Fibromuscular dysplasia of the renal artery: Management and outcome

SUMMARY: Renovascular hypertension may be caused by atherosclerotic disease or less commonly by fibromuscular dysplasia (FMD) of the renal arteries. Fibromuscular dysplasia is the commonest cause of renal artery stenosis in the younger age group and affects women predominantly. A review of our clinical database identified all patients with renovascular hypertension. All relevant clinical, biochemical and radiological findings on those with FMD were noted. the outcome of percutaneous transluminal renal angioplasty (PTRA) or reconstructive surgery was evaluated. Eight out of 62 (13%) patients with hypertension secondary to renovascular disease had FMD (all female; bilateral in four; mean age at diagnosis 37.6 years; age range 12–70 years). the mean duration of hypertension before the diagnosis of FMD was 3.3 years (range 3 months-10 years). A renal artery bruit was detected in five, hypertensive retinopathy in three and one had mild renal insufficiency. Twelve PTRAs were attempted on 10 stenotic lesions in six women. This cured the hypertension in three, while the other three have required less antihypertensive therapy. Percutaneous transluminal renal angioplasty was complicated by a trivial renal artery dissection in one, and a small upper pole infarction in another. One patient required a repeat PTRA. the other two women presented before the availability of PTRA and had successful reconstructive surgery. Fibromuscular dysplasia was the cause of hypertension in eight out of 62 (13%) patients with renovascular hypertension. Percutaneous transluminal renal angioplasty has shown encouraging results with a low complication rate. If technically feasible, PTRA should be attempted on all patients with FMD of the renal artery.     

Fibromuscular dysplasia of the renal artery: Management and outcome

Renovascular hypertension may be caused by atherosclerotic disease or less commonly by fibromuscular dysplasia (FMD) of the renal arteries. Fibromuscular dysplasia is the commonest cause of renal artery stenosis in the younger age group and affects women predominantly. A review of our clinical database identified all patients with renovascular hypertension. All relevant clinical, biochemical and radiological findings on those with FMD were noted. The outcome of percutaneous transluminal renal angioplasty (PTRA) or reconstructive surgery was evaluated. Eight out of 62 (13%) patients with hypertension secondary to renovascular disease had FMD (all female; bilateral in four; mean age at diagnosis 37.6 years; age range 12–70 years). The mean duration of hypertension before the diagnosis of FMD was 3.3 years (range 3 months–10 years). A renal artery bruit was detected in five, hypertensive retinopathy in three and one had mild renal insufficiency. Twelve PTRAs were attempted on 10 stenotic lesions in six women. This cured the hypertension in three, while the other three have required less antihypertensive therapy. Percutaneous transluminal renal angioplasty was complicated by a trivial renal artery dissection in one, and a small upper pole infarction in another. One patient required a repeat PTRA. The other two women presented before the availability of PTRA and had successful reconstructive surgery. Fibromuscular dysplasia was the cause of hypertension in eight out of 62 (13%) patients with renovascular hypertension. Percutaneous transluminal renal angioplasty has shown encouraging results with a low complication rate. If technically feasible, PTRA should be attempted on all patients with FMD of the renal artery.     

The results of treatment in renal artery stenosis due to Takayasu disease: comparison between surgery,angioplasty, and stenting. A monocentrique retrospective study

Purpose

To evaluate retrospectively and compare the long-term patency and the antihypertensive effect of open surgery, angioplasty, and stent insertion of the renal artery stenosis due to Takayasu’s arteritis.

Patients and methods

We retrospectively analyzed and compared the effects on blood pressure and permeability of the renal artery over 23 patients (age ranging from 16 to 60 years, mean 33.9 years); with renovascular hypertension caused by Takayasu’s arteritis. those patients underwent surgical treatment (11 arteries) or endovascular (19) including angioplasty (11) and stenting (8) for 30 stenotic renal arteries.

