Beyond Atherosclerosis and Fibromuscular Dysplasia: Rare Causes of Renovascular Hypertension

Abstract

Renovascular hypertension is one of the most common forms of secondary hypertension. Over 95% of cases of renovascular hypertension are due either to atherosclerosis of the main renal artery trunks or to fibromuscular dysplasia. These two causes of renal artery stenosis have been extensively discussed in recent reviews and consensus. The aim of the current article is to provide comprehensive and up-to-date information on the remaining causes. While these causes are rare or extremely rare, etiologic and differential diagnosis matters both for prognosis and management. Therefore, the clinician cannot ignore them. For didactic reasons, we have grouped these different entities into stenotic lesions (neurofibromatosis type 1 and other rare syndromes, dissection, arteritis, and segmental arterial mediolysis) often associated with aortic coarctation and other arterial abnormalities, and nonstenotic lesions, where hypertension is secondary to compression of adjacent arteries and changes in arterial pulsatility (aneurysm) or to the formation of a shunt, leading to kidney ischemia (arteriovenous fistula). Finally, thrombotic disorders of the renal artery may also be responsible for renovascular hypertension. Although thrombotic/embolic lesions do not represent primary vessel wall disease, they are characterized by frequent macrovascular involvement. In this review, we illustrate the most characteristic aspects of these different entities responsible for renovascular hypertension and discuss their prevalence, pathophysiology, clinical presentation, management, and prognosis.

Keywords: aneurysm; arteriovenous fistula; dissection; hypertension, renovascular; neurofibromatosis 1.

Figure 1.

Rare causes of renovascular hypertension in a nutshell.

Figure 2.

Rare causes of renovascular hypertension—stenotic lesions. A, A 64-y-old woman with type B aortic dissection. Axial plane shows false lumen protruding into the right renal artery causing severe static type stenosis (arrow). B, Three-dimensional reconstruction of the abdominal aorta in the same patient shows a stenosis of the right renal artery (arrow) and reduced opacification of right renal parenchyma (arrow). C, A 46-y-old woman, treated for arteritis. Computed tomographic angiography (CTA) performed during workup for difficult-to-control hypertension shows high-grade renal artery stenosis (arrow) with left kidney decreased size (arrow). D, A 62-y-old woman with moderate hypertension. Angiography, performed following noninvasive CTA diagnostic examination to plan endovascular treatment (stent), shows dissection of left renal artery (arrow). E, Angiography of an 11-y-old boy with neurofibromatosis type 1 (NF1) showing stenosis of aorta and both renal arteries (arrows). F, Three-dimensional image of the abdominal arteries depicting bilateral renal arteries stenoses (arrows) in a 12-y-old boy with NF1.

Figure 3.

Rare causes of renovascular hypertension—nonstenotic lesions. A, Female patient, 37-y-old, with moderate hypertension, screened for secondary causes of hypertension. Computed tomographic angiography (CTA) shows an arteriovenous fistula in the lower part of left kidney (arrow). B, Male patient, 35-y-old, with left flank pain at admission and worsening hypertension control. CTA performed after ultrasound screening discloses a thrombus in the abdominal branch of left renal artery (arrow), with parenchymal ischemia visible in axial scan (arrow).

Figure 4.

Abnormalities in patients with Alagille and Williams syndrome. A, Infant with Alagille syndrome showing typical facial features: a broad, prominent forehead, deep-set eyes, and a small, pointed chin (courtesy of Dr Dorota Wicher, Department of Clinical Genetics, The Children’s Memorial Health Institute, Warsaw, Poland) B, Three-dimensional image of the abdominal arteries depicting extensive collateral circulation through the Riolan arcade and marginal artery of Drummond due to celiac trunk and superior mesenteric stenoses, as well as critical right renal artery stenosis (arrow) in a 16-y-old girl with Alagille syndrome (courtesy of Dr Jędrzej Sarnecki, Department of Diagnostic Imaging, The Children’s Memorial Health Institute, Warsaw, Poland). C, Coronal multiplanar reconstruction image showing bilateral multiple renal cystic lesions in the same patient (courtesy of Dr Jędrzej Sarnecki, Department of Diagnostic Imaging, The Children’s Memorial Health Institute, Warsaw, Poland). D, Th11 butterfly vertebra in a male neonate with Alagille syndrome (courtesy of Dr Jędrzej Sarnecki, Department of Diagnostic Imaging, The Children’s Memorial Health Institute, Warsaw, Poland). E, Three-dimensional image of the thoracic aorta depicting the supravalvular aortic stenosis (arrow) in a 3-y-old boy with Williams syndrome (courtesy of Dr Jędrzej Sarnecki, Department of Diagnostic Imaging, The Children’s Memorial Health Institute, Warsaw, Poland). F, Three-dimensional image of the abdominal arteries depicting bilateral renal arteries stenoses (arrows) and celiac trunk stenosis (arrow) in a 2-y-old boy with Williams syndrome (courtesy of Dr Jędrzej Sarnecki, Department of Diagnostic Imaging, The Children’s Memorial Health Institute, Warsaw, Poland).