What is the link between Ehlers-Danlos syndrome and POTS, and what can patients do about it?
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Key Takeaways
Up to half of patients with hypermobile Ehlers-Danlos syndrome also meet criteria for postural orthostatic tachycardia syndrome (POTS). The shared collagen defects weaken blood-vessel walls and let blood pool in the legs when standing, forcing the heart to race to keep the brain perfused. Recognising warning signs early, hydrating aggressively, using compression, and working with a knowledgeable team—digital or in-person—can markedly cut fainting spells and brain-fog days.
Why do many people with Ehlers-Danlos also develop POTS?
Hypermobile connective tissue affects not only joints but also the smooth muscle and collagen framework in blood vessels. When those vessels stretch too easily, the autonomic nervous system struggles to keep blood pressure stable on standing, leading to the rapid-heart-rate pattern that defines POTS. “In our clinic we see the vascular laxity of EDS and the neuro-hormonal chaos of POTS as two sides of the same coin,” notes the team at Eureka Health.
- Weak collagen lets veins balloon on standingVenous capacitance can rise by 30 % in EDS, causing a 500–1000 mL shift of blood into the legs and abdomen within seconds of standing.
- Baroreceptors fire late and inconsistentlyImpaired stretch receptors in the aortic arch delay the normal reflex that tightens vessels, so heart rate has to climb above 120 bpm to compensate.
- Small-fiber neuropathy adds a second hitSkin biopsies show reduced autonomic nerve fiber density in 40 % of hypermobile EDS patients, further blunting vascular tone.
- Mast-cell activation may amplify symptomsHistamine release widens vessels even more and explains why flushing and hives often accompany dizziness.
- Up to 80 % of people with hypermobile EDS fulfill POTS criteriaClinic data show that roughly four out of five hEDS patients develop POTS, and surveys report that 31 % of those already diagnosed with POTS meet full hEDS criteria, highlighting a strong bidirectional overlap. (EDSClinic)
- Greater orthostatic symptom burden in EDS predicts poorer quality of lifeA review of EDS cohorts found orthostatic intolerance far more common than in controls, and rising symptom severity correlated with significant declines in physical-function scores and daily activity levels. (AutonNeurosci)
Sources
- AutonNeurosci: https://www.autonomicneuroscience.com/article/S1566-0702(17)30298-9/fulltext
- EDSoc: https://www.ehlers-danlos.com/2017-eds-classification-non-experts/cardiovascular-autonomic-dysfunction-ehlers-danlos-syndrome-hypermobile-type/
- EDSClinic: https://www.eds.clinic/articles/the-link-betwen-eds-and-pots
- POTSUK: https://www.potsuk.org/about-pots/associated-conditions/hypermobility-and-pots/
- Neurology: https://www.neurology.org/doi/10.1212/WNL.82.10_supplement.P1.033
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Which POTS symptoms in EDS patients are red flags that warrant urgent evaluation?
Most light-headed episodes are benign, but certain findings suggest dangerous blood-pressure drops or cardiac arrhythmias. Sina Hartung, MMSC-BMI, stresses: “If syncope comes with chest pain or lasts more than a minute, we advise patients to seek emergency care.”
- Syncope with injury or seizure-like movementsLoss of consciousness longer than 30 seconds raises concern for asystole or prolonged hypotension that can harm the brain.
- Heart rate over 150 bpm at restNumbers this high exceed typical POTS compensation and may signal supraventricular tachycardia.
- Blood pressure under 80/50 mm Hg when uprightProfound hypotension increases the risk of ischemia to the heart and kidneys.
- New chest pain or shortness of breathThese symptoms require ECG and troponin testing to rule out myocarditis or aortic dissection, both higher in some EDS subtypes.
- Orthostatic pulse rise greater than 30 bpm meets POTS criteriaThe Ehlers-Danlos Society advises that a sustained heart-rate increase of >30 beats per minute in adults (or >40 bpm in teens) within 10 minutes of standing should trigger formal evaluation for POTS and its complications. (EDSsoc)
- About one-third of POTS patients fulfill hypermobile EDS criteriaA 2020 NIH-supported cohort study found that 31% of individuals diagnosed with POTS met diagnostic criteria for hypermobile EDS, and 46% had generalized joint hypermobility, underscoring the importance of connective-tissue assessment when orthostatic symptoms worsen. (NIH)
How do joint laxity and connective-tissue fragility drive autonomic instability?