Results

Technical success was 96.7% (29/30) without major complications (but longer period of hospitalization among patients who had surgery). In the last follow-up CT angiography (mean 60 ± 36 months), restenosis was 18.2% (2/11) in the surgery, 9% (1/11) in the angioplasty, and 62.5% (5/8) in the stenting. Rate of the permeability of the surgery was 100%, 90.9%, 81.8%, the permeability of the angioplasty was 100%, 90.9%, 90.9%, primary patency rate stenting was 62.5%, 37.5%, 37.5%, assists permeability was 87.5%, 75%, 50% at 1, 3 and 5 years, respectively.In the clinical follow-up (mean 60 ± 37.8 months, range 48–96 months) beneficial effects on blood pressure were achieved into 91.3% of patients (21/23), and there was no significant difference between patients who have been treated by surgery and angioplasty alone and the patients who received a stent in at least one renal artery.

Conclusion

Angioplasty has shown better long-term patency and a similar clinical benefit of renovascular hypertension in renal artery stenosis caused by Takayasu’s arteritis compared with the surgery and the stenting. We suggest that stenting should be reserved in case of clear failure of the angioplasty. The surgery is our choice for patients who do not meet the indication of endovascular treatment or failure of this treatment.     

Percutaneous transluminal renal angioplasty

Summary Percutaneous transluminal renal angioplasty (PTRA) is a simple, efficient procedure with a low risk/benefit ratio when carried out by experienced interventional radiologists. The role of radiology in the diagnosis and treatment of renovascular hypertension (RVH) is discussed, with special attention to technique. PTRA and surgical results are dependent on the etiology and location of renal artery stenosis (RAS). At New York Hospital-Cornell Medical Center, PTRA is the procedure of choice in fibromuscular dysplasia (FMD), unilateral non-ostial atheroma, arteritis, renal transplantation, and pediatrics. The choice between surgery and PTRA in patients with bilateral and ostial atherosclerotic disease and/or azotemia depends on the surgical risk category of the patient. At our institution, coronary and cerebrovascular disease do not preclude PTRA.     

Long-term results after percutaneous transluminal angioplasty of atherosclerotic renal artery stenosis--the importance of intensive follow-up.

The aim of this study was to investigate the long-term results of percutaneous transluminal angioplasty of atherosclerotic renal artery stenosis (PTRA) in patients with renovascular hypertension with or without impending renal insufficiency who were followed up intensively with aggressive reintervention. Diagnostic work-up was based on angiography, pressure gradient and renal venous renin measurement. Patients were scheduled for regular follow-up after the PTRA and a deterioration in blood pressure or renal function was an indication for re-evaluation, and reintervention if necessary. Sixty-five patients had 71 renal artery stenoses where PTRA was attempted. It was technically successful in 59 stenoses and two occlusions and failed in ten (14%). At the end of follow-up (median 56 months [2-99]), the primary patency rate was 55%, 27 had restenosed and four were occluded, all but two within 12 months. Seventeen were treated by a further PTRA and eight by surgical reconstruction. At the end of follow-up the secondary patency after all interventions was 90%. One patient died 1 month after PTRA, and at the end of follow-up 21 patients (32%) had died, most of them (80%) from cardiovascular disease. Multivariate analyses showed a significantly reduced survival rate in patients with multiocular atherosclerosis, renal insufficiency, contralateral renal artery stenosis and ischaemic heart disease. At the end of follow-up 90% of the patients were cured or improved with regard to blood pressure. In patients with impending renal insufficiency renal function was improved in 50% and unchanged in 39%. With this strategy 55% of the patients needed only one treatment with PTRA, 25% needed a re-PTRA and 20% had to be operated on. PTRA can be recommended as initial treatment of atherosclerotic renal artery stenosis provided intensive follow-up and aggressive reintervention are performed when indicated.     

Is incidental renal arteriography justified in a population of patients with symptomatic peripheral arterial disease?

