Coexistent Fibromuscular Dysplasia and Atherosclerotic Renal Artery Stenosis

Renal artery stenosis is the second most common cause of secondary hypertension. Its prevalence is increasing with advancing age and has been shown to be 28% in high-risk males referred for cardiac catheterization, or 15% in an autopsy series of patients who died of a stroke.^1,2 Ninety percent of cases are due to atherosclerotic disease, with involvement of mainly the proximal renal artery in older male patients and fibromuscular dysplasia in 10% of the patients with the middle portion of the renal artery being involved, seen usually in young females.³ The combination of atherosclerotic renal artery stenosis and fibromuscular dysplasia in the same renal artery is very rare, with one case reported in the literature to the best of our knowledge.4 We report an unusual case of an elderly lady with concomitant atherosclerotic and fibromuscular dysplasia renal artery stenosis. Case Report. The patient is a 77-year-old female with a long-standing history of mitral valve prolapse, hyperlipidemia, anxiety and orthostatic hypotension due to autonomic dysfunction. She was recently diagnosed with hypertension and was treated with an angiotensin receptor blocker and a diuretic, with poor control. Further evaluation for late-onset hypertension that was difficult to control included a renal ultrasound, which showed elevated peak systolic velocity in the right renal artery at 2.2 m/second compared to 0.76 m/second on the left, suggestive of stenosis. A renal angiogram was performed to confirm the stenosis, and showed that the left renal artery had a 30–40% proximal stenosis, with no gradient across the lesion, and the right renal had a 70% ostial lesion with a 60–80 mmHg gradient. The mid-right renal artery also had a beading appearance consistent with fibromuscular dysplasia (Figure 1, top panel). The gradient was measured using the pullback technique with a 5 French JR5 diagnostic catheter. Both the fibromuscular dysplasia and the ostial portion of the renal artery were treated with percutaneous angioplasty and stenting using 6.0 mm Genesis™ stents (Cordis Endovascular, Miami, Florida). There was 0–10% stenosis at the end of the procedure. This was well tolerated, with immediate blood pressure response despite the fact that all of the patient’s blood pressure medications had been held. She was discharged from the hospital the following day. Discussion Renal artery stenosis due to atherosclerotic disease usually presents in the ostium or the proximal segment of the renal arteries. It is a disease of middle-aged men with atherosclerotic disease elsewhere in the vascular bed. Fibromuscular dysplasia is common in younger females and tends to involve the middle and distal parts of the renal artery. Due to this difference in the demographic profiles of the two disease processes, they are very rarely present in the ipsilateral renal artery in the same patient. Our patient had this very unusual pattern and it is likely that she had fibromuscular dysplasia for a long time before it was discovered on this study. However, the first presentation of an 80-year-old women with refractory hypertension due to fibromuscular dysplasia has been reported.⁵ Furthermore, it is now suggested that fibromuscular dysplasia is a progressive disorder and may not be a stable lesion as previously thought.⁶ Both atherosclerotic renal artery stenosis and fibromuscular dysplasia can now be successfully treated with percutaneous renal angioplasty and stent deployment. In various series, good success rates with long-term improvement in control of refractory hypertension and control of renal function impairment have been shown.^7–12 Our case highlights the importance of being aware of the possible concomitant existence of atherosclerotic and fibromuscular dysplasia as a cause of renal artery stenosis. We advocate the use of percutaneous angioplasty and stent placement for both these lesions in the same procedure if they are shown to be physiologically significant, as in our case, with a high pressure gradient across the lesion.

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