Clinical presentation and management of patients with hyperadrenergic postural orthostatic tachycardia syndrome. A single center experience

Abstract

Background:We present our single center experience of 27 patients of hyperadrenergic postural orthostatic tachycardia syndrome (POTS). Methods:In a retrospective analysis, we reviewed the charts of 300 POTS patients being followed at our autonomic center from 2003 to 2010, and found 27 patients eligible for inclusion in this study. POTS was defined as symptoms of orthostatic intolerance (of greater than six months' duration) accompanied by a heart rate increase of at least 30 bpm (or a rate that exceeds 120 bpm) that occurs in the first 10 min of upright posture or head up tilt test (HUTT) occurring in the absence of other chronic debilitating disorders. Patients were diagnosed as having the hyperadrenergic form based on an increase in their systolic blood pressure of ≥ 10 mm Hg during the HUTT (2) with concomitant tachycardia or their serum catecholamine levels (serum norepinephnrine level ≥ 600 pg/mL) upon standing. Results: Twenty seven patients, aged 39 ± 11 years, 24, (89%) of them female and 22 (82%)Caucasian were included in this study. Most of these patients were refractory to most of the first and second line treatments, and all were on multiple combinations of medications.Conclusions: Hyperadrenergic POTS should be identified and differentiated from neuropathic POTS. These patients are usually difficult to treat and there are no standardized treatment protocols known at this time for patients with hyperadrenergic POTS. (Cardiol J 2011; 18, 5: 527-531)

Clinical features of hyperadrenergic postural tachycardia syndrome in children

“…The hemodynamic responses to posture in hyperadrenergic POTS in our study were in accordance with previously published data . The baseline HR and BP had no difference between children with hyperadrenergic POTS and those with POTS alone, but the standing HR and BP were higher in hyperadrenergic POTS patients than in those with POTS alone, and the HR increment was also higher than in POTS‐alone patients.…”

Small‐fiber neuropathy with cardiac denervation in postural tachycardia syndrome

“…After a 5‐minute rest phase, head‐up tilt‐table testing at a 70° upright position within 20 seconds with an electrically driven tilt‐table was performed for 10 minutes. Hyperadrenergic POTS was defined as POTS associated with a systolic blood pressure (BP) increment ≥10 mm Hg during tilt‐table testing in the 70° position . The Valsalva maneuver (VM) was performed by asking the subjects to blow into a mouthpiece attached to an aneroid pressure gauge at a pressure of 40 mm Hg and to endure pressure for 15 seconds.…”

“…Hyperadrenergic POTS was defined as POTS associated with a systolic blood pressure (BP) increment 10 mm Hg during tilt-table testing in the 70 position. 28 The Valsalva maneuver (VM) was performed by asking the subjects to blow into a mouthpiece attached to an aneroid pressure gauge at a pressure of 40 mm Hg and to endure pressure for 15 seconds. The VM was performed with the patient supine.…”

Increased Prevalence of Postural Orthostatic Tachycardia Syndrome in Psychogenic Fever Patients

“…When subjects have β-adrenergic hyperresponsiveness, they often exhibit postural orthostatic tachycardia syndrome (POTS) [5,6]. POTS is a form of orthostatic intolerance, characterized by a heart rate (HR) increase upon standing of ≥30 beats/min or a maximum HR of ≥120 beats/min, without orthostatic hypotension [5].…”