Renal artery stenosis is a consequence of generalized atherosclerosis and many specialists perform routine selective renal angiography to detect and treat renal artery stenosis. The incidence of clinically important renal artery stenosis is not well defined in patients with symptomatic peripheral arterial disease. The purpose of this study was to better delineate the incidence of and the risk factors associated with renal artery stenosis, renovascular hypertension, and ischemic nephropathy incidentally discovered during angiography for symptomatic peripheral arterial disease. Two hundred consecutive patients undergoing angiographic evaluation of symptomatic lower extremity peripheral arterial disease were studied retrospectively. Angiograms were reviewed for the presence of renal artery stenosis (defined as >or= 25% diameter reduction in either renal artery) and findings were then correlated to the clinical diagnosis of renovascular hypertension (> 50% renal artery stenosis and >or= 3-drug resistive hypertension) and ischemic nephropathy (defined as > 50% bilateral renal artery stenosis, 3-drug hypertension, and creatinine >or= 1.5). Angiographic findings were also correlated with risk factors to determine if a relationship correlated to the presence of and degree of renal artery stenosis. Data were analyzed using the Student's t test, Chi-square model, and multiple logistic regression analysis. The overall incidence of any degree of renal artery stenosis in this study population was 26% (52 patients). Only 24 (12%) patients had an incidental finding of >or= 50% stenosis in either renal artery. Six (3%) of these patients were found to have associated renovascular hypertension. Additionally, 9 (4.5%) patients had coexistent renal insufficiency and significant renal artery stenosis; five with end-stage renal disease on chronic hemodialysis. Only one patient with end-stage renal disease had poorly controlled 3-drug hypertension. Thus definitive ischemic nephropathy was present in only one (0.5%) patient. Statistically significant risk factors associated with the presence of renal artery stenosis include hypertension (P < .001), coronary disease (P = .024), female gender (P = .010), diabetes (P = .039), aorto-iliac disease (P = .031), multiple levels of peripheral arterial disease (P < .001), and age over 60 ( P < .001). While the incidence of renal artery stenosis in patients being evaluated for symptomatic peripheral arterial disease is similar to that reported in the cardiology literature, the incidence of renovascular hypertension and ischemic nephropathy is exceedingly low (3% and 0.5%, respectively)-findings similar to data reported in the general hypertensive population. These data suggest that incidental selective renal angiography is not justified in patients with symptomatic peripheral arterial disease.     

肾动脉狭窄的腔内治疗经验

目的探讨腔内治疗肾动脉狭窄的临床疗效及影响因素。方法2003年2月至2005年6月共收治19例肾动脉狭窄患者,分析其中行腔内治疗12例的临床资料。其中动脉硬化9例,多发性大动脉炎1例,纤维肌性发育不良2例。结果本组12例,均有严重高血压,1例肾功能异常。支架置入10例,技术成功率91．7％(11／12)。随访12例,随访时间3—15个月,随访平均6．5个月,患者血压从(172±26／98±15)降至(156±22／88±14)mmHg。高血压治愈2例,改善6例,未愈4例,高血压控制率为66．7％。术后肾功能无明显变化。无并发症和死亡病例。再狭窄1例,再狭窄率为8．3％。结论严格掌握手术适应证,腔内治疗肾动脉狭窄是安全、有效的。     

Two cases of atherosclerotic renal artery stenosis treated by percutaneous transluminal renal angioplasty and intravascular stent placement, leading to improvement of hypertension and renal function

