Increased arterial distensibility and renovascular hypertension in Goldenhar syndrome.

This is a report of the successful angioplastic treatment of an association of renovascular hypertension with renal artery stenosis and the Goldenhar syndrome (a variant of oculoauriculovertebral dysplasia). For the first time to date, this association, which occurred in a 13-year-old girl, is reported. Additionally, increased arterial distensibility in spite of arterial hypertension was detected by noninvasive methods. The similarity of this finding and in those for other genetic diseases, suggests that the vascular lesions could be linked to the Goldenhar syndrome.

Increased arterial distensibility and renovascular hypertension in Goldenhar syndrome

Relatamos a associação de hipertensão renovascular por estenose de artéria renal e a Sindrome de Goldenhar (variante da displasia oculoauriculovertebral) em uma paciente do sexo feminino de 13 anos de idade. Este é o primeiro relato de tratamento por angioplastia. Além disso, detectamos por métodos não invasivos um aumento da distensibilidade arterial, a despeito da hipertensão arterial. A similaridade destes achados com outras doenças genéticas sugere que as alterações vasculares presentes podem estar relacionadas à Síndrome de Goldenhar.

The contrast-enhanced Doppler ultrasound with perfluorocarbon exposed sonicated albumin does not improve the diagnosis of renal artery stenosis compared with angiography.

There are no studies investigating the effect of the contrast infusion on the sensitivity and specificity of the main Doppler criteria of renal artery stenosis (RAS). Our aim was to evaluate the accuracy of these Doppler criteria prior to and following the intravenous administration of perfluorocarbon exposed sonicated albumin (PESDA) in patients suspected of having RAS. Thirty consecutive hypertensive patients (13 males, mean age of 57 +/- 10 years) suspected of having RAS by clinical clues, were submitted to ultrasonography (US) of renal arteries before and after enhancement using continuous infusion of PESDA. All patients underwent angiography, and haemodynamically significant RAS was considered when >or=50%. At angiography, it was detected RAS >or=50% in 18 patients, 5 with bilateral stenosis. After contrast, the examination time was slightly reduced by approximately 20%. In non-enhanced US the sensitivity was better when based on resistance index (82.9%) while the specificity was better when based on renal aortic ratio (89.2%). The predictive positive value was stable for all indexes (74.0%-88.0%) while negative predictive value was low (44%-51%). The specificity and positive predictive value based on renal aortic ratio increased after PESDA injection respectively, from 89 to 97.3% and from 88 to 95%. In hypertensives suspected to have RAS the sensitivity and specificity of Duplex US is dependent of the criterion evaluated. Enhancement with continuous infusion of PESDA improves only the specificity based on renal aortic ratio but do not modify the sensitivity of any index.

Malignant hypertension and hypertensive encephalopathy in primary aldosteronism caused by adrenal adenoma.

Two cases are reported as follows: 1) 1 female patient with accelerated-malignant hypertension secondary to an aldosterone-producing adrenal adenoma; and 2) 1 female patient with adrenal adenoma, severe hypertension, and hypertensive encephalopathy. This association is a rare clinical finding, and malignant hypertension may modify the hormonal characteristic of primary aldosteronism, making its diagnosis more difficult. The diagnosis of primary aldosteronism should be considered in patients with malignant hypertension or hypertensive encephalopathy if persistent hypokalemia occurs. Identification of primary aldosteronism is of paramount importance for the patient's evolution, because the surgical treatment makes the prognosis more favorable.

Hipertensão arterial maligna e encefalopatia hipertensiva em hiperaldosteronismo primário por adenoma de supra-renal / Malignant hypertension and hypertensive encephalopathy in primary aldosteronism caused by adrenal adenoma

Two cases are reported as follows: 1) 1 female patient with accelerated-malignant hypertension secondary to an aldosterone-producing adrenal adenoma; and 2) 1 female patient with adrenal adenoma, severe hypertension, and hypertensive encephalopathy. This association is a rare clinical finding, and malignant hypertension may modify the hormonal characteristic of primary aldosteronism, making its diagnosis more difficult. The diagnosis of primary aldosteronism should be considered in patients with malignant hypertension or hypertensive encephalopathy if persistent hypokalemia occurs. Identification of primary aldosteronism is of paramount importance for the patient's evolution, because the surgical treatment makes the prognosis more favorable

