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.

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.

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.

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.

OBJETIVO: Avaliar a evolução da hipertensão arterial (HA), e suas consequências, em pacientes submetidos a transplante cardíaco (TC) em uso de ciclosporina (CL). MÉTODOS: Em 65 pacientes submetidos a TC ortotópico, avaliamos a pressão arterial, creatinina sérica e níveis sangüíneos de CL nos períodos pré-operatório (15 dias antes do TC), pós-operatório precoce (15 e 30 dias) e tardio (6, 12, 24, 48 e 60 meses); em 20 pacientes analisamos índice cardíaco e resistência vascular no pré, 15 e 30 dias, 6 e 12 meses após TC; em 33 pacientes, estudamos estrutura e função ventricular ao ecocardiograma, 24±13 meses após TC. RESULTADOS: Após 30 dias, a HA estava presente em 58,5% dos pacientes (50% leve), enquanto na evolução tardia, a incidência da HA aumentou significativamente para 93% após um ano (85% moderada a grave). A creatinina sérica aumentou progressivamente do pré-TC (1,43±0,5mg/dl) até após um ano (1,83± 0,9 mg/dl), mantendo leve incremento até os 60 meses (2,4± 0,8mg/dl). Não houve correlação entre a HA, creatinina sérica e níveis de CL. O índice cardíaco aumentou na fase precoce, enquanto a resistência periférica diminuiu no início e aumentou significativamente aos 12 meses. Ao ecocardiograma, 54% dos pacientes apresentavam hipertrofia de ventrículo esquerdo com função normal. Dos 31 pacientes que faleceram durante a evolução, dois tiveram a causa mortis diretamente relacionada a HA. CONCLUSÃO: A HA em pacientes submetidos a TC em uso de CL ocorre precocemente, aumenta em prevalência e gravidade com o tempo e é mediada por aumento da resistência periférica, não se correlacionando com a nefrotoxicidade e com os níveis sangüíneos da CL, podendo agravar a insuficiência renal ou comprometer a longevidade do transplante, induzindo hipertrofia ventricular.

PURPOSE: To evaluate the progression of arterial hypertension (AH) and its consequences , in patients submitted to cardiac transplantation (CT) in use of cyclosporine (CY). METHODS: In 65 patients submitted to orthotopic CT, we evaluated blood pressure, serum creatinine and blood levels of CY before, 15 and 30 days, and 6, 12, 24, 48 and 60 months after CT; in 20 patients we analyzed cardiac index and systemic vascular resistance pre-CT, 15 and 30 days, 6 and 12 months after CT; in 33 patients, we studied anatomic and functional modifications by echocardiography, 24±13 months after CT. RESULTS: Thirty days after CT, AH was present in 58.5% (50% mild), and after one year, 93% of patients were hypertensives (85% moderate-to-severe), remaining unchanged during the rest of follow-up. The serum creatinine progressively increased, reaching values significantly higher than those pre-CT after one year, persisting with a mild increment until 60 months. Echocardiography showed left ventricle hypertrophy in 54% of patients, all of which had normal function. Two patients died as a direct consequence of hypertensive complications. CONCLUSION: AH in patients submitted to CT on CY use occurs early, increases in prevalence and severity during the follow-up and is mediated by an increase in vascular resistance. Also, the AH does not correlate to CY blood levels or nefrotoxicity, but it can impair renal function and compromise longevity of transplantation by inducing ventricular hypertrophy.