We describe here two cases of renal artery stenosis(RAS) caused by atherosclerosis. Both patients were treated by percutaneous transluminal renal angioplasty(PTRA) and stent placement, leading to the improvement of renal function as well as hypertension. The two patients were a 75-year-old male(case 1) and a 56-year-old male(case 2), who both showed mild proteinuria, renal dysfunction, and refractory hypertension. Case 1 showed a unilateral ostial stenosis in the left main renal artery. On the other hand, case 2 showed an ostial stenosis in the left renal artery and a widespread narrowing in the right renal artery. After evaluation of the lesions by renal Doppler sonography, renogram, magnetic resonance signal intensity, and magnetic resonance angiography(MRA), each stenosis was treated by balloon angioplasty and intravascular stent placement without any major complications. Thereafter, in addition to hypertension, renal function also ameliorated significantly, and has remained stable for more than 12 months. Non-invasive screening tests and appropriate therapy for renovascular lesion should be considered in the case of elderly patients with refractory hypertension and progressive renal dysfunction, since ischemic nephropathy is increasing as a common cause of end stage renal disease and is showing favorable outcomes of revascularization.     

Renal artery intervention in pediatric and adolescent patients: a 20-year experience

Between 1986 and 2005, 22 patients (mean age, 14.7 years; range, 3-21) with renal hypertension underwent renal artery revascularization for 28 lesions, 23 with open repair (OR) and 5 with percutaneous transluminal renal angioplasty (PTRA). Thirty-day morbidity was 17% (4/23). Hypertension was cured in 13 (57%), improved in 8 (38%), and unchanged in 1 (5%). Renal function worsened in 1. At a mean follow-up of 4.9 years, 1-year patency rate was 94% and maintained for 5 years. Hypertension at 1 year was cured in 6 of 14 patients (43%; OR, 4; PTRA, 2) and improved in 8 (57%; OR, 7; PTRA, 1); hypertension at 5 years was cured in 50% and improved in 50%. Renal function remained unchanged in all patients who were followed. The authors conclude that both OR and PTRA benefit pediatric patients. PTRA for selected patients may be promising as a first line treatment or as a bridge to definitive OR in children with small arteries.     

Takayasu's arteritis with renovascular hypertension: results of surgical treatment.

Renal artery stenosis caused by Takayasu's arteritis is an important cause of hypertension in young patients in the Far East. The role of surgery in Takayasu's arteritis is not as well-defined as in atherosclerosis or fibromuscular dysplasia. In this retrospective review, the author reports the results of 19 renal artery reconstructions in 12 young patients (median age 23.5 years, range 10-46 years) presenting with renovascular hypertension and Takayasu's arteritis, and discusses the different surgical options. The procedures performed included aortorenal bypass using vein (five), aortorenal bypass using polytetrafluoroethylene (PTFE) (eight), iliorenal bypass using vein (four), reimplantation of renal artery (one) and aortic replacement graft-renal bypass (one). Postoperatively, all 12 patients had a successful outcome with improved hypertension. There was no perioperative mortality, and complications included two early graft thrombosis and one late graft occlusion. These results support the view that surgical treatment for renovascular hypertension in Takaysu's disease is safe and effective.     

Percutaneous Balloon Angioplasty of Renovascular Hypertension in Pediatric Cases

Purpose : To investigate effectiveness of percutaneous transluminal renal angioplasty (PTRA) in treatment of renal artery stenoses due to Takayasu arteritis (TA), fibromuscular dysplasia (FMD) and neurofibromatosis (NF). Materials-methods : Between December 1991-October 2006, 20 pediatric cases (age range 3–18 years, meanly 12.5 year) with at least 60% stenoses were underwent PTRA. Main presenting symptom was hypertension. The aetiologies were TA in 12, and FMD in 5, NF in three cases.

Results : Totally, 46 procedures were performed on 35 stenotic segments of 20 cases. A technically good result was obtained in 21/24 arteries of TA patients, 7/7 arteries of FMD patients, and 1/3 arteries of NF1 patients. Clinical follow-up for a mean of 55.7 months revealed 100% success in all cases. Delayed clinical response was seen in two cases with Nf1. Restenosis was detected 5/12 of TA patients, 1/5 of FMD patients and 1/3 NF1 patients.

