Hypoplasia of Aorta in Postural Orthostatic Tachycardia Syndrome (POTS)

Hypoplasia of Aorta in Postural Orthostatic Tachycardia Syndrome (POTS)

120 Abstracts / Autonomic Neuroscience: Basic and Clinical 192 (2015) 56–141 (p b 0.001), while there was no significant difference in the degree of ...

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120

Abstracts / Autonomic Neuroscience: Basic and Clinical 192 (2015) 56–141

(p b 0.001), while there was no significant difference in the degree of decrement comparing brain to calf perfusion. Conclusion: In pediatric and adolescent dysautonomia patients, there was significant decrease in perfusion from baseline in cerebral hemispheres, shoulders, low back, and calves during HUTT. The degree of perfusion impairment in the brain was similar to the gastrocnemius, suggesting a defect in vascular reactivity [cerebral autoregulation] and muscle vasculature. The physiological mechanism requires further investigation.

doi:10.1016/j.autneu.2015.07.209

P17 Human Autonomic Disease- POTS P17.1 Combined β-blockade and Splanchnic Venous Compression in the Treatment of POTS L.E. Okamotoa, A. Diedricha,b, A. Gamboaa, C. Shibaoa, B.K. Blacka, S.R. Raja, D. Robertsona, I. Biaggionia a Department of Medicine, Division of Clinical Pharmacology, Vanderbilt University, Nashville, TN, USA b Department of Biomedical Engineering, Vanderbilt University, Nashville, TN, USA Splanchnic venous compression is considered a non-pharmacological approach for the treatment of orthostatic intolerance in Postural Tachycardia Syndrome (POTS), but its efficacy has not been systematically evaluated. To assess whether abdominal compression attenuates the tachycardia and improve orthostatic symptoms in POTS, eighteen POTS patients (32 ± 2 yrs) received placebo, placebo combined with abdominal compression (40 mmHg) and propranolol 20 mg on separate days, in a randomized crossover study. Blood pressure (BP), heart rate (HR), and symptoms were assessed while seated and after standing for ≤10 min, before and 2 h postdrug. Abdominal compression was applied ~2 min before each stand period. As expected, propranolol decreased standing HR compared with placebo (78 ± 2 vs. 93 ± 4 bpm; P b 0.01) and this was associated with lower standing systolic BP (92 ± 2 vs. 101 ± 2 mmHg for placebo; P b 0.01). Abdominal compression had no effect on HR (92 ± 4 bpm) but increased standing systolic BP compared with placebo and propranolol (107 ± 2 mmHg; P b 0.01). Neither propranolol nor abdominal compression improved symptoms compared to placebo. In 15 patients we compared the combination of abdominal compression and propranolol 20 mg with propranolol alone. The combination had no additional effect on standing HR (78 ± 2 vs. 77 ± 3 bpm for propranolol) but prevented the fall in standing systolic BP produced by propranolol (98 ± 1 vs. 92 ± 2 mmHg for propranolol; P = 0.036), and significantly improved symptoms associated with low BP (-2.7 vs. 0.4 arbitrary units, for propranolol; P = 0.021). The combination also tended to improve total symptom burden. In conclusion, splanchnic venous compression might be a useful adjuvant therapy to propranolol in POTS.

doi:10.1016/j.autneu.2015.07.210

P17.2 Hypoplasia of Aorta in Postural Orthostatic Tachycardia Syndrome (POTS) Abdelmassih Hany Samy, Armanious Heidi Henry, Chandralekha Ashangari, Amer Suleman Department of Cardiology -The Heartbeat Clinic, McKinney, TX

Background: POTS is a form of orthostatic intolerance that is associated with the presence of excessive tachycardia and many other symptoms upon standing. Previous Studies indicated that Patients with POTS have hypoplasia of heart and Left Ventricular Outflow tract (LVOT) velocity in POTS patients is increased. Aim: The aim of this research is to study the dimensions and velocity of blood flow in Aorta. Methods: 204 patients are referred to our clinic from January 2014 to April 2015.Out of 204 Patients, 102 are POTS patients and 102 are non-POTS controls. Width of the Aorta (cm) Proximal, Middle, Distal and Velocity of the Aorta (cm/s) in the middle aorta is recorded in all the patients .Data Analysis is done on the findings. Results: Out of 102 POTS patients, 90% are Females (n = 92, age 32.67 ± 10.22), 10% are males (n = 10,age 29.6 ± 15.83), Width of the Aorta (cm) Proximal 1.45 ± 0.31, Middle 1.31 ± 0.27, Distal 1.33 ± 0.23, Velocity of Blood in the Aorta (cm/s) Middle 114.04 ± 26.32. Out of 102 controls, 61% are Females (n = 62, age 32.68 ± 13.23), 39% are males (n = 40,age 36.62 ± 10.64) Width of the Aorta (cm) Proximal 1.72 ± 0.28, Middle 1.61 ± 0.32, Distal 1.58 ± 0.48 , Velocity of Blood in the Aorta (cm/s) Middle 86.6 ± 25.91.Width of the Aorta (cm) Proximal POTS VS controls (P b 0.1), Width of the Aorta (cm) Middle POTS VS controls (P b 0.1), Width of the Aorta (cm) Distal POTS VS controls (P b 0.1), Velocity of Blood in the Aorta (cm/s) Middle POTS VS controls (P b 0.1). Conclusion: First time, our research study demonstrated that POTS patients have lesser dimensions and High blood flow Velocity in Aorta compared to controls. doi:10.1016/j.autneu.2015.07.211

P17.3 Regulation of Circulation during Exercise in Adolescents with Postural Orthostatic Tachycardia Syndrome (POTS) P.T. Pianosi, P.R. Fischer Department of Pediatric and Adolescent Medicine, Mayo Clinic, Rochester, MN, USA Introduction: We previously showed that up to 40% of adolescents with POTS have hyperkinetic circulation (“high-flow” variant) during exercise. We postulated that a low-flow variant similarly has its own unique circulatory response to exercise. Methods: We reviewed records of adolescents presenting with POTS (orthostatic ΔHR ≥ 40 bpm) from June 2012 to Dec. 2014 who underwent maximal exercise testing with cardiac output (Q) measurements at rest and during exercise. Results: There were 239 POTS patients 10-19 yrs old (73% female). Q was plotted vs oxygen uptake (VO2) and a frequency histogram of slopes was drawn. Patients whose Q- VO2 was ≤3 L/min per L/min were designated lowflow variant (N = 28, 11%) while normal-flow patients had Q- VO2 slope of 3-8 (mean 5.29 ± 1.23) L/min per L/min. Cardiac index at rest was slightly higher in the low-flow group vs normal flow (3.94 ± .94 vs 3.39 ± .94, p = .008). Mean arterial pressure did not differ between groups at rest (83 ± 8 vs 86 ± 9 mmHg, p = .20), but rose more steeply, whereas systemic vascular conductance rose much less steeply, from rest to exercise in the low-flow group. Stroke volume rose from rest to moderate exercise in the normal Q group but tended to fall in the low Q group. Conclusions: A sub-group of adolescents with POTS has a hypokinetic circulation and maintains a relatively vasoconstricted state during exercise. We speculate this group of patients has aberrant regional vasoconstriction and may be grossly volume-contracted, such that they cannot muster adequate preload to augment stroke volume, relying instead on vasoconstriction to maintain perfusion pressure to working muscle. doi:10.1016/j.autneu.2015.07.212