Renovascular Hypertension: A Rare Cardiovascular Manifestation of the Ehlers-Danlos Syndrome

The association of the Ehlers-Danlos syndrome with many cardiovascular abnormalities is well known. To our knowledge, however, renovascular hypertension due to renal arterial aneurysms has not previously been reported in patients with this disease. We describe a patient with type IV Ehlers-Danlos syndrome who had multiple systemic and bilateral renal arterial aneurysms and hypertension. The hypertension could have been related to compression of renal tissue or arteries (or both) by the large aneurysms, associated stenoses, or small peripheral renal infarctions. A review of all patients in whom the Ehlers-Danlos syndrome had been diagnosed at our institution between 1967 and 1985 revealed that 4 of the 200 patients had hypertension. Three of these patients, and probably all four, had secondary causes of hypertension: Cushing's syndrome, renal insufficiency, or renovascular hypertension (in the present case). We conclude that hypertension is rare in patients with the Ehlers-Danlos syndrome and that, if present, it most likely is secondary hypertension. A thorough search for secondary causes of hypertension should be undertaken in these patients.