Conclusion : PTRA can be safely performed resulting in excellent angiographic and clinical results. It should be first choice in renal artery stenosis in pediatric age when medical treatment fails to control renovascular hypertension or renal function is decreasing although initial results might be not encouraging in NF1.     

Treatment of renal artery fibromuscular dysplasia with balloon angioplasty: a prospective follow-up study.

OBJECTIVES: to assess restenosis rates and blood pressure response after percutaneous transluminal renal angioplasty (PTRA) in patients treated for fibromuscular dysplastic renal artery stenosis. METHODS: a prospective 12-month follow-up study of 27 patients with 31 treated renal artery stenosis. Follow-up assessment included colour-coded duplex sonography (CCD) of renal arteries, monitoring of blood pressure, antihypertensive medication, and creatinine measurements before discharge and at 3, 6, and 12 months. Primary end point was defined as a haemodynamically significant restenosis >60% assessed by CCD. RESULTS: there was a cumulative 23% restenosis rate at 12 months. Arterial hypertension was cured or improved in 93% of patients immediately after the intervention and remained cured/improved in 74% of patients at 12 months of follow-up. Renal failure present in five patients before PTRA stabilised or improved in all patients. CONCLUSION: although restenosis rate after PTRA in fibromuscular dysplasia is as high as in non-ostial atherosclerotic lesions, there remains a considerable higher therapeutic effect. Profound pressure response and recurrent arterial hypertension with restenosis support the high probability of a renovascular origin of arterial hypertension in this young and otherwise healthy population compared to patients with atherosclerotic renal artery lesions.     

Surgical treatment of renovascular hypertension in the pediatric patient

Of 27 children who underwent surgical treatment for renovascular hypertension 16 (59 per cent) were cured, 5 (19 per cent) were improved and 6 (22 per cent) were failures. Improved results were obtained in patients with unilateral renal artery stenosis. Several reconstructive vascular procedures were used, and their relative efficacy and indications are discussed. Renovascular hypertension in children is a curable disease and revascularization with preservation of renal parenchyma should be the aim of surgical therapy in most cases.     

Renovascular disease in children and adolescents

PURPOSE: This retrospective review describes the surgical management of renovascular disease in 25 consecutive children and adolescents with severe hypertension. METHODS: Patients 95 th percentile systolic or diastolic pressure adjusted for gender, age, and height). RA repair comprised 25 bypasses (73%) consisting of 28% saphenous vein, 60% hypogastric artery, and 12% polytetrafluoroethylene; 2 patch angioplasties (6%), and 7 reimplantations (21%). Branch RA exposure was required in 28 kidneys (88%), and branch reconstruction was required in 61%. Warm in situ repair was used in 53%, in situ cold perfusion in 24%, and ex vivo cold perfusion in 23%. Of six bilateral RA repairs, one was staged and two patients are awaiting a staged repair. Combined aortic reconstruction was required in three patients. No unplanned nephrectomy was performed. There were no perioperative deaths. Hypertension was cured in 36%, improved in 56%, and failed in 8% at mean follow-up of 46.4 +/- 7.8 months. The mean calculated glomerular filtration rate increased from 82.0 mL/min/1.73 m 2 preoperatively to 98.2 mL/min/1.73 m 2 postoperatively. The postoperative patency of 30 RA reconstructions was evaluated by angiography, RDS scanning, or both. At mean follow-up of 32.8 months (median, 21.2 months), primary RA patency was 91%. No failures were observed after 2 months follow-up. Estimated survival was 100% at 60 months, with one death 9 years after surgery. CONCLUSIONS: Renovascular hypertension in children and adolescents was caused by a heterogeneous group of lesions. All patients had RA repair, with arterial autografts in most of the RA bypasses. Cold perfusion preservation was used in half of the complex branch RA repairs. These strategies provided 91% primary patency at mean follow-up of 32.8 months, with beneficial blood pressure response in 92%. Surgical repair of clinically significant renovascular disease in children and adolescents is supported by these results.     

