Diagnosis and management of systemic hypertension due to renovascular and aortic stenosis in patients with Williams-Beuren syndrome

SUMMARY AIM To describe the incidence, diagnosis, and management of systemic arterial hypertension related to renal artery stenosis in patients with Williams-Beuren syndrome. METHODS Sixty-five patients with Williams-Beuren syndrome were evaluated for hypertension. Enrolled patients underwent Doppler sonography of the renal arteries and Doppler echocardiography. Those with Doppler sonography-detected lesions or with normal Doppler sonography but severe hypertension underwent computed tomography or gadolinium-enhanced magnetic resonance angiography of the aorta and renal vessels. Patients needing vascular therapeutic intervention underwent conventional angiography. RESULTS Systemic arterial hypertension was diagnosed in 21/65 patients with Williams-Beuren syndrome (32%; 13 male) with a mean age of 13.9 years (5mo-20yrs). In 8/21 patients renovascular hypertension was detected. Angioplasty was unsuccessful in five patients with renal artery stenosis, requiring additional treatment. Doppler echocardiography showed cardiac abnormalities in 16/21 (76%) hypertensive patients. CONCLUSION Cardiac abnormalities and hypertension in patients with Williams-Beuren syndrome are common. Thus, thorough evaluation and follow-up are necessary to reduce cardiovascular risks and mortality of these patients

RESUMO OBJETIVO Descrever a incidência, o diagnóstico e o tratamento da hipertensão arterial sistêmica relacionada com estenose da artéria renal em pacientes com síndrome de Williams-Beuren. MÉTODOS Sessenta e cinco pacientes com síndrome de Williams-Beuren foram avaliados quanto à presença de hipertensão. Os pacientes foram submetidos à ultrassonografia com Doppler das artérias renais e ecocardiograma Doppler. Aqueles com suspeita de hipertensão renovascular foram submetidos à tomografia computadorizada ou angiografia por ressonância magnética da aorta e vasos renais ou angiografia convencional. RESULTADOS A hipertensão arterial sistêmica foi diagnosticada em 21/65 pacientes com síndrome de Williams-Beuren (32%, 13 do sexo masculino), com idade média de 13,9 anos (5 meses-20 anos). Em 8/21 pacientes foi detectada a hipertensão renovascular. Angioplastia não teve sucesso em cinco pacientes com estenose da artéria renal, necessitando de tratamento adicional. O ecocardiograma Doppler mostrou anormalidades cardíacas em 16/21 (76%) pacientes hipertensos. CONCLUSÃO As anormalidades cardíacas e hipertensão arterial em pacientes com síndrome de Williams-Beuren são muito frequentes, sendo necessários uma avaliação minuciosa e seguimento para diminuir o risco cardiovascular e a morbimortalidade desses pacientes

Prevalence of carotid and renal artery stenosis in patients with significant coronary artery disease

Atherosclerosis as a vascular disease can involve any artery in the body including large and medium sized arteries. Only a few angiographic studies have correlated the presence and severity of coronary artery disease with atherosclerosis in other arteries. The aim of this study is to evaluate the prevalence of the carotid and renal artery stenosis in patients with documented coronary artery disease and the role of predisposing risk factors for such affection in Egyptian patients. This study included 100 patients with documented significant coronary artery disease. All were subjected to carotid and renal angiography. They were divided into: group I which included patients with significant carotid artery stenosis [>50%] either unilateral or bilateral, and group II which included patients with normal carotid. They divided again into: group A which included patients with significant renal artery stenosis [>50%], while group B included patients with normal renal angiography. For all patients, random blood sugar, serum urea, creatinine and complete lipid profile was assessed. Group I included 17 patients while Group II included 83 patients with documented significant coronary artery disease. There were significant correlations between either of age, hypertension, dyslipedemia, diabetes mellitus and presence of carotid artery stenosis. While there was no relation between carotid artery stenosis with neither gender nor smoking. Number of the affected coronary arteries was the significant multivariate predictor of significant carotid artery stenosis. While group A included 7 patients and group B included 93 patients with documented coronary artery disease. Hypertension and serum levels of both urea and creatinine were significantly univariate predictor of the presence of renal artery stenosis. The significant multivariate predictor of presence of renal artery stenosis was serum level of blood urea. The prevalence of significant carotid artery stenosis with significant coronary artery disease was found to be 17%. The prevalence of significant renal artery stenosis with significant coronary artery disease was found to be 7%. Carotid artery disease is more common in patients with old age, hypertension, dyslipidemia and with more than two vessel diseased coronaries. Renal artery disease is more common in hypertensive patients with elevated blood urea and serum creatinine levels and with more than two vessel diseased coronaries

Prevalência de estenose das artérias renais em 1.656 pacientes que realizaram cateterismo cardíaco / Prevalence of renal artery stenosis in 1.656 patients who have undergone cardiac catheterization

