Cardiovascular findings in women suffering from Turner syndrome requesting oocyte donation.

BACKGROUND: Through oocyte donation (OD), women with Turner syndrome (TS) may achieve motherhood. However, this population has a high prevalence of cardiac malformations and carry a risk for aortic dissection that is increased by pregnancy. Until recently, the necessity for a specialized cardiac evaluation before pregnancy was underestimated as was the need for follow-up through adulthood. The aim of this study was to evaluate the follow-up (mainly cardiovascular) of women with TS requesting OD. METHODS: Disease monitoring since diagnosis and prior cardiac evaluations conducted out of our centre were assessed in 25 women with TS who requested OD. New cardiac evaluations using echocardiography and magnetic resonance imaging were performed by our specialized cardiologist in 18 of these patients. RESULTS: We observed that the medical follow-up of women with TS was often deficient throughout adulthood. Most of the prior cardiac evaluations performed by cardiologists not accustomed to women with TS, either before (n = 8) or when starting OD (n = 12), were considered normal. However, when revaluated by a cardiologist who is familiar with TS, seven women were diagnosed with a bicuspid aortic valve and thus excluded from OD. In addition, when appropriate screening was conducted by our referent cardiologist before OD no cardiac complication was observed during pregnancy or delivery. CONCLUSIONS: Careful follow-up, including cardiac evaluation, should be recommended for women diagnosed with TS, before and after puberty. Moreover, assessment of cardiovascular parameters by a cardiologist familiar with TS should be routinely repeated before undertaking OD.

P300 event-related potential in euthymic patients with bipolar disorder.

Auditory P300 event-related potential (ERP) and performance on Sustained Attention were evaluated in 24 euthymic bipolar patients and 38 healthy volunteers. There were no significant differences between groups, and performance in sustained attention had no significant influence in the P300 responses. P300 response might be driven by the presence of mood symptoms.

Brain natriuretic peptide blood levels in the differential diagnosis of dyspnea.

STUDY OBJECTIVES: In dyspneic patients without left ventricular enlargement, it may be difficult to differentiate between obstructive lung disease and diastolic heart failure. Determination of plasma brain natriuretic peptide (BNP) levels, known to increase with ventricular stretch, may be of clinical relevance in this situation. We compared the discriminant power of BNP blood levels and of echocardiography in patients with either chronic obstructive lung disease or diastolic heart failure. PATIENTS: Twenty-six New York Heart Association class III dyspneic patients with normal left ventricular systolic function were enrolled: 17 patients with chronic obstructive lung disease and 9 patients with unequivocal diastolic heart failure. RESULTS: Echocardiographic data were unable to accurately differentiate between the two groups, whereas BNP levels were significantly and markedly higher in patients with diastolic heart failure when compared to those with obstructive lung disease (224 +/- 240 pg/mL vs 14 +/- 12 pg/mL, p < 0.0001). CONCLUSIONS: These preliminary results warrant a prospective, large-scale evaluation of the value of BNP assay for determining diastolic dysfunction, a common cause of dyspnea in elderly patients, and differentiating it from other diagnoses such as obstructive lung disease.

Inflation of antishock trousers increases bronchial response to methacholine in healthy subjects.

We studied changes in lung volumes and in bronchial response to methacholine chloride (MC) challenge when antishock trousers (AST) were inflated at venous occlusion pressure in healthy subjects in the standing posture, a maneuver known to shift blood toward lung vessels. On inflation of bladders isolated to lower limbs, lung volumes did not change but bronchial response to MC increased, as evidenced by a greater fall in the forced expiratory volume in 1 s (FEV1) at the highest dose of MC used compared with control without AST inflation (delta FEV1 = 0.94 +/- 0.40 vs. 0.66 +/- 0.46 liter, P less than 0.001). Full inflation of AST, i.e., lower limb and abdominal bladder inflated, significantly reduced vital capacity (P less than 0.001), functional residual capacity (P less than 0.01), and FEV1 (P less than 0.01) and enhanced the bronchial response to MC challenge compared with partial AST inflation (delta FEV1 = 1.28 +/- 0.47 liter, P less than 0.05). Because there was no significant reduction of lung volumes on partial AST inflation, the enhanced bronchial response to MC cannot be explained solely by changes in base-line lung volumes. An alternative explanation might be a congestion and/or edema of the airway wall on AST inflation. Therefore, to investigate further the mechanism of the increased bronchial response to MC, we pretreated the subjects with the inhaled alpha 1-adrenergic agonist methoxamine, which has both direct bronchoconstrictor and bronchial vasoconstrictor effects.(ABSTRACT TRUNCATED AT 250 WORDS)

[Reading of the trials in cardiac insufficiency; therapeutic studies (phases II B and III)]. / Lecture des essais dans l'insuffisance cardiaque; études thérapeutiques (phases II B et III).

