Reflex Vasovagal Syncope in Ehlers-Danlos Syndrome
Abstract
Abstract Ehlers–Danlos syndrome (EDS) is frequently associated with autonomic dysfunction, including postural orthostatic tachycardia syndrome (POTS) and orthostatic hypotension (OH). Overlapping phenotypes such as orthostatic hypotension–tachycardia syndrome (OHTS) may complicate diagnosis and management. We report two patients with EDS presenting with recurrent presyncope and palpitations. Diagnoses were confirmed with table tilt test. Pharmacological management was tailored for POTS and OHTS, low-dose beta-blockers improved POTS, whereas beta-blockers alone worsened OHTS, requiring adjunctive fludrocortisone. These cases highlight the heterogeneous autonomic phenotypes associated with EDS and underscore the diagnostic value of tilt-table testing. Accurate differentiation between POTS and mixed orthostatic syndromes is essential, as management strategies differ and inappropriate therapy may exacerbate symptoms. Learning Objective Recognize and suspect autonomic dysfunction in patients with Ehlers–Danlos syndrome (EDS). Common manifestations include orthostatic hypotension, with variants such as postural orthostatic tachycardia syndrome (POTS) and orthostatic hypotension–tachycardia syndrome (OHTS). These conditions may present with similar symptoms, including syncope, presyncope, and palpitations. Diagnosis is confirmed by head-up tilt-table testing. Management strategies differ for POTS and OHTS; therefore, accurate differentiation is essential to guide appropriate…
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