What Is the Malmö POTS Score (MAPS)? Understanding POTS in Ehlers-Danlos Syndrome

If you have been diagnosed with — or are being assessed for — hypermobile Ehlers-Danlos Syndrome (hEDS) or Hypermobility Spectrum Disorder (HSD), there is a good chance you have heard the term POTS come up. You may have also had a practitioner mention something called the MAPS questionnaire. Both are worth understanding, because POTS is one of the most commonly missed and most impactful comorbidities in the hypermobility population.

This article explains what POTS is, why it occurs so frequently alongside hEDS and HSD, and what the Malmö POTS Score is designed to do.

Part 1: What Is POTS?

Postural Orthostatic Tachycardia Syndrome (POTS) is a condition affecting the autonomic nervous system — the part of the nervous system that regulates involuntary functions such as heart rate, blood pressure, and blood flow. Specifically, POTS involves an abnormal increase in heart rate when moving from lying or sitting to standing upright.

In a healthy person, standing up triggers a rapid automatic adjustment in heart rate and blood vessel tone to maintain blood pressure and blood flow to the brain. In POTS, this adjustment is impaired. Blood pools in the lower body, the heart rate rises sharply to compensate, and the brain may receive insufficient blood flow.

The result is a range of symptoms that can be debilitating and are often mistaken for anxiety, deconditioning, or dismissed altogether:

• Dizziness or lightheadedness on standing, particularly after prolonged lying or sitting

• Heart palpitations — a racing or irregular heartbeat when moving to upright

• Fatigue disproportionate to activity levels and poorly relieved by rest

• Brain fog — difficulty concentrating or finding words

• Nausea and gastrointestinal discomfort on standing

• Near-fainting or fainting (syncope)

• Exercise intolerance — symptoms that worsen significantly with physical exertion

Part 2: Why Is POTS So Common in hEDS and HSD?

The connection between POTS and hypermobility-related connective tissue conditions is well established in the research literature. The most widely accepted explanation involves the laxity of blood vessel walls.

Just as joint hypermobility reflects laxity in ligaments and joint capsules, the same connective tissue laxity affects the walls of veins throughout the body. Lax vein walls allow blood to pool more readily in the legs and abdomen when upright, making it harder for the body to maintain adequate blood pressure and flow to the brain.

Additional mechanisms may include:

• Autonomic nervous system dysregulation — a broader pattern of dysautonomia that appears more common in connective tissue disorders

• Small fibre neuropathy — damage to small nerve fibres regulating vascular tone, found in some studies of hEDS patients

• Deconditioning — reduced activity due to pain and fatigue worsens cardiovascular deconditioning, which further exacerbates POTS symptoms

Studies suggest that between 30% and 50% of people with hEDS may have some degree of orthostatic intolerance, with a meaningful proportion meeting criteria for POTS. This overlap is a key reason that hEDS management often benefits from a multidisciplinary team.

Part 3: What Is the Malmö POTS Score (MAPS)?

The Malmö POTS Score — abbreviated MAPS — is a validated symptom questionnaire developed and studied at Skåne University Hospital in Malmö, Sweden. It was designed to help clinicians screen for POTS without requiring cardiology equipment or a formal tilt table test.

POTS is formally diagnosed using a tilt table test or an active stand test that measures heart rate changes over time. These require specific equipment not routinely available in a physiotherapy or chiropractic clinic. The MAPS questionnaire fills this gap: it allows a practitioner to assess the probability of POTS based on a patient's reported symptoms, before deciding whether onward referral for formal testing is warranted.

How the MAPS questionnaire works

The MAPS is a self-reported questionnaire assessing the presence and severity of autonomic symptoms. Each item is scored, and the total indicates the probability of POTS.

In the validation study from Malmö, a total MAPS score of 42 or more was found to discriminate between confirmed POTS patients and healthy controls with a sensitivity of 97% and specificity of 98%. These are strong psychometric properties for a self-reported screening tool, making MAPS a useful clinical aid in the assessment of suspected dysautonomia.

What MAPS is and is not

• MAPS is a screening tool, not a diagnostic test. A high MAPS score suggests that POTS-like symptoms are present and further assessment may be warranted.

• A MAPS score does not confirm a POTS diagnosis. Formal diagnosis requires objective testing — typically an active stand test or tilt table test in an appropriate clinical setting.

• MAPS informs referral decisions. A high score may prompt referral to a cardiologist or physician with an interest in dysautonomia for further evaluation.

Part 4: What This Means at Velca

At Velca, our assessment process for people presenting with possible hEDS or HSD includes screening for autonomic symptoms. The MAPS questionnaire is one of the tools that may be used alongside the Beighton Score, the five-part hypermobility questionnaire, and a thorough symptom history.

If your MAPS score suggests autonomic symptoms warrant further investigation, our practitioners will discuss this with you and, where appropriate, support a referral to the right medical practitioner for formal evaluation.

Understanding that POTS may be part of your picture informs how the physiotherapy programme is structured — pacing strategies, how exercise is introduced, and advice about posture and positional changes are all areas where knowledge of dysautonomia significantly influences the approach.

If dizziness, fatigue, and heart rate changes on standing are part of your daily experience and have not yet been assessed in the context of your hypermobility, it is worth raising this with your practitioner at Velca.

Want to find out more? Book an appointment at Velca in Howick. Our team will assess your situation and discuss what options may be available for you.

This article is for general information purposes only. It is not a substitute for advice from a registered health practitioner. If you have concerns about your health, please consult a qualified professional.

Velca Health Centre | 3/10 Wellington St, Howick, Auckland 2014 | velca.co.nz | 022 639 2705