Pathogenesis of hyperadrenergic orthostatic hypotension. Evidence of disordered venous innervation exclusively in the lower limbs.

Streeten, David H. P.

doi:10.1172/jci114878

Cited by 166 publications

(92 citation statements)

References 19 publications

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“…On the one hand, these results can be interpreted as being consistent with hypotheses regarding the origins of POTS involving distal extremity denervation, or the so-called "long tract syndrome" (4,19), in which peripheral nerve deficit is confined to the legs. On the other hand, our results suggest a more widespread loss of vasoconstrictor tone involving both arm and leg.…”

Section: Discussionsupporting

confidence: 84%

Inappropriate Early Hypotension in Adolescents: A Form of Chronic Orthostatic Intolerance with Defective Dependent Vasoconstriction

Stewart

Weldon

2001

Pediatr Res

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Instantaneous orthostatic hypotension (INOH) has been reported in children and adolescents as a new entity of orthostatic intolerance in children who underwent rapid standing as an orthostatic stress test. Children with INOH were discovered among patients presenting with symptoms of chronic orthostatic intolerance, which is often related to orthostatic tachycardia. We used head-up tilt table testing at 70°to investigate children presenting with symptoms of chronic orthostatic intolerance. We compared 24 patients aged 12-17 y, with chronic orthostatic intolerance and symptoms for Ն3 mo, with 13 healthy normal control patients. We recorded continuous heart rate, blood pressure, and respiratory rate and used venous occlusion strain gauge plethysmography to measure calf and forearm blood flow while supine and calf blood flow during head-up tilt. Patients with chronic orthostatic intolerance fulfilled criteria for the postural orthostatic tachycardia syndrome. Postural orthostatic tachycardia syndrome patients were divided into two groups by the occurrence of INOH. Supine forearm and calf arterial resistance was decreased in patients with INOH (n ϭ 8) compared with postural orthostatic tachycardia syndrome patients without INOH (n ϭ 16) and compared with control (n ϭ 13). Resting calf venous pressure was elevated, suggesting excess venous filling because of vasodilation. During early head-up tilt, calf blood flow increased markedly in INOH, less in No-INOH, postural orthostatic tachycardia syndrome patients and least in control patients. Flow was temporally related to calf swelling and negatively correlated to hypotension. The data suggest that INOH occurs in patients with chronic orthostatic intolerance and orthostatic tachycardia and is related to rapid caudal blood flow when upright because of a vasoconstrictor defect. INOH has recently been reported as a new entity of orthostatic intolerance in children and adolescents who underwent rapid standing as an orthostatic stress test (1, 2). Data from that original study suggested that some patients developed systolic hypotension whereas others did not. Upright tachycardia was a common feature of all patients. Orthostatic tachycardia with COI has been described at least since 1940 (3) and more recently by investigators designating the syndrome POTS (for postural orthostatic tachycardia syndrome) (4 -6) or COI (7,8) in adults. We and others have described the syndrome in children and adolescents (6, 9 -12), often in the context of the chronic fatigue syndrome (12). We suspect that vascular resistance is lower than healthy control subjects in many COI-POTS patients. We hypothesized that abnormally increased blood flow to the lower extremities in COI-POTS patients during orthostatic challenge related to reduced lower limb resistance causes a precipitous early fall in blood pressureergo INOH. To investigate this hypothesis we used venous occlusion plethysmography to study blood flow supine and during HUT, comparing 24 patients with COI-POTS with 13 healthy control patients....

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Section: Discussionsupporting

confidence: 84%

Inappropriate Early Hypotension in Adolescents: A Form of Chronic Orthostatic Intolerance with Defective Dependent Vasoconstriction

Stewart

Weldon

2001

Pediatr Res

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“…Venous pooling occurs in the lower limbs as the heart rate increases during the HUTT. 6,7,11 Because similar mechanisms are involved in vitamin B 12 deficiency, we believe that patients with CFS, POTS, and syncope should be screened for vitamin B 12 deficiency.…”

Section: Discussionmentioning

confidence: 99%

“…9,10 Neurally mediated hypotension has been shown to be the most common cause of unexplained recurrent syncope, and overlapping findings have been found in CFS and neurally mediated hypotension. 9 When defining hyperadrenergic orthostatic hypotension with increased plasma levels of epinephrine and norepinephrine, Streeten 11 found that orthostatic hypotension developed due to the accumulation of blood resulting from anatomic or functional postganglionic denervation in the lower-limb veins.…”

mentioning

confidence: 99%

Postural Orthostatic Tachycardia Syndrome (POTS) and Vitamin B12 Deficiency in Adolescents

et al. 2014

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OBJECTIVE: Vitamin B12 is involved in the production of adrenaline from noradrenaline. It is the cofactor involved in catecholamine degradation and plays a role in myelin synthesis. The current study aimed to investigate the association between vitamin B12 levels and postural orthostatic tachycardia syndrome (POTS) during adolescence when accelerated myelin synthesis increases the vitamin B12 need. METHODS: One hundred twenty-five patients (mean age 11.1 ± 2.3 years; 60% female) reporting short-term loss of consciousness and diagnosed with vasovagal syncope based on anamnesis with a normal distribution and 50 control subjects (mean age 10.94 ± 2.5 years, 62% female) were included in this study. Serum vitamin B12, folic acid, and ferritin levels were measured prospectively in addition to other tests. We defined vitamin B12 deficiency as a serum level <300 pg/mL.1–4 RESULTS: Vitamin B12 levels were significantly lower in the patient group compared with the control group (47.2% vs 18%, P < .001). In the patient group, children with the POTS pattern had significantly lower vitamin B12 levels compared with children without the POTS response (P = .03). CONCLUSIONS: Vitamin B12 deficiency in patients with POTS may lead to sympathetic nervous system baroreceptor dysfunction.

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“…This compensatory mechanismwasnot seen in the patient. Although Streeten and Scullard stressed that the disordered venous innervation exists exclusively in the lower limbs in patients with hyperadrenergic orthostatic hypotension (6,9), the present case could have generalized adrenergic denervation because the decreased sweat response to iontophoresis of acetylcholine was seen in both upper limbs and lower limbs. Another characteristic finding of this patient was an excessive blood pressure elevation in the phase IV of the Valsalva maneuver.…”

mentioning

confidence: 53%

Mechanisms of Postural Tachycardia Syndrome

Inoue

2001

Intern. Med.

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Pathogenesis of hyperadrenergic orthostatic hypotension. Evidence of disordered venous innervation exclusively in the lower limbs.

Abstract: The pathogenesis of hyperadrenergic orthostatic hypotension was studied in eight patients. Correction of the abnormal orthostatic changes by an inflated pressure suit (MAST) confirmed previous evidence of excessive gravitational

Cited by 166 publications

References 19 publications

Inappropriate Early Hypotension in Adolescents: A Form of Chronic Orthostatic Intolerance with Defective Dependent Vasoconstriction

Inappropriate Early Hypotension in Adolescents: A Form of Chronic Orthostatic Intolerance with Defective Dependent Vasoconstriction

Postural Orthostatic Tachycardia Syndrome (POTS) and Vitamin B12 Deficiency in Adolescents

Mechanisms of Postural Tachycardia Syndrome

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