Successful laparoscopic operation of bilateral pheochromocytoma in a patient with Beckwith-Wiedemann syndrome.

We report the case of a 20-year-old female patient with Beckwith-Wiedemann syndrome presenting with high blood pressure and bilateral adrenal pheochromocytoma successfully removed with laparoscopy in the same time. To our knowledge, the present case is the first observation of a bilateral pheochromocytoma occurring in the Beckwith-Wiedemann syndrome. It provides further support for a genetic anomaly in this condition. Our case also indicates the interest of laparoscopy for the surgical treatment of adrenal pheochromocytoma, even in bilateral tumors.

[Sensitivity and specificity of criteria of left ventricular hypertrophy for predicting cardiovascular complications in the Bordeaux cohort of hypertensive patients]. / Sensibilité et spécificité des différents critères d'hypertrophie ventriculaire gauche pour la prédiction des complications cardiovasculaires dans une cohorte d'hypertendus bordelais.

Left ventricular hypertrophy (LVH) is associated with an increased risk of cardiovascular complications independently of other known risk factors. Since 1983, we have followed up a cohort of initially untreated hypertensive patients with echocardiographic measurements of left ventricular mass (LVM). We analyzed the data on 474 patients with more than five years' follow-up to assess the prognostic value of LVM and the sensitivity and specificity of the different ways to index for LVM through ROC curves. Forty patients were lost to follow-up. The mean follow-up period was 89 +/- 31 months. A cardiovascular complication was recorded in 40 individuals. There was a strong link between increased LVM and the occurrence of complications. Indexation by body surface area or height to the power 2.7 give the [table: see text] greatest area under the curve (AUC) to discriminate between patient with or without cardiovascular events. To get the same sensitivity in females and in males for the prediction of future events, lower cut-off must be used in females.

Prognostic significance of blood pressure measured on rising.

Previous works using ambulatory blood pressure (BP) monitoring demonstrated that independently of the mean level of BP, the variability in BP, or the day-night range, could have prognostic significance. We have also found that the value of BP on rising in the morning is strongly correlated with left ventricular mass of hypertensive individuals independently of the 24-h value. In the present study, we sought its predictive value for cardiovascular complications in a cohort of hypertensive patients. The population studied belongs to a cohort of initially untreated hypertensive patients recruited since 1983 and followed for more than 5 years. Patients were then treated and followed by their family doctor. At entry, all patients were equipped with a device to measure ambulatory BP. They were requested to trigger a measurement manually on rising in the morning (arising BP). The data on their outcome were collected by a physician unaware of the initial state of the patients. A total of 256 patients have been followed up for 5 years or more, 19 were lost to follow-up. The mean follow-up period was 84 +/- 29 months. Cardiovascular complications were recorded in 23 individuals. The arising systolic BP (SBP) was significantly higher in the group who presented a complication. In a stepwise discriminant analysis including age, office, fitting, arising and 24-h average SBPs only age and arising SBP entered the equation. In conclusion, the single BP value measured by an ambulatory device on rising in the morning seems more discriminant of future cardiovascular events than the value of BP measured on fitting the device or the average of three measurements taken under standardised conditions in the hospital or office.

Ambulatory measurement of the QKD interval normalized to heart rate and systolic blood pressure to assess arterial distensibility--value of QKD(100-60).