The object of therapeutic trials in cardiac failure is to improve the quality of life by controlling the principal symptoms which are dyspnoea and tiredness on effort and to improve survival. A critical review of therapeutic trials in cardiac failure must examine whether these two objectives have been attained and if they are attained simultaneously. In some cases, there is symptomatic improvement but with decreased life expectancy whereas in others, survival is prolonged without adequate control of symptoms. A critical analysis should examine the pertinence of these criteria of efficacy, and confirm that the problem of survival has been assessed, that reliable data has been provided, that the study population is representative of the real population of the patients to be treated, and finally, that the experimental protocol is realistic, i.e. a prospective study with analysis of intention to treat.

[Mechanisms of exertion dyspnea in cardiac insufficiency]. / Mécanismes de la dyspnée d'effort de l'insuffisance cardiaque.

Effort dyspnea in cardiac failure corresponds to a subjective perception of difficulty in breathing which is itself secondary to a disproportion between the central bulbar respiratory regulation and the level of pulmonary ventilation attained. In cardiac failure, this situation is the result of dysfunction of both the respiratory apparatus and the skeletal striated muscle during exercise. During exercise a rise in left ventricular end diastolic pressure causes: a reduction in pulmonary compliance. The bronchial and alveolar capillaries drain into the pulmonary veins. The congestion of these capillaries and the resulting oedema makes the lung "stiffer"; an increase in the resistances of the small airways due to direct compression by congested bronchial vessels. The physiological dead space increases: the respiratory muscles ventilate pulmonary zones which are not perfused to no benefit. The reduction of pulmonary compliance, the increased resistances of the airways and of the physiological dead space all contribute to increase the work of the respiratory muscles. In addition to these mechanical phenomena, there is greater stimulation of the respiratory centre in the brain stem by the metabolic abnormalities of the skeletal striated muscles. During effort, they rapidly function under anaerobic conditions and the resulting hyperproduction of lactate and carbon dioxide stimulates the respiratory centres.

[Adrenergic betareceptors and cardiac insufficiency]. / Bêtarécepteurs adrénergiques et insuffisance cardiaque.

Noradrenergic sympathetic tone is always increased in chronic left ventricular failure of which it is one of the main compensatory mechanisms. Beta-1-adrenergic stimulation increases the heart rate and left ventricular contractility. However, the efficacy of this "compensatory" mechanism is limited on the one hand by the energetic cost of inotropic stimulation and, on the other hand, by the phenomenon of desensitisation and down-regulation of myocardial beta-1-receptors during intense and prolonged noradrenergic stimulation as observed in chronic cardiac failure. These physiopathological concepts raise the question of the indications of drugs affecting betareceptors in cardiac failure. Positive inotropic beta-mimetics can only be used during short periods of acute decompensation: low dose betablocker therapy protects the betareceptors from the phenomenon of desensitisation and seem to exert a beneficial action in this way in some cases of cardiac failure; these preliminary results require confirmation by large scale controlled therapeutic trials. Finally, a new pharmacological class of drugs, the beta-1 adrenergic partial agonists, seems to be useful in the management of moderate degrees of cardiac failure due to ischemic heart disease.

[Vasomotility of the bronchial circulation in cardiac failure]. / Vasomotricité de la circulation bronchique dans l'insuffisance cardiaque.

The bronchial circulation is a physiological left-to-left shunt; the venous return of the bronchial arteries vascularising the intra-pulmonary bronchi drains directly into the left heart chambers. In cardiac failure, increased left ventricular filling pressures affects the bronchial circulation and causes stasis and congestion. Congestion of the arterial and venous bronchial microcirculation leads to thickening of the bronchial mucosa and submucosa, resulting in a tendency to obstruct small and medium-sized airways. The bronchial circulation can be explored indirectly in cardiac failure by the spirometric response to adrenergic agonists and antagonists administered by inhalation: bronchial vasomotor phenomena explain the symptomatology of cardiac asthma and also seem to play a role in the genesis of the dyspnoea of effort in patients with cardiac failure.

[Bartonella quintana endocarditis. Aortic localization and mitral valve abscess]. / Endocardite à Bartonella quintana. Localisation aortique et abcès mitral.

Bartonella Quintana is an exceptionally rare cause of endocarditis. The frequency of this condition is increasing and is probably underestimated because of the difficulty in diagnosing. The clinical context is that of socially deprived classes, the agent of transmission of the germ being body lice. The commonest valve affected is the aortic valve. This infection may also give rise to a mitral annulus abscess, as in this particular case. The diagnosis should be suspected in all cases of endocarditis with negative blood cultures in socially deprived patients as special diagnostic methods are required. Bartonellosis serology and drainage of effusions to perform a Gimenez stain to show intra-cellular bacteria and a polymerase chain reaction to sequence the nucleic acids, will give an accurate diagnosis. Similarly, the operative specimens should be sent to a specialised department for the culture of these bacteria (in France, the Marseille Ricketsioses Institute). The treatment of Bartonella endocarditis is very controversial. The usual recommendations are an association of betalactamines and an aminoside for one month and macrolides or tetracyclines. There is lack of consensus about follow-up. It would seem prudent to perform a clinical examination to search for a relapse of the infection and monthly serologies until they return to normal values.

