Renovascular hypertension in children

AbstractPaediatric hypertension, defined as systolic blood pressure > 95th percentile for age, sex and height is often incidentally diagnosed. Renovascular hypertension (RVH) is responsible for 5–25% of hypertension in children. Renal artery stenosis and middle aortic syndrome can both can be associated with various conditions such as fibromuscular dysplasia, Williams syndrome & Neurofibromatosis type 1. This paper discusses the approaches to diagnosis and interventional management and outcomes of renovascular hypertension in children. Angiography is considered the gold standard in establishing the diagnosis of renovascular disease in children. Angioplasty is beneficial in the majority of patients and generally repeated angioplasty is considered more appropriate than stenting. Surgical options should first be considered before placing a stent unless there is an emergent requirement. Given the established safety and success of endovascular intervention, at most institutions it remains the preferred treatment option.

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Beyond Atherosclerosis and Fibromuscular Dysplasia: Rare Causes of Renovascular Hypertension

Hypertension ◽

10.1161/hypertensionaha.121.17004 ◽

2021 ◽

Author(s):

Alexandre Persu ◽

Caitriona Canning ◽

Aleksander Prejbisz ◽

Piotr Dobrowolski ◽

Laurence Amar ◽

...

Keyword(s):

Renal Artery ◽

Renovascular Hypertension ◽

Fibromuscular Dysplasia ◽

Clinical Presentation ◽

Neurofibromatosis Type ◽

Secondary Hypertension ◽

Main Renal Artery ◽

Current Article ◽

Stenotic Lesions

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.

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Molecular genetic evaluation of pediatric renovascular hypertension due to renal artery stenosis and abdominal aortic coarctation in neurofibromatosis type 1

Human Molecular Genetics ◽

10.1093/hmg/ddab241 ◽

2021 ◽

Author(s):

Dawn M Coleman ◽

Yu Wang ◽

Min-Lee Yang ◽

Kristina L Hunker ◽

Isabelle Birt ◽

...

Keyword(s):

Renal Artery ◽

Renal Artery Stenosis ◽

Renovascular Hypertension ◽

Neurofibromatosis Type 1 ◽

Aortic Coarctation ◽

Mapk Pathway ◽

Neurofibromatosis Type ◽

Artery Stenosis ◽

Abdominal Aortic

Abstract The etiology of renal artery stenosis (RAS) and abdominal aortic coarctation (AAC) causing the midaortic syndrome (MAS), often resulting in renovascular hypertension (RVH), remains ill-defined. Neurofibromatosis type 1 (NF-1) is frequently observed in children with RVH. Consecutive pediatric patients (N = 102) presenting with RVH secondary to RAS with and without concurrent AAC were prospectively enrolled in a clinical data base, and blood, saliva and operative tissue, when available, were collected. Among the 102 children, 13 were having a concurrent clinical diagnosis of NF-1 (12.5%). Whole exome sequencing was performed for germline variant detection, and RNA-Seq analysis of NF1, MAPK pathway genes and MCP1 levels were undertaken in five NF-1 stenotic renal arteries, as well as control renal and mesenteric arteries from children with no known vasculopathy or NF-1. In 11 unrelated children with sequencing data, 11 NF1 genetic variants were identified, of which 10 had not been reported in gnomAD. Histologic analysis of NF-1 RAS specimens consistently revealed intimal thickening, disruption of the internal elastic lamina and medial thinning. Analysis of transcript expression in arterial lesions documented an approximately 5-fold reduction in NF1 expression, confirming heterozygosity, MAPK pathway activation and increased MCP1 expression. In summary, NF-1-related RVH in children is rare but often severe and progressive and, as such, important to recognize. It is associated with histologic and molecular features consistent with an aggressive adverse vascular remodeling process. Further research is necessary to define the mechanisms underlying these findings.

