blurred vision, palpitations, chest discomfort, gastrointestinal symptoms, profuse perspiration, and syncope was significantly different among head-up tilt test–negative, vasovagal syncope, and postural tachycardia syndrome groups. And the odds ratios of the above 8 symptoms by multiple logistic regression analysis were all greater than 1, which indicated risk factors for head-up tilt test positivity.…”

“…6 In contrast, postural tachycardia syndrome is historically chronic, not episodic, and there is usually day-to-day orthostatic symptoms like palpitations, chest discomfort, or fatigue. Li et al 7 and Staples et al 8 showed that dizziness is the most common presenting symptom (84%-88%) in the postural tachycardia syndrome subjects, followed by syncope (54%-63%). According to a previous study, 22% dizziness can be attributed to vasovagal syncope or orthostatic hypotension, 20 but it is not a predominant feature of vasovagal syncope (19/88, 22% in our study).…”

“…6 Syncope is not a predominant feature of postural tachycardia syndrome. 7,8 Several orthostatic intolerance quantitative diagnostic scores were proposed on the basis of a careful history, such as Calgary Syncope Diagnostic Scores, 9 Orthostatic Intolerance Questionnaire, 10 Orthostatic Grading Scale. 11 However, subjects of most studies were adults.…”