Blood Pressure Classification in a 39-Year-Old Woman with Hypermobile EDS and Suspected POTS
A blood pressure of 126/86 mm Hg in this 39-year-old woman does NOT constitute hypertension and requires no antihypertensive treatment; instead, focus should be on managing her suspected POTS and the autonomic dysfunction inherent to hypermobile Ehlers-Danlos syndrome.
Blood Pressure Classification
By ACC/AHA 2017 criteria: This BP (126/86 mm Hg) falls into the "Elevated" category for systolic (120-129 mm Hg with diastolic <80 mm Hg) but crosses into Stage 1 Hypertension due to the diastolic component (80-89 mm Hg), technically classifying as Stage 1 Hypertension 1.
By ESC/ESH 2024 criteria: This BP is classified as "Normal" (120-129 mm Hg systolic and/or 80-84 mm Hg diastolic) to "High Normal" (130-139 mm Hg systolic and/or 85-89 mm Hg diastolic), specifically falling in the high-normal range due to the diastolic reading of 86 mm Hg 1.
The European classification is more appropriate here because it does not mandate pharmacological treatment at this BP level in the absence of established cardiovascular disease, diabetes, chronic kidney disease, or a 10-year CVD risk ≥10% 1.
Critical Considerations in EDS and POTS
The presence of hypermobile EDS with suspected POTS fundamentally changes the management approach:
Orthostatic intolerance is extremely common: 48.6% of hEDS patients demonstrate postural orthostatic tachycardia, 31.4% show orthostatic intolerance, and only 20% have normal tilt-table results 2.
Autonomic dysfunction is nearly universal: Widespread but mild autonomic failure is present in 90% of hEDS patients, with reduced orthostatic cerebral blood flow velocity in 79% correlating with orthostatic dizziness 3.
Orthostatic hypotension risk: While only one patient in a study of 35 hEDS adults had frank orthostatic hypotension, the autonomic dysregulation creates vulnerability to blood pressure drops with position changes 2.
Management Recommendations
Do NOT Initiate Antihypertensive Therapy
Antihypertensive medications would be contraindicated in this clinical context because lowering BP could exacerbate orthostatic symptoms, worsen cerebral hypoperfusion, and increase fall risk 1, 4.
The 2024 ESC guidelines explicitly recommend deferring BP-lowering drug treatment in patients with pre-treatment symptomatic orthostatic hypotension until BP exceeds 140/90 mm Hg 1.
Even if using ACC/AHA criteria (which would technically classify this as Stage 1 HTN), treatment would only be indicated if the patient had a 10-year ASCVD risk ≥10% or high-risk conditions—neither of which applies to a 39-year-old with EDS/POTS 1.
Confirm the Diagnosis First
Obtain out-of-office BP measurements: Home BP monitoring or 24-hour ambulatory BP monitoring is essential to exclude white-coat hypertension and assess true BP patterns, as both ACC/AHA and ESC/ESH guidelines recommend confirmation before diagnosis 1.
Measure orthostatic vital signs: Document BP and heart rate supine and after 2-5 minutes of standing to quantify orthostatic changes (≥20 mm Hg systolic or ≥10 mm Hg diastolic drop defines orthostatic hypotension; heart rate increase ≥30 bpm or to ≥120 bpm defines POTS) 1, 4.
Consider tilt-table testing: Given the high prevalence of autonomic dysfunction in hEDS (with 48.6% showing POTS and 22% showing hypocapnic cerebral hypoperfusion), formal autonomic testing may clarify the diagnosis 2, 3.
Focus on POTS and Autonomic Management
Increase fluid and salt intake: This is first-line therapy for POTS to expand intravascular volume and improve orthostatic tolerance 2, 5.
Implement compression garments: Abdominal binders and compression stockings reduce venous pooling in the lower extremities 5.
Gradual position changes and physical countermaneuvers: Teach the patient to rise slowly, perform leg crossing, and use muscle tensing to prevent orthostatic symptoms 5.
Exercise reconditioning: A structured, gradual exercise program (starting recumbent or semi-recumbent) can improve cardiovascular fitness and reduce POTS symptoms, though fatigue and muscle pain are common barriers in EDS 5, 6.
Lifestyle Measures for BP Optimization
If lifestyle modification is pursued (which is reasonable given the "high-normal" classification by European standards), focus on: maintaining healthy weight, regular physical activity as tolerated, limiting sodium to <2,300 mg/day only if truly hypertensive (but NOT in POTS where salt loading is therapeutic), limiting alcohol to ≤1 drink/day for women, and stress reduction 1.
Monitor annually: Recheck BP yearly and reassess cardiovascular risk, as the 2024 ESC guidelines recommend for patients with elevated BP 1.
Common Pitfalls to Avoid
Do not treat BP numbers in isolation: The clinical context of EDS and POTS makes this patient vulnerable to harm from BP lowering, even though the numbers might suggest treatment by some criteria 1.
Avoid medications that worsen orthostatic intolerance: Alpha-blockers, tricyclic antidepressants, and benzodiazepines significantly increase fall risk and orthostatic hypotension 4.
Do not assume single-visit office readings are accurate: Confirm with out-of-office measurements, as white-coat effect is common and can lead to inappropriate treatment 1.
Recognize that baroreflex sensitivity is altered in hEDS: These patients show significantly higher baroreflex sensitivity at rest compared to controls, indicating dysregulated rather than simply deficient autonomic function 2.