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Review
. 2018 Jan;141(1):e20171673.
doi: 10.1542/peds.2017-1673. Epub 2017 Dec 8.

Pediatric Disorders of Orthostatic Intolerance

Julian M Stewart  1 , Jeffrey R Boris  2 , Gisela Chelimsky  3 , Phillip R Fischer  4 , John E Fortunato  5 , Blair P Grubb  6 , Geoffrey L Heyer  7 , Imad T Jarjour  8 , Marvin S Medow  9 , Mohammed T Numan  10 , Paolo T Pianosi  4 , Wolfgang Singer  4 , Sally Tarbell  11 , Thomas C Chelimsky  3 ; Pediatric Writing Group of the American Autonomic Society
Affiliations
Review

Pediatric Disorders of Orthostatic Intolerance

Julian M Stewart et al. Pediatrics. 2018 Jan.

Abstract

Orthostatic intolerance (OI), having difficulty tolerating an upright posture because of symptoms or signs that abate when returned to supine, is common in pediatrics. For example, ∼40% of people faint during their lives, half of whom faint during adolescence, and the peak age for first faint is 15 years. Because of this, we describe the most common forms of OI in pediatrics and distinguish between chronic and acute OI. These common forms of OI include initial orthostatic hypotension (which is a frequently seen benign condition in youngsters), true orthostatic hypotension (both neurogenic and nonneurogenic), vasovagal syncope, and postural tachycardia syndrome. We also describe the influences of chronic bed rest and rapid weight loss as aggravating factors and causes of OI. Presenting signs and symptoms are discussed as well as patient evaluation and testing modalities. Putative causes of OI, such as gravitational and exercise deconditioning, immune-mediated disease, mast cell activation, and central hypovolemia, are described as well as frequent comorbidities, such as joint hypermobility, anxiety, and gastrointestinal issues. The medical management of OI is considered, which includes both nonpharmacologic and pharmacologic approaches. Finally, we discuss the prognosis and long-term implications of OI and indicate future directions for research and patient management.

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Conflict of interest statement

POTENTIAL CONFLICT OF INTEREST: Dr Boris has served as a paid expert witness on the subject of postural orthostatic tachycardia syndrome. H. Lundbeck A/S (which underwrote a meeting of the Pediatric Subgroup of the American Autonomic Society) produces Northera (droxidopa) to treat orthostatic intolerance as well as citalopram and escitalopram; the other authors have indicated they have no potential conflicts of interest to disclose.

Figures

FIGURE 1
FIGURE 1
A standing test of IOH is shown. The upper panel shows HR in beats per minute, and the lower panel shows BP in mm Hg. The vertical line indicates standing. BP decreases, and HR increases briefly. Hypotension resolves within 30 seconds. HR stabilizes gradually to its upright, steady-state value.
FIGURE 2
FIGURE 2
A tilt table test shows true OH. The upper panel shows HR in beats per minute, and the lower panel shows BP in mm Hg. The vertical line indicates tilt. There is a monotonic decrease in BP and a compensatory increase in HR, which can often exceed 40 beats per minute in youngsters.
FIGURE 3
FIGURE 3
A tilt test shows VVS. The upper panel shows HR in beats per minute, and the lower panel shows BP in mm Hg. Vertical lines indicate the start and end of tilt. On tilt, BP is initially stable then slowly falls because HR rises often by >40 beats per minute. BP then falls rapidly, followed by HR (hypotension bradycardia).
FIGURE 4
FIGURE 4
A tilt test shows POTS. The upper panel shows HR in beats per minute, and the lower panel shows BP in mm Hg. Vertical lines indicate the start and end of tilt. On tilt, BP is initially stable, with a small downward drift during tilt. There is excessive tachycardia but no hypotension.
See this image and copyright information in PMC

References

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