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Arterial Stiffness in a Toddler with Neurofibromatosis Type 1 and Refractory Hypertension

Case Reports in Pediatrics ◽

10.1155/2018/5957987 ◽

2018 ◽

Vol 2018 ◽

pp. 1-4

Author(s):

Stella Stabouli ◽

Euthymia Vargiami ◽

Olga Maliachova ◽

Nikoleta Printza ◽

John Dotis ◽

...

Keyword(s):

Blood Pressure ◽

Arterial Stiffness ◽

Renovascular Hypertension ◽

Blood Pressure Monitoring ◽

Neurofibromatosis Type ◽

Response To Treatment ◽

Common Finding ◽

Refractory Hypertension ◽

Midaortic Syndrome

Arterial hypertension is a common finding in patients with neurofibromatosis (NF) type 1. Renovascular hypertension due to renal artery stenosis or midaortic syndrome could be the underlying cause. We report the case of a 4-year-old girl with NF type 1 and midaortic syndrome whose changes in blood pressure and pulse wave velocity suggested the evolution of vasculopathy, diagnosis of renovascular hypertension, and provided insights of response to treatment. Hypertension persisted after percutaneous transluminal angioplasty in the abdominal aorta, requiring escalation of antihypertensive treatment, while arterial stiffness demonstrated a mild decrease. Regular assessment of blood pressure using ambulatory blood pressure monitoring and noninvasive assessment of arterial stiffness may enhance the medical care of patients with NF type 1.

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Revascularization in a 17-Year-Old Girl with Neurofibromatosis and Severe Hypertension Caused by Renal Artery Stenosis

Texas Heart Institute Journal ◽

10.14503/thij-15-5466 ◽

2017 ◽

Vol 44 (1) ◽

pp. 50-54

Author(s):

Carmen C. Beladan ◽

Oliviana D. Geavlete ◽

Simona Botezatu ◽

Marin Postu ◽

Bogdan A. Popescu ◽

...

Keyword(s):

Blood Pressure ◽

Renal Artery ◽

Renal Artery Stenosis ◽

Neurofibromatosis Type 1 ◽

Severe Hypertension ◽

Neurofibromatosis Type ◽

Artery Stenosis ◽

Left Renal Artery ◽

Renal Angioplasty

Renal artery stenosis caused by neurofibromatosis is a rare cause of renovascular hypertension. This hypertension can develop during childhood and is one of the leading causes of poor outcome. We report the case of a 17-year-old girl who was incidentally diagnosed with severe hypertension. During her examination for secondary hypertension, we reached a diagnosis of neurofibromatosis type 1 on the basis of a cluster of typical findings: optic nerve glioma, café au lait spots, nodular neurofibromas, and axillary freckling. Renal angiograms revealed a hemodynamically significant left renal artery stenosis (70%). Renal angioplasty with a self-expanding stent was performed one month later for rapidly progressive renal artery stenosis (90%) and uncontrolled blood pressure. Excellent blood pressure control resulted immediately and was maintained as of the 2-year follow-up evaluation. We think that percutaneous transluminal renal angioplasty can be effective in select patients who have neurofibromatosis type 1 and refractory hypertension caused by renal artery stenosis.

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RENOVASCULAR HYPERTENSION IN CHILDREN

PEDIATRICS ◽

10.1542/peds.46.3.362 ◽

1970 ◽

Vol 46 (3) ◽

pp. 362-370

Author(s):

Ernst P. Leumann ◽

Robert P. Bauer ◽

Paul E. Slaton ◽

Edward G. Biglieri ◽

Malcolm A. Holliday

Keyword(s):

Renal Artery ◽

Renal Artery Stenosis ◽

Renovascular Hypertension ◽

Pediatric Patient ◽

Renal Vein ◽

Diagnostic Problem ◽

Bilateral Renal Artery Stenosis ◽

Bilateral Renal Artery ◽

Fibromuscular Hyperplasia ◽

Hypertension In Children

Three children with renovascular hypertension are presented in order to demonstrate the wide clinical spectrum of this disease. Two patients had relatively minor symptoms, but one with neurofibromatosis showed frank hypokalemia, polyuria, and hyponatremia. Three different anatomical lesions were found: bilateral renal artery stenosis in the patient with neurofibromatosis, fibromuscular hyperplasia in the patient with stenosis of one renal artery, and an isolated malformation of one small intrarenal artery. The last of our patients presented a complicated diagnostic problem which required repeated arteriograms and renal vein catheterizations for differential renin assay. Renovascular hypertension should be excluded in any pediatric patient with otherwise unexplained hypertension.