Hipertensão renovascular: novas diretrizes no tratamento / Renovascular hypertension: new guidelines on the treatment

Os objetivos principais do tratamento da hipertensão renovascular são o controle efetivo da hipertensão arterial e a preservação da função renal. As três modalidades terapêuticas disponíveis incluem tratamento clínico, angioplastia ou implante de stent e revascularização cirúrgica. A escolha do tratamento deve ser individualizada com base na idade, etiologia da estenose e presença de doenças associadas. O tratamento clínico tem sido reservado para os casos de impossibilidade técnica, quando há alto risco para o procedimento intervencionista e, para doentes especiais nos quais a pressão arterial e a função renal se mantêm controladas com o tratamento clínico convencional. A angioplastia percutânea primária é a terapêutica de escolha em indivíduos com displasia fibromuscular devido aos bons resultados a longo prazo, comparáveis aos resultados cirúrgicos. Em pacientes com doença renal aterosclerótica, há superioridade do tratamento cirúrgico sobre o tratamento clínico, mas recentes ensaios randomizados comparando angioplastia e tratamento clínico tem mostrado resultados similares em relação ao controle da pressão arterial e da função renal. Assim, o implante de stent tem sido indicado para estenose unilateral ou bilateral não associada a doença de aorta, principalmente nas lesões ostiais nas quais os resultados são melhores do que os obtidos com a angioplastia. Para os casos complicados de estenose de artéria renal com aneurisma de aorta ou oclusão total da artéria, a revascularização cirúrgica é mais indicada.

Hipertensäo arterial na mulher / Arterial hypertension in womem

Inicialmente, säo apresentados conceitos, dados epidemiológicos principalmente da hipertensäo essencial e aspectos da hipertensäo secundária na mulher. Em seguida, os autores avaliam o tópico hipertensäo e contraceptivos orais, com dados sobre prevalência, mecanismos de açäo, quadro clínico, fatores predisponentes e tratamento. A hipertensäo e pós-menopausa é discutida, sendo apresentados em detalhes dados sobre a incidência, os mecanismos envolvidos e a influência do tratamento hormonal substitutivo (TRH). O tratamento da hipertensäo após a menopausa também é apresentado, mencionando-se resultados de metanálises e estudos internacionais (WHI) e multicêntricos. Finalizando, os autores reavaliam o tópico da gestaçäo e hipertensäo, mencionando definiçöes, fatores de risco, dados demográficos incluindo os diversos transtornos: pré-eclâmpsia, hipertensäo crônica, pré-eclâmpsia superajuntada à hipertensäo crônica, hipertensäo gestacional, bem como a eclâmpsia. O tratamento é também discutido para os diversos tópicos, incluindo a hipertensäo arterial crônica, a pré-eclâmpsia e a eclâmpsia.(au)

Avaliaçäo hormonal e de reflexos cardiovasculares em portadora de falência autonômica pura / Hormonal and cardiovascular reflex assessment in a female patient with pure autonomic failure

We report the case of a 72-year-old female with pure autonomic failure, a rare entity, whose diagnosis of autonomic dysfunction was determined with a series of complementary tests. For approximately 2 years, the patient has been experiencing dizziness and a tendency to fall, a significant weight loss, generalized weakness, dysphagia, intestinal constipation, blurred vision, dry mouth, and changes in her voice. She underwent clinical assessment and laboratory tests (biochemical tests, chest X-ray, digestive endoscopy, colonoscopy, chest computed tomography, abdomen and pelvis computed tomography, abdominal ultrasound, and ambulatory blood pressure monitoring). Measurements of catecholamine and plasmatic renin activity were performed at rest and after physical exercise. Finally the patient underwent physiological and pharmacological autonomic tests that better diagnosed dysautonomia.