BACKGROUND: Reduced distensibility of large arteries plays an important role in cardiovascular risk. Determination of the QKD interval during the ambulatory measurement of blood pressure enables calculation of an index of arterial distensibility. This index, the QKD(100-60), is the theoretical value of QKD at systolic blood pressure of 100mmHg and heart rate of 60bpm obtained from the linear bivariate relationship linking QKD, systolic blood pressure and heart rate on a hundred successive values measured over 24h. This study was designed to examine the relationship between QKD and QKD(100-60) on heart rate and systolic function of the left ventricle, the two parameters governing the pre-ejection time which is part of the QKD interval. METHODS AND RESULTS: In a population of 203 untreated hypertensive patients having benefited from an ambulatory measurement of blood pressure over 24h with QKD monitoring and an M-mode echocardiographic recording of the left ventricle, we found that although mean QKD was linked to heart rate and systolic function of the left ventricle, QKD(100-60) was not. It fell significantly with age, and to a greater extent in the sustained hypertensives than in white-coat hypertensives. CONCLUSION: QKD(100-60) constitutes an index of arterial distensibility independent of the pre-ejection time. As an adjunct to the ambulatory measurement of blood pressure, its determination is simple and completely automatic, thus eliminating observer bias.

Prognostic value of left ventricular mass and its evolution during treatment in the Bordeaux cohort of hypertensive patients.

Left ventricular hypertrophy (LVH) is associated with an increased risk of cardiovascular complications independently of other known risk factors, but so far the predictive value of its evolution under treatment has been studied relatively little. Since 1983 we have followed up a cohort of initially untreated hypertensive patients with echocardiographic measurements of left ventricular mass (LVM). We analyzed the data on 474 patients with more than 5 years of follow-up to assess the prognostic value of LVM and its evolution during treatment for high blood pressure. A total of 40 patients were lost to follow-up. The mean follow-up period was 89 +/- 31 months. A cardiovascular complication was recorded in 40 individuals. There was a strong link between increased LVM and the occurrence of complications (P < .001). At least a second determination of LVM was performed in 311 patients, and the last value before the occurrence of any complication (60 +/- 38 months after the initial examination) was retained. In this subgroup, 28 patients presented with a cardiovascular event. There was a trend toward a reduction of the incidence of events in the group with a regression of LVH as compared to the group with persistent LVH, but there was no difference when patients were split into quartiles according to LVM evolution between baseline and follow-up. Thus, the reduction of LVM on treatment was not a good marker of future cardiovascular events and its seems at least premature to say that LVM fulfils all conditions for a surrogate end point in the evaluation of morbidity/ mortality in the hypertensive.

[Surgical evaluation of transthoracic tridimensional echocardiography in the anatomic study of atrial septal defect]. / Validation chirurgicale de l'échocardiographie tridimensionnelle transthoracique pour l'évaluation anatomique des communications interauriculaires.

The closure of atrial septal defects by interventional catheterisation requires an accurate assessment of their morphology and anatomical relationships. This study evaluated transthoracic three-dimensional echocardiography for the selection of atrial septal defects accessible to an occlusive prosthesis. The transthoracic three-dimensional echocardiographic measurements of 17 patients (4 to 55 years) with ostium secundum atrial septal defects were compared with those of the surgeon in a prospective study. The maximal diameters of the defect, the height of the interatrial septum, the distances to the superior vena cava (postero-superior border) and inferior vena cava (postero-inferior border), to the coronary sinus and the tricuspid valve were measured as a reconstruction of the interatrial septum seen from the right atrium. The aortic border was measured from a three-dimensional view from the left atrium. Thirteen of the 17 investigations (76%) were exploitable. The diameters of the defect varied during the cardiac cycle (p = 0.0002). Ther correlations between the surgical and echocardiographic measurements varied from 0.82 for the maximal diameter to 0.6 for the postero-inferior limits. Three-dimensional echocardiography is capable of detecting all the contra-indications of an occlusive prosthesis: 2 inadequate postero-inferior and 1 inadequate aortic borders, 9 maximal diameters which were too large, 3 insufficiently high atrial septa, 1 double atrial septal defect. The coronary sinus was only visualised in 1 case. Transthoracic three-dimensional echocardiography is a non-invasive technique capable of improving the selection of atrial septal defects for interventional closure. The transoesophageal approach should be reserved for candidates selected by the transthoracic investigation for the detection of small structures (coronary sinus) and when the transthoracic window is poor.