Postural Orthostatic Tachycardia Syndrome

Vikki Earley, ADN

#Pots

Postural Orthostatic Tachycardia Syndrome (POTS) is a type of dysautonomia¹, in which the blood vessels do not constrict properly and the patient experiences tachycardia when shifting positions from supine or sitting to standing. POTS is not rare² and is more prevalent than multiple sclerosis. It is estimated to affect one to three million Americans, and though it affects both sexes, 80-85% of POTS patients are females between ages 15 and 50. The odds are high that you will encounter at least one POTS patient during your nursing career.

What to Watch for in POTS Patients: Common Symptoms of POTS

The primary defining symptom of POTS is orthostatic intolerance while standing upright, resulting in significant tachycardia. The adult patient's heart rate increases at least 30 bpm or is greater than 120 bpm during the first 10 minutes upon standing, with an absence of orthostatic hypotension. Tachycardia is often accompanied by a cluster of other specific symptoms, which may include:

Chest Pain
SOB
Lightheadedness
GI issues³
Migraines
Peripheral vascular pooling
Acral cyanosis
Cold sweaty extremities, I.e., "clammy" hands and feet
Cognitive impairment, I.e., "brain fog"
Exercise intolerance
Syncope

Types of POTS

POTS is classified as primary or secondary.

Primary forms of POTS are not correlated with other diseases. Secondary forms are associated with other diseases or conditions that may have contributed to the development of POTS symptoms, such as Lyme disease, autoimmune disorders, diabetes, chemotherapy, and heavy metal poisoning.

There are 4 characteristic forms of POTS and patients may present with multiple characteristics.

Neuropathic – small-fiber neuropathy affects constriction of the peripheral and abdominal vessels
Hypovolemic – abnormally low volume of blood
Partial Dysautonomic – the most common primary form, characterized by excessive blood pooling in the extremities and abdomen while upright
Hyperadrenergic – elevated levels of norepinephrine

Causes of POTS

POTS is not a disease, but a collection of disorders with similar symptoms and is a syndrome that is not fully understood. It is thought to have many causes and therefore the underlying root cause of POTS can be difficult to determine and may not be discovered for every patient. Since approximately 40% of patients living with POTS have a relative with POTS or another type of dysautonomia, some patients have a possible genetic link.

POTS-Associated Conditions and Comorbidities

There are numerous diseases and conditions associated with POTS. The following is not an all-inclusive list, but some conditions and comorbidities associated with POTS are:

Amyloidosis
Autoimmune Diseases
Antiphospholipid Syndrome
Diabetes
Lupus
Multiple Sclerosis
Sjogren's Syndrome
Sarcoidosis
Chiari Malformation
Genetic Disorders
Infections
Epstein Barr Virus
Mononucleosis
Lyme Disease
Hepatitis C
Ehlers Danlos Syndrome
Mast Cell Activation Syndrome (MCAS)

Diagnosis of POTS

Lack of awareness in the medical community and limited providers specializing in POTS can cause prolonged diagnosis for many POTS patients. The average search for a POTS diagnosis is 4 years. This delay can have a negative impact on the patient's mental health, increase physical suffering, and create financial hardships for the patient and their family. The most common diagnostic tool used to identify POTS is the tilt table test. Additional diagnostic tools to further evaluate the functionality of the patient's autonomic nervous system may be helpful, such as QSAT, TST, EKG, gastric motility studies, and skin nerve biopsies.

POTS Treatment Options, Prognosis, and Quality of Life

Although a small percentage of adolescent patients have reported recovery, POTS is a chronic condition for the majority. POTS can present as mild, moderate, or severe and 25% of POTS patients struggle to perform ADLs, attend school or work, and participate in social events. Some physicians and researchers have compared these functional limitations as equivalent to the functional limitations of patients with COPD or CHF. There is no cure and there are no FDA-approved medications for POTS, but it can be managed with a combination of multiple therapies, off-label use of pharmaceuticals, and lifestyle changes. A team of POTS-knowledgeable providers is optimal to provide an adequate treatment plan and management. Treatment plans may include:

Lying down
Compression stockings with at least 30 mm HG ankle counterpressure
Abdominal compression wear
Increased salt intake to 3 – 10 grams per day (except in hyperadrenergic form)
Increased non-caffeinated fluid intake to 2-3 liters per day
Physical reconditioning using reclined exercises (rowing, swimming, recumbent bicycling)
Healthy diet
Elevating the head of bed
Medications used off-label to improve conditions enough for exercise reconditioning such as:
Alpha-adrenergic agonists
ACE-inhibitors
Beta-blockers
Calcium channel blockers
Diuretics
EPO
Ganglionic blocking agents
MAOIs
TCAs
SSRIs
SNRIs

How Nurses Can Support POTS Patients

Many POTS patients have spent years trying to obtain a proper diagnosis and may have developed a mistrust of the medical system as a result. Unfortunately, providers have often incorrectly diagnosed POTS symptoms as stemming from an anxiety disorder. Although many patients with chronic illnesses like POTS may suffer from anxiety and depression, these are not causes of POTS. Approach POTS patients with an open mind and a listening ear to learn how you can best support the patient. Connect the patient with online and local resources. Educate the family members and caregivers about POTS and how they can best support their loved ones.