Falso diagnóstico de miocardiopatia hipertrófica em feocromocitoma / A false dignosis of hypertrophic myocardiopathy in pheochromocytoma

A 24 year-old man was admitted with hypertensive crises and diagnosis hypothesis of neurofibromathosis and pheochromocytoma with blood pressure of 150 x 110mmHg and in use of anti-hypertensive drugs. The electrocardiogram (EKG) showed left ventricle hypertrophy. An echocardiogram showed interventricular septum (IVS) thickness of 16mm, posterior wall (PW) thickness of 11mm (ratio IVS/PW was 1.4). Diastolic ventricular diameter was 39mm with gradient of 52mmHg and mild mitral-valve murmur by pulsate Doppler. Increased vanillylmandelic acid and metanephrines in a 24-hour sample of urine has confirmed diagnosis of pheochromocytoma within was localized by 131I metaiodobenzyl-guanidine scan and computerized axial tomography. The patient was submitted to right adrenalectomy. Blood pressure was normalized. Evaluation an year later revealed a healthy man with normal laboratory exams, EKG and echocardiogram. It seems that the hypertrophy was consequence of the hypertension and pheochromocytoma, was not hypertrophic cardiomyopathy.

Homem de 24 anos foi admitido com hipótese diagnóstica de neurofibromatose e feocromocitoma, com pressão arterial (PA) de 150 x 110 mmHg e em uso de medicação anti-hipertensiva. O eletrocardiograma mostrava sobrecarga de ventrículo esquerda. O ecocardiograma relatado com diagnóstico de miocardiopatia hipertrófica assimétrica, mostrava a espessura de septo interventricular de 16mm, parede posterior de 11mm, diâmetro diastólico de 39mm, com gradiente intra-ventricular de 52mmHg e discreta regurgitação proto-sistólica valvar mitral ao doppler pulsátil. As dosagens de ácido vanilmandélico e de metanefrina urinárias confirmaram a suspeita clínica de feocromocitoma, localizado através da cintilografia com metaiodobenzilguanidina e tomografia axial computadorizada. Um ano após adrenalectomia direita se encontra assintomático, com PA, alterações laboratoriais, eletrocardiográficas e ecocardiográficas normais, evidenciando assim, que a hipertrofia de ventrículo esquerdo (HVE) era conseqüência da hipertensão arterial do feocromocitoma e não da miocardiopatia hipertrófica. Esta regressão da HVE excluiu, assim, o falso diagnóstico de micardiopatia hipertrófica

Papel da hipertrofia e da funçäo ventricular esquerda sobre a ocorrência de arritmias ventriculares na hipertensäo arterial / The role of hypertrophy and left ventricular function on the ocurrence of ventricular arrhythmias in arterial hypertension

PURPOSE--To evaluate the role of left ventricular hypertrophy (LVH), left ventricular systolic function an other clinical parameters on prevalence and complexity of ventricular arrhythmias in hypertension. METHODS--Ventricular arrhythmias were studied in 39 hypertensives by 24 hours ambulatory electrocardiographic monitoring. Frequency and complexity of ventricular arrhythmias were compared among 3 groups: A and B, respectively without and with LVH, both with normal left ventricular function; and C with LVH and systolic dysfunction. LVH and systolic dysfunction were established echocardiographically. Linear regression analysis was performed in order to identify an independent correlation between clinical parameters and presence of arrhythmias. RESULTS--Group C patients were older and had significantly higher systolic and diastolic blood pressures, greater mass index, diastolic posterior wall thickness and end-systolic stress and increased prevalence of electrocardiographic strain. Left ventricular diastolic diameter in C group patients was increased only when compared to A group. Frequencies of cases with more than 10 ectopic ventricular beats/hour, pairs and nonsustained ventricular tachycardia episodes were all significantly increased in C when compared to B and to A. However, only left ventricular mass index or diastolic posterior wall thickness identified independently patients with ventricular arrhythmias. CONCLUSION--Left ventricular hypertrophy is the main predictor of potential high risk rhythm disturbances in hypertension

Papel da pressão arterial casual e do exercício e a influência de fatores clínicos sobre a hipertrofia ventricular esquerda na hipertensäo arterial / Role of Resting and Exercising Blood Pressure and Importance of Clinical Factors on Hypertensive Left Ventricular Hypertrop