Evaluating patients with renal failure for renal artery stenosis with gadolinium-enhanced magnetic resonance angiography

The incidence and prevalence of end-stage renal disease (ESRD) continues to increase, especially in the elderly population. The role of renovascular disease in contributing to ESRD is still not well defined. The objective of this study was to determine the utility of gadolinium (Gd)-enhanced magnetic resonance angiography (MRA) in evaluating elderly patients with renal insufficiency for renal artery stenosis (RAS). A 7-month prospective study conducted in a tertiary referral center evaluated 40 consecutive patients with progressive renal insufficiency (18 men and 22 women; mean age, 70 +/- 5.6 [standard deviation] years) and high clinical suspicion for renovascular disease with Gd-enhanced MRA. Digital subtraction angiography (DSA) was obtained in only those patients with significant RAS detected by MRA. Twelve patients had significant RAS. Six of these patients had percutaneous transluminal renal angioplasty (PTRA), five patients had renal artery bypass surgery, and one patient had a stent placed after PTRA. Seventy-eight renal arteries were satisfactorily evaluated by MRA. Twenty-two renal arteries were evaluated by both MRA and DSA. Of the 12 significant stenoses detected by the MRA, 11 were confirmed by DSA and 1 was confirmed at the time of surgical revascularization. It is concluded that Gd-enhanced MRA is a useful test for the evaluation of RAS in patients with compromised renal function.     

Pediatric renovascular hypertension: 132 primary and 30 secondary operations in 97 children

PURPOSE: This study was undertaken to characterize the contemporary surgical treatment of pediatric renovascular hypertension. METHODS: A retrospective analysis was conducted of the clinical data of 97 consecutive pediatric patients (39 girls, 58 boys), aged from 3 months to 17 years, who underwent operation at the University of Michigan from 1963 to 2006. All but one patient had refractory hypertension not responsive to contemporary medical therapy. Developmental renal artery stenoses accounted for 80% of the renal artery disease, with inflammatory and other ill-defined stenoses encountered less frequently. Splanchnic arterial occlusive lesions affected 24% and abdominal aortic coarctations, 33%. RESULTS: Primary renal artery operations were undertaken 132 times. Procedures included resection beyond the stenosis and implantation into the aorta in 49, renal artery in 7, or superior mesenteric artery in 3; aortorenal and iliorenal bypasses with vein or iliac artery grafts in 40; focal arterioplasty in 10; resection with reanastomosis in 4; operative dilation in 4; splenorenal bypass in 2; and primary nephrectomy in 13 when arterial reconstructions proved impossible. Bilateral renal operations were done in 34 children, and 17 underwent celiac or superior mesenteric arterial reconstructions, including 15 at the time of the renal operation. Thirty patients underwent abdominal aortic reconstructions with patch aortoplasty (n = 19) or thoracoabdominal bypass (n = 11). Twenty-five of the aortic procedures were performed coincidently with the renal operations. Thirty secondary renal artery procedures were done in 19 patients, including nine nephrectomies. Hypertension was cured in 68 children (70%), improved in 26 (27%), and was unchanged in three (3%). Follow-up averaged 4.2 years. No patients required dialysis, and there were no operative deaths. CONCLUSION: Contemporary surgical treatment of pediatric renovascular hypertension emphasizes direct aortic implantation of the normal renal artery beyond its stenosis and single-staged concomitant splanchnic and aortic reconstructions when necessary. Benefits accompany carefully executed operative procedures in 97% of these children.     

Treatment of severe renal artery stenosis by percutaneous transluminal renal angioplasty and stent implantation

The clinical course of two children with mid-aortic syndrome and renal artery stenosis (RAS) who suffered from severe arterial hypertension is described. Hypertension was uncontrollable by antihypertensive medication and was managed by percutaneous transluminal renal angioplasty (PTRA) with stent implantation. The pediatric experience with PTRA is limited, and there are only few cases reported with additional stent implantation. Complications of these procedures are well known from experience with adult patients. However, since surgical revascularization may be technically difficult especially in small children, PTRA with or without stenting should be considered as a valuable treatment option in pediatric RAS.     