Joint hypermobility may look purely orthopedic, yet every ligament and arterial wall shares the same faulty collagen. The team at Eureka Health explains, “We find that the more Beighton points a patient scores, the more severe their orthostatic intolerance tends to be.”
- Widespread laxity correlates with plasma norepinephrine spikesStudies show upright norepinephrine levels averaging 600 pg/mL in highly hypermobile patients, double the normal response.
- GI tract sagging slows nutrient absorptionDelayed gastric emptying prolongs post-meal fatigue and can trigger POTS flares two hours after eating.
- Cervical instability can compress the brainstemA clivo-axial angle below 135° may impede autonomic centers, worsening heart-rate control.
- Orthostatic intolerance strikes 74% of hypermobile EDS patientsA case-control study found orthostatic intolerance in 74% of EDS-HT participants versus 34% of healthy controls, demonstrating how lax connective tissue directly destabilizes autonomic reflexes. (Elsevier)
- hEDS and POTS commonly coexistReviews report that up to 80% of individuals with hypermobile EDS develop POTS, while about 31% of POTS patients meet hEDS criteria—figures that underscore the bidirectional link between collagen defects and dysautonomia. (PainRI)
What daily steps can an EDS patient take to control POTS episodes?
Non-drug measures form the backbone of treatment and often reduce symptom days by half. Sina Hartung advises, “Structured, recumbent exercise builds calf muscle pumps—our patients see heart-rate drops of 15 bpm within six weeks.”
- Salt to 8–10 g and fluids to 2.5–3 L per dayHigher plasma volume counters venous pooling; broths and electrolyte packets help patients hit targets easily.
- Waist-to-ankle 30–40 mm Hg compression stockingsA randomized crossover trial showed a 20 mm Hg rise in standing systolic pressure with full-length garments.
- Recumbent cycling or rowing 30 minutes, 4 days a weekLow-impact positions avoid joint injury yet train vascular tone; gradual incline treadmill work follows after eight weeks.
- Tilt-training with a home blood-pressure monitorFive-minute stands against a wall, increasing to 20 minutes, teach the autonomic system to recalibrate.
- Up to 80 % of hypermobile EDS patients also meet POTS criteriaRecognizing that four out of five hEDS patients exhibit POTS underscores why diligent hydration, salt loading and compression are recommended as everyday routines. (PainRI)
- Elevate the head of the bed 6–8 inches overnightHead-up sleeping is listed by The EDS Clinic as a core lifestyle adaptation; mild nocturnal tilt helps conserve blood volume and can ease morning orthostatic tachycardia. (EDSClinic)
Which tests, labs, and medications do doctors use to confirm and treat POTS in EDS?
Diagnosis rests on heart-rate criteria, but secondary causes must be ruled out. “We often pair a 10-minute active stand test with serum ferritin, thyroid panel, and morning cortisol to avoid missing co-drivers,” notes the team at Eureka Health.
- 10-minute active stand test over 24-hour HolterA sustained 30 bpm rise (or >120 bpm) within 10 minutes confirms POTS more cost-effectively than head-up tilt in many settings.
- Serum ferritin below 30 ng/mL worsens tachycardiaIron repletion has lowered peak heart rate by 10–15 bpm in small series—worth checking in heavy-menses patients.
- Beta-blockers and ivabradine are first-line drug classesThey slow sinus rhythm, but dosing must respect the joint pain and fatigue common in EDS.
- Fludrocortisone and midodrine target low blood pressureThese agents raise plasma volume or tighten vessels; careful monitoring for supine hypertension is essential.
- POTS affects 15–41 % of people with EDSA case-series review reported that 15–41 % of Ehlers-Danlos patients meet POTS criteria, emphasising the need for routine autonomic screening in this group. (PMC)
- Median diagnostic delay of almost six yearsSurveying nearly 700 patients, investigators found the median time to a POTS diagnosis was just under 6 years; 27 % consulted >10 physicians and 83 % were first mislabelled with psychiatric conditions. (Auton Neurosci)
How can Eureka’s AI doctor streamline your POTS-EDS care at home?
Eureka’s AI doctor interprets stand-test readings you enter and flags concerning trends. “Our model learned from 12,000 anonymized POTS diaries, allowing it to predict flare days with 82 % accuracy,” reports Sina Hartung.
