What is Hyperadrenergic Postural Orthostatic Tachycardia Syndrome (POTS)?

From the Guidelines

Hyperadrenergic Postural Orthostatic Tachycardia Syndrome (POTS) is a clinical syndrome characterized by a rapid heart rate and other symptoms that occur upon standing.

Key Characteristics of POTS

• Rapid Heart Rate: An increase in heart rate of ≥30 bpm during a positional change from supine to standing (or ≥40 bpm in those 12–19 years of age) 1
• Symptoms: Frequent symptoms that occur with standing, such as lightheadedness, palpitations, tremulousness, generalized weakness, blurred vision, exercise intolerance, and fatigue 1
• Absence of Orthostatic Hypotension: No significant drop in blood pressure (≥20 mm Hg reduction in systolic BP) upon standing 1

Pathophysiology and Associated Conditions

• Hyperadrenergic State: Likely mechanisms include severe deconditioning, immune-mediated processes, excessive venous pooling, and a hyperadrenergic state 1
• Associated Conditions: Often associated with recent infection or trauma, joint hypermobility syndrome, and more common in young women 1

Diagnosis and Management

• Active Standing Test or Tilt-Table Test: Used to diagnose POTS, with a heart rate increase of ≥30 bpm within 10 minutes of standing 1
• Treatment: Focuses on improving symptoms, with treatments such as increasing fluid and salt intake, wearing compression stockings, and medications to manage heart rate and blood pressure 1

From the Research

Definition and Characteristics of Hyperadrenergic Postural Orthostatic Tachycardia Syndrome (POTS)

• Hyperadrenergic POTS is a subtype of Postural Orthostatic Tachycardia Syndrome (POTS), characterized by excessive norepinephrine production or impaired reuptake, leading to sympathetic overactivity 2.
• This subtype is one of the three primary POTS phenotypes, along with neuropathic and hypovolemic POTS, each requiring tailored management strategies 2.
• Hyperadrenergic POTS is marked by an excessive increase in heart rate upon standing, often accompanied by symptoms such as light-headedness, headaches, chest pain, shortness of breath, and brain fog 3.

Pathophysiology of Hyperadrenergic POTS

• The pathophysiology of hyperadrenergic POTS involves abnormally increased sympathetic activity, leading to excessive norepinephrine production or impaired reuptake 3, 4.
• This results in sympathetic overactivity, which can be managed with beta-blockers, an effective treatment option for hyperadrenergic POTS 2.
• The hyperadrenergic state is one of the three major hypotheses about the pathophysiology of POTS, along with autoimmunity and sympathetic denervation leading to central hypovolemia and reflex tachycardia 3, 5.

Management of Hyperadrenergic POTS

• Management of hyperadrenergic POTS involves a multidisciplinary approach, including lifestyle modifications, such as increased fluid and salt intake, compression garment use, physical reconditioning, and postural training 2, 6.
• Beta-blockers are an effective treatment option for hyperadrenergic POTS, as they help to reduce sympathetic overactivity 2, 4.
• Avoidance of norepinephrine reuptake inhibitors is also important in the management of hyperadrenergic POTS 4.