The Postural Tachycardia Syndrome

A Concise Guide to Diagnosis and Management

Blair P. Grubb, M.D.; Yousuf Kanjwal, M.D.; Daniel J. Kosinski, M.D.

J Cardiovasc Electrophysiol. 2006;17(1):108-112.

Introduction

During the last 20 years, there has been a tremendous growth in our knowledge of disorders that affect the autonomic nervous system. While at first these investigations centered on neurocardiogenic syncope, a subgroup of patients was identified who suffered from a similar, yet distinct, disorder manifested by postural tachycardia and exercise intolerance. This disorder is now referred to as the postural tachycardia syndrome (POTS) and encompasses a heterogenous group of disorders that share similar clinical characteristics.^[1] The aim of this brief report is to outline the clinical picture, subtypes, diagnosis, and management of POTS.

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The Grading of Orthostatic Intolerance ^*
Grade 0
Normal orthostatic tolerance
Grade I
Orthostatic symptoms are infrequent or occur only under conditions of increased orthostatic stress
Subject is able to stand >15 minutes on most occasions
Subject typically has unrestricted activities of daily living
Grade II
Orthostatic symptoms are frequent, developing at least once a week
Orthostatic symptoms commonly develop with orthostatic stress
Subject is able to stand >5 minutes on most occasions
Some limitation in activities of daily living is typical
Grade III
Orthostatic symptoms develop on most occasions and are regularly unmasked by orthostatic stresses
Subject is able to stand for >1 minute on most occasions
Patient is seriously incapacitated, being bed- or wheelchair-bound because of orthostatic intolerance
Syncope/presyncope is common if patient attempts to stand

^*Symptoms may vary with time and state of hydration and circumstances. Orthostatic stresses include prolonged standing, a meal, exertion, and head stress.

Pharmacologic Agents That May Cause or Worsen Orthostatic Intolerance
Angiotensin-converting enzyme inhibitors
Alpha receptor blockers
Calcium channel blockers
Beta blockers
Phenothiazines
Tricyclic antidepressants
Bromocriptine
Ethanol
Opiates
Diuretics
Hydralazine
Ganglionic-blocking agents
Nitrates
Sildenafil citrate
MAO inhibitors

MAO, monoamine oxidase.

Therapeutic Options in POTS
Treatment	Application	Form Effective in	Problems
Reconditioning	Aerobic exercise 20 min 3 times/week	PD, H	If too vigorously done may worsen symptoms
Hydration	2 liters po/day	PD	Edema
Salt	2-4 grams/day	PD	Edema
Fludrocortisone	0.1-0.2 mg po q day	PD	Hypokalemia, hypomagnesemia, edema
Midodrine	5-10 mg po tid	PD	Nausea, scalp itching, supine hypertension
Methylphenidate	5-10 mg po tid	PD	Anorexia, insomnia, dependency
Bupropion	150-300 mg XL/q day	PD, H	Tremor, agitation, insomnia
SSRI-Escitaloprim	10 mg po/day	PD, H	Tremor, agitation, sexual problems
Pyridostigmine	30-60 mg po bid	PD	Nausea, diarrhea
Erythropoietin	10,000-20,000 i.v. sq q week	PD	Pain at injection site, expensive
Octreotide	50-200 micrograms sq tid	PD	Nausea, diarrhea, gallstones
Clonidine	0.1-0.3 mg po bid 0.1-0.3 mg patch q week	H	Dry mouth, blurred vision
Labetalol	100-200 mg po bid	H	Fatigue