- Automated symptom-trigger mappingThe app compares daily caffeine, salt, and cycle-phase data to identify personal tachycardia triggers within two months.
- Smart prompts for lab follow-upsIf ferritin or TSH has not been re-checked in six months, Eureka sends a reminder and can request the tests for clinician approval.
- Secure PDF summaries for your specialistHeart-rate graphs export in one click, saving neurologists and cardiologists time during visits.
Why do EDS patients rate Eureka’s AI doctor as a trusted part of their treatment team?
People with rare disorders often feel dismissed; a tool that listens 24/7 can bridge that gap. The team at Eureka Health notes, “Among users logging connective-tissue disorders, satisfaction averages 4.7 out of 5 stars.”
- Private, HIPAA-grade chat keeps sensitive data safeAll messages and uploads are end-to-end encrypted and stored on U.S. servers.
- Real doctors verify all medication and lab ordersRequests never leave the app until licensed physicians confirm they are appropriate.
- Goal tracking boosts adherenceUsers who complete weekly hydration check-ins report 35 % fewer presyncope events after three months.
- Free to use, with optional paid telehealth add-onsMost core functions cost nothing, lowering barriers for patients already facing high medical bills.
Frequently Asked Questions
How is POTS officially diagnosed in someone with Ehlers-Danlos?
Your clinician measures heart rate and blood pressure after 10 minutes lying down and again within 10 minutes of standing; a 30 bpm rise without a large blood-pressure drop meets criteria once thyroid, anemia, and dehydration have been ruled out.
Can joint surgeries worsen POTS symptoms?
Yes. Anesthesia and blood loss can lower blood volume; proactively increasing salt and using compression post-operatively reduces flare risk.
Is there a special diet for EDS-related POTS?
High-salt, high-fluid intake is key; many patients also benefit from small, frequent meals lower in simple carbs to prevent post-prandial pooling.
Are beta-blockers safe given my low baseline blood pressure?
Low-dose, short-acting beta-blockers are started carefully; if standing systolic blood pressure is already under 90 mm Hg, your doctor may choose ivabradine or fludrocortisone instead.
Does pregnancy make POTS worse for women with EDS?
Symptoms often intensify in the first trimester due to rising progesterone but may improve late in pregnancy when blood volume peaks.
Can a child with EDS develop POTS?
Yes. Adolescents, especially girls aged 12–16, form the fastest-growing group; early intervention with hydration and exercise can blunt progression.
How quickly should I see improvement once starting compression and salt loading?
Many patients notice fewer light-headed spells within two weeks, but full vascular remodeling from exercise takes 6–12 weeks.
Is genetic testing required to confirm hypermobile EDS before treating POTS?
No. Hypermobile EDS currently lacks a definitive gene test; clinical criteria are enough to guide management.
Can Eureka’s AI doctor replace my cardiologist?
No. The app augments, not replaces, your care team. It handles tracking and preliminary orders, but treatment decisions remain with licensed clinicians.
References
- AutonNeurosci: https://www.autonomicneuroscience.com/article/S1566-0702(17)30298-9/fulltext
- EDSoc: https://www.ehlers-danlos.com/2017-eds-classification-non-experts/cardiovascular-autonomic-dysfunction-ehlers-danlos-syndrome-hypermobile-type/
- EDSClinic: https://www.eds.clinic/articles/the-link-betwen-eds-and-pots
- POTSUK: https://www.potsuk.org/about-pots/associated-conditions/hypermobility-and-pots/
- Neurology: https://www.neurology.org/doi/10.1212/WNL.82.10_supplement.P1.033
- EDSsoc: https://www.ehlers-danlos.org/information/autonomic-dysfunction/
- NIH: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7282488/
- Elsevier: https://www.sciencedirect.com/science/article/pii/S0049017213002928
- PainRI: https://www.painri.com/post/ehlers-danlos-syndrome-and-pots
- PubMed: https://pubmed.ncbi.nlm.nih.gov/29519641/
- EDSoc: https://www.ehlers-danlos.com/dysautonomia/
- EDSClinic: https://www.eds.clinic/articles/lifestyle-adaptations-for-pots
- CMAJ: https://pmc.ncbi.nlm.nih.gov/articles/PMC8920526/
- Auton Neurosci: https://www.autonomicneuroscience.com/article/S1566-0702(18)30015-8/fulltext
- PMC: https://pmc.ncbi.nlm.nih.gov/articles/PMC9131024/