Abstract 14031: Postural Orthostatic Tachycardia Syndrome With Associated Gastrointestinal Symptoms in Children; Is There an Underlying Neuropathic Substrate?
Introduction: Postural orthostatic tachycardia syndrome (POTS) results in multiple organ system symptoms. While the etiology for cardiovascular symptoms seems straightforward, the underlying pathophysiology for dysfunction in other organ systems remains unclear. Gastrointestinal symptoms (GIS) of nausea, vomiting and abdominal pain are common, up to 80% in some pediatric series. Treatment of cardiovascular problems improves GIS in some but not all POTS patients. We hypothesized that GIS may be secondary to an associated neuropathy of the upper GI tract.
Methods: Upper intestinal antroduodenal manometry (ADM) was performed for 24 hours in conjunction with pharmacologic (amitriptyline, metoclopramide, and erythromycin), autonomic and tilt table testing (TTT) to evaluate GI motility and assess its relationship to POTS symptoms. Twenty-six adolescent and young adult patients with POTS and GIS (age from 10 to 23.8, mean = 16.9 ± 2.7, years) were studied with ADM. Sixteen patients were female. Symptoms consisted of nausea - 24, abdominal pain - 23, dizziness - 26, and syncope - 8.
Results: Valsalva testing (+ 40 mm Hg) was abnormal in 17/25. QTc interval was ≥ 0.44 in 12/26. TTT was positive in 26/26 with a maximum increase in the HR= 57 ± 15 bpm, and maximum decrease in the systolic BP = 41 ± 28 mm Hg. ADM was abnormal in the baseline state in 7/26 (27%). Increase in antral contraction amplitude in response to pharmacologic challenge was limited, erythromycin (12/26), amitriptyline (13/25) and metoclopramide (4/25). ADM was abnormal during 14/24 TTT. Tilt induced nausea was associated with either duodenal - antral regurgitation (DAR) or isolated high amplitude duodenal contractions. TTT reproduced GIS in 20/25. ADM was abnormal in 22/26 (85%) either in the baseline state or during TTT.
Conclusions: ADM in children with POTS and GIS is frequently abnormal; some exhibit dysmotility in the baseline state, and many during TTT. Our ADM results concur with the poor clinical response observed during treatment with promotility agents. We found a specific association with DAR in this population, which may be secondary to an associated neuropathic intestinal dysmotility. POTS patients with GIS frequently demonstrate concomitant cardiovascular and intestinal neuropathy.
- © 2013 by American Heart Association, Inc.