PURPOSE--To evaluate the role of casual and exercise blood pressure as well as the importance of clinical factors on the presence and degree of left ventricular hypertrophy in hypertension. METHODS--Fifteen normotensives (control group) and 30 hypertensives, 14 of them with and 16 without left ventricular hypertrophy (groups with LVH and without LVH, respectively) were studied. LVH diagnosis was established when mass index was higher than 2 standard-deviations of the mean values calculated for each sex in control group. Resting, casual determined, and bicycle exercise systolic and diastolic blood pressures along with age, body surface area, sex and race distribution were compared between groups. In addiction, their relation with mass index as independent variables were also tested. RESULTS--Hypertensives in group with LVH had higher diastolic septal, posterior wall, and relative wall thicknesses. No significant statistical difference was observed neither in sex and race distribution, nor in age and body surface area between groups. Otherwise, there were significant differences in both resting and exercise blood pressure. In the entire population studied, left ventricular mass index significantly correlated with age (r=0,33, p=0,03) as well as with both casual (systolic - r=0,72, p=0,0001; diastolic - r=0,69, p=0,0001) and exercise (systolic - r=0,62, p=0,0001; diastolic - r=0,66, p=0,0001) blood pressures. However, linear regression analysis demonstrated that only resting systolic (p=0,0001) and exercise diastolic (p=0,0303) blood pressures were significant and independent determinants of mass index. CONCLUSION--Resting and exercising blood pressures are the main determinants of left ventricular hypertrophy in hypertension

Angioplastia percutânea e revascularizaçäo cirúrgica em hipertensäo renovascular: experiência no tratamento e seguimento de longo prazo em 124 pacientes / Percutaneous Angioplasty and Surgical Revascularization in Renovascular Hypertension. Experience on Long-Term Follow-up and Treatment in 124 Patients

PURPOSE--To report the experience in the treatment and long-term follow-up of patients with renovascular hypertension submitted to angioplasty and/or surgical revascularization, and to compare blood pressure and renal function responses to both treatment. METHODS--One hundred and twenty-four patients diagnosed with renovascular hypertension, divided, according to the etiology, as atherosclerosis (76 patients), fibromuscular dysplasia (32) and arteritis (16), were submitted to isolated or associated treatment of renal revascularization by percutaneous transluminal angioplasty (71 patients) or surgery (84). The patients were followed-up during 2.5 +/- 1.5 years and we evaluate blood pressure response and renal function preservation. In relation to blood pressure control, the patients were considered as cured when normotensives without antihypertensives, improved when normotensives with fewer drugs than previously, and unchanged, when out of these criteria. RESULTS--From 124 patients, 20 per cent were considered cured, 54 per cent improved and 26 per cent unchanged, after a mean follow-up of 2.5 +/- 1.5 years. Patients with atherosclerosis and fibromuscular dysplasia presented a higher rate of care and improvement after surgery. The blood pressure and the number of antihypertensive drugs decreased significantly during the follow-up among patients that cured or improved arterial hypertension, either after angioplasty (190 +/- 26/115 +/- 14 mmHg vs 130 +/- 34/85 +/- 7 mmHg. 2.70 vs 1.60 drugs) or after surgery (194 +/- 17/115 +/- 16 mmHg vs 143 +/- 18 mmHg vs 88 +/- 8 mmHg, 1.88 vs 1.51 drugs). It was also observed a significant decrease of serum creatinine among these patients. CONCLUSION--Renal revascularization in patients with renovascular hypertension, either by angioplasty or surgery, is beneficial to control blood pressure and to preserve renal function in the majority of patients

Hipertensão arterial e arritmias ventriculares complexas: evoluçäo após tratamento de longo prazo da pressäo arterial / Hypertension and complex ventricular arrhythmias: evolution following a long-term blood pressure treatment

