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THOMAS F. LÜSCHER, M.D.  Mayo Clinic Proceedings 

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1 Renovascular Hypertension: A Rare Cardiovascular Manifestation of the Ehlers-Danlos Syndrome 
THOMAS F. LÜSCHER, M.D.  Mayo Clinic Proceedings  Volume 62, Issue 3, Pages (March 1987) DOI: /S (12) Copyright © 1987 Mayo Foundation for Medical Education and Research Terms and Conditions

2 Fig. 1 Arteriograms (taken in 1973) in patient with Ehlers-Danlos syndrome. A, Abdominal arteriogram shows multiple renal arterial aneurysms extending into branch arteries in both kidneys. B, Arteriogram of left kidney clearly discloses renal arterial aneurysms. Mayo Clinic Proceedings  , DOI: ( /S (12) ) Copyright © 1987 Mayo Foundation for Medical Education and Research Terms and Conditions

3 Fig. 2 Data on renal venous renin activity in hypertensive patient with Ehlers-Danlos syndrome and multiple bilateral renal arterial aneurysms. Blood sampling locations, corresponding plasma renin activity (PRA), and indices used to assess activation of renin-angiotensin-aldosterone system are shown. Normal values are less than 1.5 for PRA ratio and less than 0.24 in each kidney or less than 0.48 in both kidneys for (V – A)/A ratio. Plasma renin activity in inferior vena cava (IVC) is used as arterial value. Mayo Clinic Proceedings  , DOI: ( /S (12) ) Copyright © 1987 Mayo Foundation for Medical Education and Research Terms and Conditions

4 Fig. 3 A, Follow-up arteriogram (1983) in patient with Ehlers-Danlos syndrome 10 years after first arteriogram (shown in Figure 1). Note development of infrarenal abdominal aneurysm and only minor changes in renal circulation. B, Subtraction angiogram, demonstrating contrast-filled aneurysm (arrows) and calcifications in wall of aneurysm (arrowhead) of right tibial artery, which had also developed since initial studies had been done. Mayo Clinic Proceedings  , DOI: ( /S (12) ) Copyright © 1987 Mayo Foundation for Medical Education and Research Terms and Conditions

5 Fig. 4 Paired hematoxylin-eosin (A) and elastic stain (B) photomicrographs of right common iliac artery with chronic dissection; note false lumen in right lower corner. Dissection had occurred at usual plane of outer third of media, and false lumen is lined by thick fibrocellular neointima. (Magnification, A and B x16.) Mayo Clinic Proceedings  , DOI: ( /S (12) ) Copyright © 1987 Mayo Foundation for Medical Education and Research Terms and Conditions

6 Fig. 5 Paired hematoxylin-eosin (A) and elastic stain (B) photomicrographs of boxed-in areas of Figure 4. Note mild dysplastic changes in dissected inner two thirds of media and also fibrocellular neointima that lines false lumen of arterial dissection. (Magnification, A and B ×64.) Mayo Clinic Proceedings  , DOI: ( /S (12) ) Copyright © 1987 Mayo Foundation for Medical Education and Research Terms and Conditions


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