Objective-To determine the prognostic value of exercise testing, valve area, and maximum transaortic pressure gradient in asymptomatic patients with aortic valve stenosis. Setting-The outpatient service of a tertiary referral centre for cardiology. Design-Prospective clinical study. Patients-66 consecutive patients with isolated severe aortic stenosis (aortic valve area < 1.0 cm 2 ) were selected over a 58 month period. Mean (SD) follow up was 14.77 (11.93) months. Interventions-At the initial visit Doppler echocardiography and exercise testing were performed to evaluate ST segment depression and the development of symptoms of aortic stenosis, ventricular arrhythmia, or inadequate rise of systolic blood pressure during exercise. Follow up clinical examinations were performed every three months thereafter to record the onset of symptoms. Main outcome measures-Sudden death or the development of symptoms. Results-Eight patients developed dizziness during exercise testing but made a rapid and spontaneous recovery. No other complications of exercise testing occurred. Survival curves, with or without the occurrence of end point events for the variables studied, showed significant diVerences for positive versus negative exercise testing (p = 0.0001) and aortic valve area < 0.7 cm 2 v > 0.7 cm 2 (p = 0.0021). There was no relation between the end points and transaortic gradient (p = 0.6882). In multivariate analysis, a hazard ratio of 7.43 was calculated for patients with a positive versus a negative exercise stress test. Although asymptomatic in daily life, 6% of the patients (4/66) experienced sudden death; all these had a positive exercise test and an aortic valve area of < 0.6 cm 2 . Conclusions-Exercise testing is safe and is of prognostic value in asymptomatic patients with aortic stenosis. (Heart 2001;86:381-386)

Langerhans Cell Histiocytosis (LCH) is a rare disorder with a great variety of clinical manifestations. The purpose of this retrospective study was to evaluate the pattern and the long-term course of clinical, laboratorial and radiological findings in pediatric-onset LCH. We reviewed 46 children with histological diagnosis of LCH. Ten children (22%) showed endocrine disorders. Central diabetes insipidus (DI) was observed in all ten patients; GH deficiency was confirmed in four and hypogonadism in two children. There were no adrenal, prolactin or thyroid axis abnormalities. Obesity was observed in three patients. Eight patients showed soft tissue infiltration and five bone involvement. The MRI showed a lack of posterior pituitary bright spot in all DI patients; infundibular infiltration (II) associated or not with sellar or supra-sellar mass was observed in 4 patients. We conclude that the investigation of LCH, a multi-systemic disease, should include central nervous system images. The presence of II and/or DI should raise the diagnosis of LCH. Complete endocrine evaluation, allowing an early hormone therapy, is required to obtain a better quality of life in children with LCH.

Moyamoya disease (MMD) is an uncommon cerebrovascular disorder characterized by progressive stenosis of the terminal portion of the internal carotid artery and its main branches. Direct and indirect bypass techniques have been devised with the aim of promoting neoangiogenesis. The current study aimed to investigate the role of multiple cranial burr hole (MCBH) operations in the prevention of cerebral ischemic attacks in children with MMD. Seven children suffering from progressive MMD were submitted to the MCBH and arachnoid opening technique. Ten to 20 burr holes were drilled in the fronto-temporo-parieto-occipital area of each hemisphere in each patient, depending on the site and extent of the disease. All patients were evaluated pre- and postoperatively by means of Barthel index (BI), CT, MR, angio-MR, and angiography. Patients had no recurrence of ischemic attacks postoperatively. Neoangiogenesis was observed in both hemispheres. One patient developed a persistent subdural collection after surgery, thus requiring placement of a subdural-peritoneal shunt. Postoperative BI was statistically significantly improved (P=0.02). This report suggests that MCBH for revascularization in MMD is a simple procedure with a relatively low risk of complications and effective for preventing cerebral ischemic attacks in children. In addition, MCBH may be placed as an adjunct to other treatments for MMD.