PURPOSE--To evaluate the effects of long-term antihypertensive treatment in the frequency as well as in the complexity of ventricular arrhythmias in arterial hypertension. METHODS--Twenty three patients, 14 males and 11 whites, with mean age of 46 years, were submitted to 24 hours ambulatory electrocardiographic monitoring and echocardiographic studies before and 9 months after antihypertensive treatment. RESULTS--There was no significant serum potassium level alteration, but significant reductions of both systolic (from 192 +/- 29mmHg to 161 +/- 25mmHg) and diastolic (from 122 +/- 17mmHg to 99 +/- 16mmHg) blood pressure. Left ventricular percent of fiber shortening significantly increased, even though only from 26 +/- 9 per cent to 30 +/- 9 per cent, and end-systolic wall stress did not change at all (before 258 +/- 94 10(3) dyn/cm2, after 255 +/- 101 10(3) dyn/cm2). Left ventricular mass index showed significant but also a discrete reduction from 211 +/- 75g/m2 to 196 +/- 70g/m2. Ambulatory electrocardiographic monitoring did not show any significant decrease in neither ventricular ectopic beats nor in couplets. Non-sustained ventricular tachycardia episodes remained unchanged too. Four out of 8 patients with more than 30 ventricular ectopic beats per hour reduced it by more than 70 per cent. On the other hand, the number of patients with couplets was reduced from 10 to 8 whilst those with non-sustained ventricular tachycardia increased from 5 to 7. Furthermore, in 7 patients reevaluated 24 months thereafter results were not expressively dissimilar. CONCLUSION--In hypertensive patients with either severe degree of left ventricular hypertrophy or myocardial dysfunction, long-term blood pressure treatment that produce no impressive changes in these abnormalities also do not modify complex ventricular arrhythmias, in spite of a great reduction in the increase blood pressure

Hiperaldosteronismo primário e tumores adrenais: experiência clínico-cirúrgica em nove pacientes / Cardiac Surgery in Childhood. Impact in Young People Quality of Life

PURPOSE--To report seven patients with diagnosis of primary aldosteronism, five of them due to aldosterone-producing adenoma (APA) and two due to idiopathic hyperaldosteronism (IHA), and two patients with adrenal non-producing tumors in order to discuss our experience on differential diagnosis and treatment of this hypertensive disease. METHODS--Hypokalemia and higher values of urinary potassium in the absence of diuretics were useful to the screening diagnosis of primary aldosteronism, reinforced by suppressed plasma renin activity either at rest and after deambulation and by higher values of plasma aldosterone. Computerized tomography in all patients and selenium-cholesterol scintigraphy were used to make the localization of tumors and differential diagnosis between APA and IHA. RESULTS--The patients with adrenal tumors were submitted to surgical treatment and the two patients with IHA were submitted to spironolacone therapy. After 1 to 5 years of follow-up, we observed cure of hypertension and hypokalemia in three patients after surgery and improvement of blood pressure control and normalization of serum potassium in the six others. CONCLUSION--The diagnosis of primary aldosteronism is important, besides its rarity, because surgical or appropriated clinical treatment provide cure of hypertension or improvement of blood pressure control in most of patients

Proteinúria em pacientes com insuficiência cardíaca congestiva: papel da hipertensäo arterial / Proteinuria in Congestive Heart Failure. Role of Arterial Hypertension

Objetivo - Avaliar a importância da hipertensäo arterial (HA), quando fator etiológico da insuficiência cardíaca congestiva (ICC), no desenvolvimento de proteinúria em pacientes com ICC descompensada. Métodos - Foram estudados 30 pacientes com ICC descompensada, analisando a presença da proteinúria acima de 150 mg em amostra urinária de 24h antes e após o tratamento. Os pacientes foram divididos em dois grupos conforme a presença de HA como causa da ICC: grupo A, 18 pacientes com miocardiopatia hipertensiva e grupo B, 12 pacientes com miocardiopatia dilatada idiopática ou chagásica sem história prévia de HA. Resultados - Näo houve diferenças emtre os grupos com relaçäo a idade, sexo, funçäo renal ou tempo da ICC. No período pré-tratamento, 88// dos pacientes do grupo A apresentavam proteinúria (média 1580mg/24h) enquanto que apenas 25// dos pacientes do grupo B (média 320mg/24h) a apresentavam (p<0,01). Após o tratamento da ICC, 38// dos pacientes do grupo A persistiram com proteinúria com média de 1128 mg/24h, enquanto nenhum paciente do grupo B manteve-se com proteinúria. Após 6 meses de evoluçäo, dois pacientes persistiram com proteinúria, um dos quais, submetido a biópsia renal, revelou nefrosclerose benigna. Conclusäo - Em pacientes com ICC descompensada, näo secundária a HA, a proteinúria é discreta, totalmente revesível com o tratamento que por sua vez melhora a funçäo renal. Nos pacientes onde a ICC é secundária a HA, a proteinúria é mais freqüente, tem maior gravidade e pode näo ser reversível com o tratamento, por vezes elevando-se, com piora da funçäo renal, o que indicaria lesäo secundária à HA

