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Short-Term Outcome in Children and Adolescents with Orthostatic Intolerance (OI) and Postural Tachycardia Syndrome (POTS)
Abstracts / Autonomic Neuroscience: Basic and Clinical 192 (2015) 56–141
P17.4 Effects of High Sodium Intake on Plasma Volume and Physical Fitness in Patients with Postural Tachycardia Syndrome and Healthy Females Jorge E. Celedonio, Emily M. Garland, Victor C. Nwazue, Sachin Y. Paranjape, Bonnie K. Black, Luis E. Okamoto, Cyndya A. Shibao, Alfredo Gamboa, Italo Biaggioni, David Robertson, André Diedrich, Satish R. Raj Autonomic Dysfunction Center, Departments of Medicine, Pharmacology and Neurology, Vanderbilt University, Nashville TN, USA Background: Patients with postural tachycardia syndrome (POTS) often have low blood volume and are advised to increase sodium intake to restore intravascular volume. However, the benefit of a high-sodium diet has not been systematically examined in POTS. Methods: We studied 12 female POTS patients (mean ± SD; 34 ±9 years, BMI 23 ± 3 kg/m2) and 8 female healthy control subjects (HC; 29 ± 4 years, BMI 24 ± 3 kg/m2) randomly assigned to 6 days of low (LS; 10 mEq/day) or high sodium (HS; 300 mEq/day) diet and then crossed-over. Procedures performed on Day 6 included: posture study, plasma volume (PV) measurement by 131I-albumin, maximal oxygen consumption (VO2max) using a supine bicycle ergometer, and cardiac output (CO) and stroke volume (SV) assessments by the inert gases rebreathing technique in the flat and tilted head-up positions. Results: PV was significantly higher after HS than LS in POTS patients (PV: 2706 ± 110 ml vs. 2390 ± 89 ml, P b 0.001). Orthostatic tachycardia was reduced in POTS with HS (49 ± 16 bpm vs. 63 ±11 bpm, P = 0.001), but still increased compared with HC (49 ± 16 bpm vs. 23 ± 11 bpm, P = 0.001). Upright SV was significantly higher after HS than LS in POTS (35 ± 11 ml vs. 25 ± 7 ml, P = 0.023) but remained lower than HC (35 ± 11 ml vs. 52 ± 16 ml, P = 0.033). Upright CO and VO2max did not differ significantly between POTS for either LS or HS. Conclusions: HS diet caused a reduction in orthostatic tachycardia, and increased PV and SV in POTS patients. Patients with POTS experienced improvement in their condition in association with higher sodium intake, but did not ‘normalize’ compared with HC.
doi:10.1016/j.autneu.2015.07.213
P17.5 Short-Term Outcome in Children and Adolescents with Orthostatic Intolerance (OI) and Postural Tachycardia Syndrome (POTS) I.T. Jarjoura, A.M. Hernandezb, L.K. Jarjoura a Department of Pediatrics, Texas Children’s Hospital, Baylor College of Medicine, Houston b Department of Pediatrics, Children’s Hospital of Los Angeles, University of Southern CA, USA Background: OI and POTS may cause severe functional disability. Outcome data in children and adolescents are scarce. Main symptoms include OI with lightheadedness, syncope, chronic fatigue (CF), gastrointestinal dysfunction (GI), cognitive dysfunction (CD), and headaches (HA). Methods: We reviewed the records of patients with chronic OI. We defined POTS as postural symptoms and postural heart rate increment N40 and OI if HR increment was ≥30- ≤ 40 bpm. Patients were treated with extra fluids, salt, exercise, vasoconstrictors, Beta-blockers, SSRI medications, iron and vitamin D, as clinically indicated. Recovery was defined as 100% improvement. The association between recovery rates of various symptoms was analyzed by Chi Square and regression. Results: 62 patients were
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studied (32 POTS, 30 OI), ages 15.2 ±2.2 years, follow up for 8.7 ± 9.1 months, 51 were females (82%). Total of 64% recovered in at least one main symptom. Rates of recovery were as follows (%): syncope (60), GI (36), OI (24), CF (19), HA (10), and CD (10). Length of follow up was similar between those who did or did not recover. One patient had full recovery of all 6 main symptom groups, and 5 had no recovery in any symptom. Symptom associations with less likely recovery (all p b 0.05) included OI and CD (CI 0.027-0.78), syncope and CD (CI 14-0.693), CF and CD (CI 0.008-0.844), and GI and constipation (CI 0.039-0.936). Conclusions: Complete recovery from POTS/OI is rare after short-term follow up. Syncope has best prognosis, whereas CF, HA, and CD have worse prognosis. Further research is needed to confirm these preliminary findings.