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Ischaemic nephropathy

10.1093/med/9780199592548.003.0212 ◽

2015 ◽

Author(s):

Helen Alderson ◽

Constantina Chrysochou ◽

James Ritchie ◽

Philip A. Kalra

Keyword(s):

Blood Pressure ◽

Renal Artery ◽

Filtration Rate ◽

Blood Pressure Reduction ◽

Western World ◽

Pressure Reduction ◽

Renovascular Disease ◽

Angiotensin Blockade ◽

Atherosclerotic Renovascular Disease ◽

Common Manifestation

Ischaemic nephropathy describes loss of renal function or renal parenchyma due to stenosis or occlusion of the renal artery or its branches. In the Western world, this is usually the result of atherosclerotic renovascular disease, but other aetiologies include arteritis, embolic disease, dissection, and fibromuscular disease.Chronic kidney disease is the most common manifestation of ischaemic nephropathy, but hypertension, flash pulmonary oedema, sensitivity to angiotensin blockade, and sensitivity of glomerular filtration rate to blood pressure reduction are all possible manifestations of occlusive diseases of the renal artery or its branches. Proteinuria may also occur.This chapter describes these clinical features and the outcomes of ischaemic nephropathy. It goes on to discuss the haemodynamics and mechanisms and what we understand of the pathophysiology of the condition.

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Thromboxane A2-prostaglandin H2 and renovascular hypertension in rats

AJP Regulatory Integrative and Comparative Physiology ◽

10.1152/ajpregu.1994.267.5.r1190 ◽

1994 ◽

Vol 267 (5) ◽

pp. R1190-R1197 ◽

Cited By ~ 4

Author(s):

E. H. Boussairi ◽

J. Sacquet ◽

J. Sassard ◽

D. Benzoni

Keyword(s):

Blood Pressure ◽

Renal Artery ◽

Receptor Antagonist ◽

Renovascular Hypertension ◽

Vascular Reactivity ◽

Thromboxane A2 ◽

Malignant Hypertension ◽

Receptor Blockade ◽

Vascular Responsiveness ◽

Goldblatt Hypertension

To evaluate the contribution of thromboxane (Tx) A2-prostaglandin (PG) H2 in two-kidney, one-clip Goldblatt hypertension (GH), 26 GH rats were chronically treated (GHT) with a specific TxA2-PGH2 receptor antagonist, CGS-22652 (30 mg.kg-1.24 h-1 sc); 28 others as well as 17 sham-clipped (SC) rats received vehicle. Twelve GH and 3 GHT rats developed malignant hypertension and died. After 6 wk of treatment, GH rats exhibited higher mean blood pressure (BP; 189 +/- 3 vs. 118 +/- 2 mmHg) and an increased vascular reactivity to the main pressor agents compared with SC rats. Chronic TxA2-PGH2 receptor blockade lowered mean BP in 13 GHT rats (125 +/- 3 mmHg) and decreased their vascular reactivity compared with GH rats. However, 10 GHT rats remained hypertensive (190 +/- 9 mmHg) and differed from the former by an increased vascular reactivity to vasopressin. It is concluded that renal artery clipping induces either benign or malignant hypertension. In benign forms, TxA2-PGH2 blockade normalizes BP through decreasing the vascular responsiveness to the main pressor agents. In malignant forms, it limits the elevation of BP and markedly reduces mortality.

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