objective:To evaluate the effect of smoking on blood pressure trends during a 24-hour period, by analyzing the parameters of the ambulatory blood pressure monitoring (ABPM). Methods:The results of 289 ABPM tests conducted on patients classified as smokers or nonsmokers were studied. The parameters analyzed were: mean 24-hour, daytime and nighttime systolic and diastolic blood pressure readings; nocturnal dipping of systolic and diastolic pressures and blood pressure load. The patients were classified in four groups according to whether or not they used antihypertensive medication: 1A -nonsmokers using medication; 1B -smokers using medication; 2A -nonsmokers not using medication; and 2B -smokers not using medication. Variables were expressed as minimum, maximum, median, mean and standard deviation values. Univariate analysis was used for comparing the smoking and nonsmoking groups. The significantly different variables from the groups were selected using multivariate analysis. The significance level adopted was 5%.results: Mean daytime systolic and diastolic blood pressures were significantly higher in the smokers, regardless of whether or not they used antihypertensive medication. Mean nocturnal blood pressure readings were similar between smokers and nonsmokers. Mean 24-hour systolic blood pressure readings were significantly higher in the smokers, regardless of whether or not they used antihypertensive medication. Nocturnal dipping was similar for all groups. Blood pressure loads were consistently and significantly higher in the smokers regardless of medication use.conclusion: Mean daytime systolic and diastolic blood pressure readings were consistently higher in the smokers when compared to nonsmokers regardless of antihypertension medication use. Nocturnal dipping was similar for smokers and nonsmokers.

Plástica da valva mitral em pacientes consecutivos. Como é a evolução tardia?: avaliação clínica e ecocardiográfica

Rev. Bras. Gir. Gardiovasc., 8(2} : 163-166, 1993.RESUMO: O aneurisma subanular mitral é doença pouco conhecida em nosso país. Foi descrita, inicialmente, e é vista com maior freqüência na população negra das regiões sul e oeste da África. Parece relacionar-se a uma fraqueza da parede ventricular na área de implantação da cúspide posterior da valva mitral, levando à insuficiência dessa valva. É relatado o caso de homem de 20 anos, branco, com queixa de dispnéia e taquicardia. Foi internado devido a evolução para classe funcional IV. Após compensação clínica, apresentava, ainda, sopro sistólico {+++/++++} em área mitral. A radiografia de tórax mostrou aumento da área cardíaca {+++/++++} às custas de átrio e ventrículo esquerdo. O ecocardiograma, além do aumento dessas câmaras, revelou dilatação aneurismática na região posterior do ventriculo esquerdo, justa-valvar em relação à valva mitral, com sinais de incompetência severa, assim como a cineangiocardiografia. Submetido a cirurgia em 20/10/86, quando foi realizada ressecção do aneurisma, utilizando-se placas de pericárdio bovino para reconstrução. Foi feita, também , troca de valva mitral por válvula biológica. O paciente evoluiu bem, tendo recebido alta hospitalar no oitavo dia de pós-operatório. Atualmente, é assintomático, o ecocardiograma de controle revelou correção cirúrgica satisfatória.

, sendo a principal causa de óbito o baixo débito cardíaco. As principais complicaçóes imediats foram : baixo débito cardíaco, arritmias e sangramento. Noventa por cento dos pacientes encontravam-se em classe funcional (NYHA) III e IV no pré-operatório, evoluindo para as classes funcionais I e II em 89% das trocas aórticas de 82% das trocas mitrais . A curva atuarial de sobrevida,em 5 semestres , foi, para a posição mitral , de 85,7% e, para a aórtica, de 91 ,3%. Os autores concluem que os cuidados de técnica, a proteção miocárdica e o tipo de prótese utilizada foram os responsáveis pelos resultados bastante satisfatórios.

Patients with significant aortic stenosis even without symptoms, have a low survival rate and the exercise test is a good prognostic factor during follow-up.