doi:10.1016/j.autneu.2015.07.214
P17.6 Arterial stiffness characterization in patients with Postural Orthostatic Tachycardia Syndrome and Ehlers-Danlos Syndrome Type 3 J.L. Chenga, J.S. Aua, H.L. Choia, C.A. Morillob, J.C. Guzmanb, M.J. MacDonalda a Department of Kinesiology, McMaster University and Department of Medicine b McMaster University, Hamilton, ON, Canada Background: Postural orthostatic tachycardia syndrome (POTS) is characterized by symptoms of orthostatic intolerance associated with an exaggerated increase in heart rate (HR) on standing. Recently, researchers have been reported that patients with Joint Hypermobility Syndromes, genetic disorders that affect collagen synthesis and deposition such as Ehlers-Danlos syndrome type 3 (EDS-3), have associated symptoms of POTS. The combination of these disorders may produce an altered vascular phenotype that has not been previously investigated. Aim: To characterize arterial stiffness in POTS + EDS-3 individuals and compare to healthy age-sex-matched controls (CON). Methods: Nine clinically diagnosed individuals with POTS + EDS-3, and 9 CON participated in the study. Vascular assessments were conducted after 10 minutes of supine rest. Primary outcome measures include central, upper limb, and lower limb arterial pulse wave velocity (PWV) obtained using applanation tonometry; local carotid artery distensibility measured with simultaneous ultrasound imaging and applanation tonometry; and carotid intima-media thickness (IMT) measured with ultrasound imaging 2-5 cm proximal to the carotid bifurcation. Results: No differences were observed between the POTS + EDS-3 and CON groups in carotid elasticity including compliance, distensibility and beta-stiffness. There were also no differences between groups in regional PWV including central, upper limb and lower limb pulse wave velocity. There were no significant differences in IMT between groups. Conclusions: Arterial stiffness is not significantly different between individuals with POTS + EDS-3 and CON. Vasoactive medications used by POTS + EDS-3 patients may preclude the detection of arterial stiffness differences. Further investigations should increase the sample size and be conducted in conditions absent of vasoactive medications.
doi:10.1016/j.autneu.2015.07.215
P17.4 Effects of High Sodium Intake on Plasma Volume and Physical Fitness in Patients with Postural Tachycardia Syndrome and Healthy Females Jorge E. Celedonio, Emily M. Garland, Victor C. Nwazue, Sachin Y. Paranjape, Bonnie K. Black, Luis E. Okamoto, Cyndya A. Shibao, Alfredo Gamboa, Italo Biaggioni, David Robertson, André Diedrich, Satish R. Raj Autonomic Dysfunction Center, Departments of Medicine, Pharmacology and Neurology, Vanderbilt University, Nashville TN, USA Background: Patients with postural tachycardia syndrome (POTS) often have low blood volume and are advised to increase sodium intake to restore intravascular volume. However, the benefit of a high-sodium diet has not been systematically examined in POTS. Methods: We studied 12 female POTS patients (mean ± SD; 34 ±9 years, BMI 23 ± 3 kg/m2) and 8 female healthy control subjects (HC; 29 ± 4 years, BMI 24 ± 3 kg/m2) randomly assigned to 6 days of low (LS; 10 mEq/day) or high sodium (HS; 300 mEq/day) diet and then crossed-over. Procedures performed on Day 6 included: posture study, plasma volume (PV) measurement by 131I-albumin, maximal oxygen consumption (VO2max) using a supine bicycle ergometer, and cardiac output (CO) and stroke volume (SV) assessments by the inert gases rebreathing technique in the flat and tilted head-up positions. Results: PV was significantly higher after HS than LS in POTS patients (PV: 2706 ± 110 ml vs. 2390 ± 89 ml, P b 0.001). Orthostatic tachycardia was reduced in POTS with HS (49 ± 16 bpm vs. 63 ±11 bpm, P = 0.001), but still increased compared with HC (49 ± 16 bpm vs. 23 ± 11 bpm, P = 0.001). Upright SV was significantly higher after HS than LS in POTS (35 ± 11 ml vs. 25 ± 7 ml, P = 0.023) but remained lower than HC (35 ± 11 ml vs. 52 ± 16 ml, P = 0.033). Upright CO and VO2max did not differ significantly between POTS for either LS or HS. Conclusions: HS diet caused a reduction in orthostatic tachycardia, and increased PV and SV in POTS patients. Patients with POTS experienced improvement in their condition in association with higher sodium intake, but did not ‘normalize’ compared with HC.