Purpose - To evaluate the role of arterial hypertension (AH) in the development of proteinuria in patients with decompensated congestive heart failure (CHF). Methods - Twenty - four - hour urinary protein determinations were obtained from 30 patients with decompensated CHF, before and affer therapy of CHF. The patients wore divided in two groups according with the presence of AH as cause of CHF: group A, 18 patients with CHF due to AH and group B. 12 patients with CHF due to idiopathic dilated or chagasic cardiomyopathy without previous AH. Results - There was no differences between the groups concerning age, sex, renal function and duration of CHF. Before treatment, 88% of group A presented proteinuria exceeding 150mg/24h compared to 25% of group B (p<0,01). After treatment of CHF, proteinuria >150mg/24h remained in 38% of group A and in none of group B. Proteinuria did not reverse after 6 months of follow-up in 2 patients, one of them showed benignnephrosclerosis in renal biopsy. Conclusion - Patients with decompensated CHF, unrelated to AH, presented mild proteinuria reversible after the treatment, that improved renal function. In patients with CHF due to AH, proteinuria was more frequent, more severe and did not improve after the treatment, suggesting renal lesion

Feocromocitoma: peculiaridades diagnósticas e terapêuticas / Pheochromocytoma. dicignostics and therapeutics peculiorities

O feocromocitoma é causa de hipertensão arterial (HA) na qual o tratamento cirúrgico oferece grande probabilidade de cura com normalização da pressão arterial. O presente relato de 5 casos de feocromocitoma com peculiaridades especiais tem como objetivo transmitir nossa experiência no diagnóstico e tratamento desta doença hipertensiva. Em 4 dos cinco casos observamos características pouco usuais da doença associada à neurofibromatose (1 caso), à valvopatia reumática (1 caso), a caráter maligno (1 caso) e à presença de alterações eletrocardiográficas indicativas de miocardite adrenérgica, revertidas após o tratamento cirúrgico (1 caso). Diante dos sintomas de hiperatividade adrenérgica presentes em todos os casos, a elevação dos níveis de ácido vanil-mandélico e metanefrinas urinárias foram úteis para a confirmação da suspeita clínica inicial. A associação da tomografia computadorizada e do mapeamento radioisotópico com metaiodobenzilguanidina (mIBG) permitiu a localização das massas tumorais, bem como das metástases ósseas de um caso de feocromocitoma maligno. Com o tratamento cirúrgico, após um período de 6 meses a 4 anos de evolução, quatro pacientes apresentaram cura da hipertensão, estavam assintomáticos e com normalização dos metabólitos urinários de catecolaminas. O doente, portador da forma maligna, permaneccu com produção elevada de catecolaminas, hipertenso após a retirada do tumor adrenal e realiza atualmente irradiação das metástases com mIBG radioativo

Pheochromocytoma is a cause of hypertension that frequently can be cured by surgery. The aim of this paper, based on 5 cases of pheochromocytoma, is to relate our experience in diagnosis and treatment in this pathology. In four of 5 patients with pheochromocytoma we observed unusual caracteristics of the disease. Association with neurofibromatosis in one case, with rheumatic mitral regurgitation in another; and in a third case the tumor was malignant. One patient had catechol amine-mediated electrocardiographic changes which disappeared with treatment. Since symptoms of adrenergic hiperactivity were present in all cases, the rise in the levels of vanilmandelic acid and urinary metanephrines were useful in confirming the diagnosis. Computed tomography and I-131 metabenzylquanidine for radioisotopic imaging, displayed not only all tumoral masses but also bone metastases in the malignant case. During the follow-up period, from the sixth month to the fourth year after surgery, four patients were asymptomatic, and have normal urinary catechoIAMine metabolite levels. The patient with a malignant form of pheochromocytoma continued to show elevated catecholamines release and remained hypertensive in spite of adrenal mass ressection