Functional assessment of the kidneys in a 10 month‐old child with renal artery stenosis by intravoxel incoherent motion

Renovascular stenosis is an important cause for arterial hypertension in childhood. We report a 10‐month‐old girl with arterial hypertension caused by right‐sided renal artery stenosis detected by Doppler ultrasound. Magnetic resonance imaging (MRI) was performed before renal artery angioplasty to depict vascular anatomy in detail and to retrieve additional functional information of the kidneys by analysis of intravoxel incoherent motion (IVIM). The value of quantitative diffusion weighted imaging of the kidneys prior to percutaneous transluminal renal angioplasty (PTRA) is discussed.     

Reduction in renal function after renal arteriography and after renal artery angioplasty.

OBJECTIVE: To investigate the incidence and risk factors for renal function deterioration after renal angiography and angioplasty or stenting. METHODS: A retrospective study of 85 consecutive patients undergoing selective renal artery arteriography (n = 53) or renal artery angioplasty % (PTRA) stenting (n = 32) for renal artery stenosis. Multivariate logistic regression analysis was used to determine independent predictors of deterioration of renal function, defined as an increase of serum creatinine by at least one third within 24 h. RESULTS: Deterioration of renal function occurred in 13 patients (15%), [8/53 (15%) after angiography and 5/32 (16%) after PTRA/stenting]. Only pre-existing renal impairment (se-creatinine > or = 177 mumol/l) (Odds ratio: 40; 95% confidence interval 1.2-72, p = 0.02) and administered dosage of contrast agent (more than 225 ml) (OR 67; 95% CI 11.8-100, p = 0.02) were independently associated with renal function deterioration. CONCLUSION: Transient renal dysfunction after renal artery angiography or PTRA/stenting occurs in about 15% of patients, but persistent renal failure is uncommon. Pre-existing renal impairment and amount of contrast agent are independent risk factors. Endovascular treatment of renal artery stenosis is not associated with a higher risk of renal deterioration compared to selective renal angiography.     

Renovascular hypertension in children: current concepts in evaluation and treatment

Since 1981, we have evaluated and treated 22 children with renovascular hypertension (RVH). Seventeen patients had stenosis of their native renal arteries, and five had stenosis of the artery in a transplanted kidney. RVH was caused by fibromuscular dysplasia in 13 patients, by trauma in 2 patients, and by arteritis in 2 patients. Among the patients who had transplanted kidneys, three had technical causes for stenosis and two had stenosis due to rejection. The disease was unilateral in 10 patients, bilateral in 5, and present in a solitary kidney in 7, including the five renal transplants. Diagnostic studies that strongly suggested the presence of renovascular disease were an initial diastolic blood pressure greater than 100 mm Hg, an elevated peripheral vein renin activity level, and an abnormal renal scan if the patient's hypertension was being controlled with an angiotensin-converting enzyme inhibitor (ACEI). Only the renal arteriogram was 100% accurate in confirming the presence of RVH. Percutaneous angiographic correction was attempted in 13 patients and resulted in lasting improvement of the hypertension in five (38%). Surgical revascularization was attempted in 17 children, including the 8 with failed angioplasty, with improvement or cure of the hypertension in 15 patients (88%). Combining percutaneous transluminal angioplasty (PTA) and surgical results gave 20 of 22 patients (91%) with cure or improvement of their hypertension. Four of 27 affected kidneys (15%) could not be revascularized and were removed. We conclude from this series of patients that despite improvements in noninvasive studies, renal arteriogram remains the only study that is 100% accurate in evaluating children for RVH.(ABSTRACT TRUNCATED AT 250 WORDS)