doi:10.1016/j.autneu.2015.07.213
P17.5 Short-Term Outcome in Children and Adolescents with Orthostatic Intolerance (OI) and Postural Tachycardia Syndrome (POTS) I.T. Jarjoura, A.M. Hernandezb, L.K. Jarjoura a Department of Pediatrics, Texas Children’s Hospital, Baylor College of Medicine, Houston b Department of Pediatrics, Children’s Hospital of Los Angeles, University of Southern CA, USA Background: OI and POTS may cause severe functional disability. Outcome data in children and adolescents are scarce. Main symptoms include OI with lightheadedness, syncope, chronic fatigue (CF), gastrointestinal dysfunction (GI), cognitive dysfunction (CD), and headaches (HA). Methods: We reviewed the records of patients with chronic OI. We defined POTS as postural symptoms and postural heart rate increment N40 and OI if HR increment was ≥30- ≤ 40 bpm. Patients were treated with extra fluids, salt, exercise, vasoconstrictors, Beta-blockers, SSRI medications, iron and vitamin D, as clinically indicated. Recovery was defined as 100% improvement. The association between recovery rates of various symptoms was analyzed by Chi Square and regression. Results: 62 patients were
121
studied (32 POTS, 30 OI), ages 15.2 ±2.2 years, follow up for 8.7 ± 9.1 months, 51 were females (82%). Total of 64% recovered in at least one main symptom. Rates of recovery were as follows (%): syncope (60), GI (36), OI (24), CF (19), HA (10), and CD (10). Length of follow up was similar between those who did or did not recover. One patient had full recovery of all 6 main symptom groups, and 5 had no recovery in any symptom. Symptom associations with less likely recovery (all p b 0.05) included OI and CD (CI 0.027-0.78), syncope and CD (CI 14-0.693), CF and CD (CI 0.008-0.844), and GI and constipation (CI 0.039-0.936). Conclusions: Complete recovery from POTS/OI is rare after short-term follow up. Syncope has best prognosis, whereas CF, HA, and CD have worse prognosis. Further research is needed to confirm these preliminary findings.
doi:10.1016/j.autneu.2015.07.214
P17.6 Arterial stiffness characterization in patients with Postural Orthostatic Tachycardia Syndrome and Ehlers-Danlos Syndrome Type 3 J.L. Chenga, J.S. Aua, H.L. Choia, C.A. Morillob, J.C. Guzmanb, M.J. MacDonalda a Department of Kinesiology, McMaster University and Department of Medicine b McMaster University, Hamilton, ON, Canada Background: Postural orthostatic tachycardia syndrome (POTS) is characterized by symptoms of orthostatic intolerance associated with an exaggerated increase in heart rate (HR) on standing. Recently, researchers have been reported that patients with Joint Hypermobility Syndromes, genetic disorders that affect collagen synthesis and deposition such as Ehlers-Danlos syndrome type 3 (EDS-3), have associated symptoms of POTS. The combination of these disorders may produce an altered vascular phenotype that has not been previously investigated. Aim: To characterize arterial stiffness in POTS + EDS-3 individuals and compare to healthy age-sex-matched controls (CON). Methods: Nine clinically diagnosed individuals with POTS + EDS-3, and 9 CON participated in the study. Vascular assessments were conducted after 10 minutes of supine rest. Primary outcome measures include central, upper limb, and lower limb arterial pulse wave velocity (PWV) obtained using applanation tonometry; local carotid artery distensibility measured with simultaneous ultrasound imaging and applanation tonometry; and carotid intima-media thickness (IMT) measured with ultrasound imaging 2-5 cm proximal to the carotid bifurcation. Results: No differences were observed between the POTS + EDS-3 and CON groups in carotid elasticity including compliance, distensibility and beta-stiffness. There were also no differences between groups in regional PWV including central, upper limb and lower limb pulse wave velocity. There were no significant differences in IMT between groups. Conclusions: Arterial stiffness is not significantly different between individuals with POTS + EDS-3 and CON. Vasoactive medications used by POTS + EDS-3 patients may preclude the detection of arterial stiffness differences. Further investigations should increase the sample size and be conducted in conditions absent of vasoactive medications.
doi:10.1016/j.autneu.2015.07.215