Abstract

Objective: To describe the clinical features and diagnostic findings in children and adolescents with POTS. Background POTS involves chronic orthostatic intolerance (OI) and multi-system, non-orthostatic symptoms. Clinical description of a large series of pediatric patients with POTS is needed given the challenges in diagnosis and treatment. Design/Methods: We undertook a retrospective study and medical records review of 64 patients evaluated between April 2007-March-2011 for probable POTS by standing or head-up tilt test (HUT) or both at a Tertiary Pediatric Neurology Clinic. POTS was defined as frequent, 2 or more symptoms of OI for >3 months and a HR increment of >30 bpm or HR >120 bpm within 10 minutes of active standing or HUT, without arterial hypotension. Results: 57 patients had POTS (84% female; age 15 ± SD of 2.2 years), with mean duration of symptoms of 2 years. Mean HR increment with 33 bpm with standing and 41 with HUT, and mean maximal absolute HR was 105 with standing and 110 with HUT. Pre-existing conditions included ADHD (23%), anxiety (23%), Depression (19%), and 35% of menstruating females had menorrhagia. Antecedent infectious illness was reported in 29%. Non-orthostatic complaints included nausea (60%), abdominal pain (42%), vomiting (26%), weight loss of 1-8 kg (26%), rumination (9%), and sweating disorder (49%). Other symptoms included chronic fatigue (79%), cognitive dysfunction (44%), problems concentrating (42%), brain fog (35%), memory problems (26%), migraine (46%), chronic daily headache (30%), and sleep disorder (56%). Quantitative sudomotor axon testing was abnormal in 24 of 35 patients (67%), whereas Valsalva and deep breathing tests abnormalities were rare. Gastrointestinal diagnostic tests included abnormal GI motility with gastroparesis (11/44, (25%), gastric emptying (11/17, 67%), and HIDA scans (3/8, 37%). Conclusions: Our data demonstrate the multi-system involvement in pediatric POTS and the high prevalence of significant autonomic dysfunction and abnormal diagnostic tests. Disclosure: Dr. Hernandez has nothing to disclose. Dr. Jarjour has nothing to disclose. Dr. Jarjour has nothing to disclose.

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