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

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Presentation transcript:

Renovascular Hypertension: A Rare Cardiovascular Manifestation of the Ehlers-Danlos Syndrome THOMAS F. LÜSCHER, M.D. Mayo Clinic Proceedings Volume 62, Issue 3, Pages 223-229 (March 1987) DOI: 10.1016/S0025-6196(12)62447-1 Copyright © 1987 Mayo Foundation for Medical Education and Research Terms and Conditions

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 1987 62, 223-229DOI: (10.1016/S0025-6196(12)62447-1) Copyright © 1987 Mayo Foundation for Medical Education and Research Terms and Conditions

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 1987 62, 223-229DOI: (10.1016/S0025-6196(12)62447-1) Copyright © 1987 Mayo Foundation for Medical Education and Research Terms and Conditions

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 1987 62, 223-229DOI: (10.1016/S0025-6196(12)62447-1) Copyright © 1987 Mayo Foundation for Medical Education and Research Terms and Conditions

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 1987 62, 223-229DOI: (10.1016/S0025-6196(12)62447-1) Copyright © 1987 Mayo Foundation for Medical Education and Research Terms and Conditions

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 1987 62, 223-229DOI: (10.1016/S0025-6196(12)62447-1) Copyright © 1987 Mayo Foundation for Medical Education and Research Terms and Conditions