The Connection Between HEDS and POTS & Exploring the Three Types of POTS

Postural Orthostatic Tachycardia Syndrome (POTS) is a condition that affects many people with Hypermobile Ehlers-Danlos Syndrome (HEDS). If you or someone you know has HEDS, understanding why POTS is so common in this group and learning about the different types of POTS can help manage symptoms and improve quality of life.

Diagram illustrating the autonomic nervous system involved in POTS

Why POTS Is Common in People with HEDS

HEDS is a connective tissue disorder characterized by joint hypermobility, skin elasticity, and tissue fragility. These features affect the body's structural support and can influence how the autonomic nervous system functions. The autonomic nervous system controls involuntary functions like heart rate and blood pressure, which are central to POTS.

People with HEDS often have weaker connective tissue in blood vessels. This weakness can cause blood vessels to stretch more than usual, leading to blood pooling in the lower body when standing. The body reacts by increasing heart rate to maintain blood flow to the brain, which is a hallmark of POTS.

Additionally, the nervous system in HEDS patients may not regulate blood pressure and heart rate effectively. This dysregulation contributes to the symptoms of POTS, such as dizziness, rapid heartbeat, fatigue, and fainting.

Key reasons POTS is common in HEDS:

• Vascular laxity: Weaker blood vessel walls allow blood to pool.

• Autonomic nervous system dysfunction: Poor regulation of heart rate and blood pressure.

• Joint hypermobility: Can affect nerve function and circulation.

• Chronic fatigue and pain: Common in HEDS, these symptoms overlap with POTS, making diagnosis more frequent.

  
  

Understanding this connection helps doctors and patients recognize symptoms early and tailor treatment plans.

What Is POTS?

POTS is a form of dysautonomia, a disorder of the autonomic nervous system. It causes an abnormal increase in heart rate when moving from lying down to standing up. This rapid heart rate increase often comes with symptoms like lightheadedness, palpitations, weakness, and sometimes fainting.

POTS affects about 1 to 3 million people in the United States, with many cases linked to connective tissue disorders like HEDS. The condition can vary widely in severity and symptoms, which is why identifying the type of POTS is important for effective management.

The Three Types of POTS

POTS is not a one-size-fits-all diagnosis. It has three main types, each with different causes and treatment approaches. Knowing the type can help patients and healthcare providers choose the best strategies to reduce symptoms.

1. Neuropathic POTS

Neuropathic POTS results from damage or dysfunction in the small nerve fibers that control blood vessel constriction in the legs and abdomen. When these nerves don’t work properly, blood vessels fail to tighten enough when standing, causing blood to pool in the lower body.

Symptoms and features:

• Cold or discolored feet and hands due to poor circulation

• Numbness or tingling in the legs

• Fatigue and dizziness on standing

• Often linked to autoimmune conditions or nerve damage

  
  

Management tips:

• Compression stockings to improve blood flow

• Medications to support nerve function

• Physical therapy to improve circulation

  
  

2. Hyperadrenergic POTS

This type involves an overactive sympathetic nervous system, which controls the "fight or flight" response. People with hyperadrenergic POTS have high levels of norepinephrine (a stress hormone) when standing, causing excessive heart rate increase and high blood pressure.

Symptoms and features:

• Tremors or shaking

• Anxiety or panic-like feelings

• Headaches and sweating

• High blood pressure when upright

  
  

Management tips:

• Beta blockers to reduce heart rate and tremors

• Stress management techniques

• Avoiding triggers like caffeine or stress

  
  

3. Hypovolemic POTS

Hypovolemic POTS is caused by low blood volume. When the body does not have enough blood circulating, the heart beats faster to compensate, especially when standing.

Symptoms and features:

• Low blood pressure

• Fatigue and weakness

• Frequent dizziness or fainting

• Often linked to dehydration or blood loss

  
  

Management tips:

• Increasing fluid and salt intake

• Medications to retain fluid

• Exercise programs to improve blood volume

  
  

How Understanding POTS Types Helps People with HEDS

Since POTS is common in HEDS, knowing the type of POTS can guide treatment. For example, someone with neuropathic POTS may benefit more from compression garments, while someone with hyperadrenergic POTS might need medications to calm the nervous system.

Doctors often use tests like tilt table testing, blood volume measurements, and blood tests to identify the POTS type. This personalized approach improves symptom control and quality of life.

Living with both HEDS and POTS can be challenging, but some practical steps can help:

• Stay hydrated: Drink plenty of fluids and increase salt intake if advised.

• Wear compression stockings: These help prevent blood pooling.

• Exercise regularly: Low-impact activities like swimming or recumbent biking improve circulation.

• Avoid triggers: Heat, prolonged standing, and stress can worsen symptoms.

• Work with healthcare providers: Regular check-ups and tailored treatment plans are essential.