Cardiovascular Complications in hEDS

(Excluding POTS — see separate information page)

While hypermobile Ehlers-Danlos syndrome (hEDS) is not classified as a vascular subtype of EDS, many individuals still experience cardiovascular involvement. These complications are typically mild and non-life-threatening, but they can still meaningfully affect quality of life through symptoms such as fatigue, palpitations, dizziness, and reduced exercise tolerance. Understanding these cardiovascular features can help patients and clinicians monitor for changes, manage symptoms, and reduce unnecessary worry.

Mitral Valve Prolapse (MVP)

• What It Is

MVP is the most commonly observed structural heart issue in hEDS. It occurs when the mitral valve, which separates the left atrium and ventricle, becomes floppy or redundant due to loose connective tissue. This can allow the valve leaflets to bulge backward into the atrium during heart contraction.

• Prevalence and Cause

  • Affects 6–8% of people with hEDS in recent studies.

  • Caused by connective tissue laxity in the valve apparatus.

• Symptoms

  • Often asymptomatic (no symptoms)

  • When symptoms occur

    • Palpitations (feeling of skipped or rapid heartbeats)

    • Atypical chest discomfort

    • Fatigue or reduced stamina

    • Rarely, mild mitral regurgitation (backward flow of blood through the valve)

• Health Impact

  • Generally mild and non-progressive

  • Rarely requires surgery or leads to serious complications

• Management

  • Conservative care is standard; no surgery or major intervention is needed, only monitoring and basic treatment if symptoms occur.

  • Echocardiograms (heart ultrasounds) are performed only if symptoms are present or regurgitation is suspected.

  • Beta-blockers may help with palpitations or discomfort.

Aortic Root Dilation

• What It Is

The aortic root is the section of the aorta closest to the heart. In some individuals with hEDS, this area becomes mildly dilated (widened), likely due to weakened aortic wall connective tissue.

• Prevalence and Cause

  • Reported in 1–15% of individuals with hEDS

  • More common in males

  • Typically non-progressive and does not lead to complications in most cases

• Symptoms

  • Usually none

  • Rarely, dull chest discomfort

• Health Impact

  • The risk of aortic aneurysm or dissection (tearing) is extremely rare in hEDS

  • Important to distinguish from vascular EDS (vEDS), which is a different syndrome that carries high vascular risk

• Management

  • Follow GeneReviews and cardiology guidelines

  • Baseline echocardiogram at diagnosis

  • Repeat imaging only if

    • Dilation is observed

    • There is a family history of aortic disease

  • Avoid heavy weightlifting and high-impact sports

  • For borderline cases, medications such as beta-blockers or ARBs (angiotensin II receptor blockers) may be used

Other Valvular and Conduction Abnormalities

• What They Are

  • Minor issues in other heart valves, such as tricuspid valve insufficiency

  • Conduction abnormalities, such as

    • Premature atrial or ventricular contractions (PACs/PVCs)

    • Occasional mild arrhythmias

• Symptoms

  • Often subclinical (no symptoms)

  • When present: mild palpitations or irregular heartbeats

• Health Impact

  • Rarely cause complications

  • Most do not require treatment

• Management

  • Routine monitoring only if symptoms or findings on ECG/echocardiogram appear

  • Reassurance is often sufficient

Extra-Aortic Arterial Involvement (Rare but Serious)

• What It Is

Though rare, individuals with hEDS may experience arterial dissections (a tear in the inner wall of an artery) outside the aorta. These include

• Cervical artery dissection (CAD) — affects arteries supplying the brain

• Spontaneous coronary artery dissection (SCAD) — affects heart arteries

These events are uncommon but have been documented in hEDS and related connective tissue disorders.

• Symptoms

  • Stroke-like symptoms (sudden headache, facial droop, limb weakness)

  • Chest pain or heart attack symptoms

• Health Impact

  • Potentially life-threatening

  • Require immediate emergency care

• Management

  • Managed using standard stroke or cardiac protocols

  • hEDS may be a predisposing factor due to fragile blood vessels

Blood Pressure Instability (Outside of POTS)

This section excludes postural orthostatic tachycardia syndrome (POTS), which is addressed separately.

• What It Is

Some individuals with hEDS experience blood pressure instability unrelated to full-blown POTS. Possible causes include

• Excessive vascular compliance: blood vessels are too stretchy, making it hard to maintain pressure

• Subclinical autonomic dysfunction: mild nervous system irregularities affecting heart rate and blood pressure.

• Symptoms

• Mild orthostatic hypotension (drop in BP when standing)

• Neurally mediated syncope (reflex fainting from overstimulation or stress)

• Lightheadedness, especially with standing, heat, or prolonged activity

• Health Impact

  • May contribute to

    • Fatigue

    • Dizziness

    • Fall risk

    • Reduced exercise tolerance

  • Can exacerbate issues with coordination and proprioception

• Management

  • Increase hydration and salt intake (under provider guidance)

  • Use of compression garments (e.g., abdominal binders or thigh-high socks)

  • Medications such as fludrocortisone or midodrine for persistent symptoms

Secondary Effects on Physical Function

Even when not dangerous, cardiovascular issues in hEDS can affect daily life. These effects may be amplified by other hEDS symptoms such as joint instability, fatigue, and proprioceptive dysfunction.

• Common secondary impacts

  • Reduced aerobic capacity

  • Increased fatigue or need to rest during activity

  • Exercise avoidance due to fear of dizziness, palpitations, or injury

  • Heightened anxiety about cardiovascular symptoms

  • Potential for deconditioning, which can worsen symptoms over time

A proactive but cautious approach to physical activity, with support from physical medicine or cardiac rehab, can be valuable.

Cardiovascular Complications in hEDS

Summary

While most cardiovascular complications in hEDS are mild and manageable, they can meaningfully affect energy, stamina, and daily life. Serious complications are rare, but a baseline echocardiogram is recommended at diagnosis to check for mitral valve issues or aortic root dilation—especially in symptomatic individuals or those with a family history of aortic disease.

Understanding these symptoms within the broader context of hEDS helps avoid misdiagnosis, unnecessary anxiety, or over-testing. A multidisciplinary team, including cardiologists, primary care providers, and physical therapists, can support individuals with hEDS in safely maintaining mobility and cardiovascular health.