OBJETIVO: Determinar a prevalência de estenose da artéria renal (EAR) em pacientes submetidos a cineangiocoronariografia. MÉTODOS: Estudo prospectivo, considerando 1.656 cinean-giocoronariografias seguidas de aortografia, entre janeiro/2002 e fevereiro/2004, de pacientes encaminhados à cineangiocoronariografia diagnóstica com história ou não de hipertensão arterial sistêmica (HAS). RESULTADOS: Dos 1.656 pacientes, a idade média foi de 61,6 ± 11,8 anos, 53,8 por cento eram do sexo masculino, 10,2 por cento eram diabéticos, 63,8 por cento apresentavam coronariopatia obstrutiva. A presença de EAR maior que 50 por cento foi observada em 228 (13,8 por cento) pacientes, e em 25 (1,5 por cento) destes, ocorreu bilateralmente. A coronariopatia obstrutiva foi definida como estenose que causa redução do lúmen do vaso em 50 por cento ou mais, em um, dois ou três vasos principais, denominados uniarterial, biarterial ou triarterial, respectivamente.A quantificação era realizada através da análise visual da angiografia. Comparando os grupos com e sem EAR > 50 por cento, observou-se diferença estatisticamente significativa quanto a gênero, idade, ocorrência de diabete melito, PA e função ventricular esquerda. Não houve diferença significativa, no entanto, quanto à ocorrência de obstrução coronariana > 50 por cento. Quando, porém, a EAR considerada é > 70 por cento, observa-se diferença significativa quanto a PA, associação à obstrução coronariana > 50 por cento e à disfunção ventricular esquerda, maiores no grupo com EAR. CONCLUSÃO: A prevalência de EAR neste estudo foi comparável àquela das grandes casuísticas da literatura e, em razão de sua importância pela associação com HAS e doença renal terminal (DRT) e suas seqüelas, devemos estar atentos para seu diagnóstico angiográfico.

OBJECTIVE: To determine the prevalence of renal artery stenosis (RAS) in patients who have undergone cineangiocoronariography. METHODS: Prospective study of cineangiocoronariography and aortography examinations conducted between January 2002 and February 2004 on 1,656 hypertensive and normotensive patients who underwent the examinations to confirm the diagnosis of obstructive coronary artery disease or valve disease. RESULTS: The average age of the 1,656 patients was 61.6 ± 11.8 years. Eight hundred and ninety-one (53.8 percent) were male, 169 (10.2 percent) were diabetic and 1,054 (63.8 percent) presented obstructive coronary artery disease. Renal stenosis greater than 50 percent was observed in 228 (13.8 percent) patients, and 25 (1.5 percent) had bilateral stenosis. Obstructive coronary artery disease was defined as stenosis greater than or equal to 50 percent of the vessel lumen, in one, two or three main arteries, classified as single, double or triple vessels, respectively. Quantification was conducted using visual analysis of the angiography. Comparison of the groups with and without renal artery obstruction > 50 percent, revealed significant statistical differences in relation to gender, age, diabetes mellitus, blood pressure and left ventricular function. However, no statistical difference was noted in relation to the occurrence of coronary artery obstructions > 50 percent. Nevertheless, renal artery obstructions > 70 percent, revealed significant differences in relation to blood pressure, coronary artery obstructions > 50 percent and left ventricular function, which were all higher in the renal artery obstruction group. CONCLUSION: The prevalence of RAS found in our study was comparable to that reported by major medical literature case studies. RAS is associated with systemic hypertension (SH), end-stage renal disease (ESRD) and its sequelae, emphasizing how important it is that we are aware of possible candidates for angiographic diagnosis of this disease.

Prevalence of renal atery stenosis in patients undergoing routine cardiac catheterization

To determine the prevalence of renal artery stenosis [RAS] and associated risk factors in patients undergoing cardiac catheterization for suspected coronary artery disease. Three hundred and fifty-four consecutive patients [71 female] were studied at the Cardiology Unit of King Abdullah University Teaching Hospital, Irbid, Jordan, between May 2002 and May 2003. Left-sided cardiac catheterization and abdominal aortography were performed to screen for coronary and renal artery disease. Of the 354 patients, 285 had coronary artery disease and 27 had RAS. Significant RAS was present in 11 patients. Patients with RAS were older [66 +/- 8 versus 59 +/- 10, mean +/- SD; p=0.004], had higher incidence of systolic hypertension [156 +/- 14 versus 130 +/- 16 mm Hg; p=0.005], diabetes mellitus [72% versus 38%; p=0.004], smoker [85% versus 55; p=0.005], and had less than 2 coronary lesions. The prevalence of significant and insignificant RAS is 3.1% and 4.5%. Diagnostic yield increase in elderly patient with less than 2 coronary lesions, elevated systolic pressure, smoking, diabetes mellitus, and electrocardiogram criteria of left ventricular hypertrophy

The prevalence and associated risk factors of renal artery stenosis in patients undergoing cardiac catheterization

Renal artery stenosis may be a cause of hypertension and a potential contributor to progressive renal insufficiency. However, the prevalence of renal artery disease in a general population is poorly defined. The purposes of this study were to evaluate the prevalence of angiographically-determined renal artery narrowing in a patient population undergoing routine cardiac catheterization, and to identify the risk factors for renal artery stenosis. After left ventriculography, abdominal aortography was performed to screen for the presence of renal artery stenosis. A total of 427 patients (274 males, 153 females) were studied and the mean age was 59 years. Renal artery narrowing was identified in 10.5% of patients. Significant (> or = 50% diameter narrowing) renal artery stenosis was found in 24 patients (5.6%) and insignificant stenosis was found in 21 patients (4.9%). Significant unilateral stenosis was present in 4.2% of patients and bilateral stenosis was present in 1.4%. The stem of the renal artery was a more common site of stenosis in 62.2% of patients than in the ostium (37.8%), but the severity of stenosis was not significantly different according to the site of stenosis. By univariate and multivariate logistic regression analysis, the association of clinical variables with renal artery stenosis was assessed. Multivariable predictors included age, hypertension and peripheral vascular disease (p < 0.05). The variables such as sex, smoking history, hyperlipidemia, renal insufficiency, as well as the presence of obesity, severity of coronary heart disease and D.M., were not associated. In conclusion, the prevalence of angiographically-determined renal artery narrowing in a patient population undergoing cardiac catheterization is 10.5%. Old age, hypertension and evidence of peripheral vascular disease represent the predictors of renal artery stenosis.