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hEDS Cardiac Manifestations — EECC MCQ

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EasyCongenital Heart Disease (Adult)hEDS Cardiac ManifestationsEECC

A 42-year-old man with known Ehlers-Danlos syndrome (hypermobile type, hEDS) asks about cardiac screening. His echocardiogram shows mitral valve prolapse with mild MR. Is this expected?

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Correct answer: E — Yes — mitral valve prolapse is common in hEDS (up to 30%) due to connective tissue laxity affecting the valve leaflets and annulus; regular echocardiographic surveillance is recommended, though the clinical significance is usually mild

Yes. Hypermobile EDS is a connective-tissue disorder; laxity of the mitral valve leaflets and annulus can produce mitral valve prolapse (MVP) with mild mitral regurgitation (MR). MVP is a recognised cardiovascular feature of hEDS, historically reported in up to about 30%, and when present is usually mild. This does not imply vascular EDS: arterial rupture/dissection is the hallmark concern of vascular EDS, not typical hEDS. Mild MVP/MR does not require surgery; it is managed with clinical review and periodic echocardiographic surveillance, escalating only if MR progresses, symptoms develop, LV changes occur, or aortic root dilatation is found.

Reference: GeneReviews: Hypermobile Ehlers-Danlos Syndrome. Last update 22 February 2024. https://www.ncbi.nlm.nih.gov/books/NBK1279/