[Pericardial and myocardial adiastole in rheumatoid polyarthritis]. / Adiastolie péricardique et myocardique au cours d'une polyarthrite rhumatoïde.

The authors describe a case of clinical, echocardiographic and haemodynamic adiastole in a man with severe rheumatoid arthritis with a previous history of pericardial effusion. The adiastole was mixed, fibrous pericarditis, confirmed by ultra fast CT scan and at surgery; myocardial adiastole was suspected on finding thickening of the ventricular walls (in the absence of hypertension and coronary artery disease) and, unfortunately, confirmed by the persistence of adiastole despite very satisfactory pericardectomy. The authors underline the involvement of the three cardiac tunics in rheumatoid arthritis and the value of different diagnostic methods in the differentiation between constrictive pericarditis and restrictive cardiomyopathy.

[How to search for a cardiac cause of an arterial embolism]. / Comment rechercher une cause cardiaque à un accident embolique artériel.

Owing to a very high-definition image, transesophageal echocardiography (TEE) allows screening lesions that are not detected by other examination techniques. Its superiority is especially obvious in the analysis of the atrial structure of the mitral valve and of the interatrial septum, therefore for the analysis of structures that are most commonly involved in embolic strokes. After history taking, a clinical examination, and the analysis of the electrocardiogram and of the thoracic X-rays, two cases are possible: 1) There is an indisputable emboligenic heart disease: atrial fibrillation (AF), mitral stenosis, bacterial endocarditis, or a valvular prosthesis. Systematic transthoracic cardiac ultrasonography (TTE) completes this assessment. 2) When the initial findings are negative, ETT being included in the assessment, the identification of a potential cardiac cause of embolism depends on: the degree of investigation implemented: Holter to check for paroxysmal AF, TEE knowing that the diagnostic efficiency of this examination is low when the initial assessment is negative; what is selected as an emboligene cardiac cause. In fact, in addition to commonly recognized causes called major causes, there are so-called minor abnormalities that are still ill-defined but are known to be associated with arterial embolism. For example, a patent foramen ovale or an aneurysm of the interatrial septum are very easily diagnosed with TEE, but the exact mechanism of embolism and the appropriate therapeutic attitude still remain to be defined for this type of pathology.

[Pregnancy and the heart]. / Coeur et grossesse.

Pregnancy causes important alterations in the cardiovascular haemodynamics which have important bearing in the case of pre-existing cardiac disease. In addition, cardiovascular medications may be contraindicated in pregnant women. With a better recognition of the specific interactions between pregnancy and cardiac conditions, it is now possible to allow pregnancies in numerous women with recognised cardiac diseases.

[Primary leiomyosarcoma of the pulmonary artery. Apropos of a case. Review of the literature]. / Léiomyosarcome primitif de l'artère pulmonaire. A propos d'un cas: revue de la littérature.

A 59-year-old woman hospitalised because of dyspnea and a heart murmur in a context of pyrexia was found to have evidence of obstruction of the pulmonary arterial system, clearly defined by ultrasonography, catheterisation and angiography and Imatron scan. The particular feature of this fifth reported case of pulmonary artery leiomyosarcoma is its documentation by transesophageal ultrasonography and tumor biopsy during catheterisation. Surgery with partial excision of the tumor was followed by survival for 6 months, bearing in mind the absence of chemo- or radiosensitivity of this type of tumor. Cases from the literature are reviewed.

[Bronchial hyperreactivity and heart failure]. / Hyperréactivité bronchique et insuffisance cardiaque.

A moderate to severe bronchial hyperresponsiveness to methacholine is frequently observed in patients with left heart failure. This bronchial obstruction can be prevented by inhalation of methoxamine, an alpha-adrenergic vasoconstrictor agent. The mechanisms of this hyperresponsiveness include a direct vasodilating effect of methacholine on bronchial vessels and a contractile effect of an airway smooth muscles. This bronchoconstriction is the consequence of a direct effect of methacholine and a contractile effect of an extravased mediators and of an increased vagal tone due to the stimulation a bronchial afferent nerves. The role of bronchial hyperresponsiveness in cardiac dyspnea is currently under investigations.

[The function of beta receptors in chronic left ventricular insufficiency]. / La fonction des récepteurs bêta au cours de l'insuffisance ventriculaire gauche chronique.

Hyperactivity of the noradrenergic sympathetic system is one of the essential "compensatory"mechanisms in chronic left ventricular failure. The ensuing stimulation of myocardial beta-adrenergic receptors results in an increase of heart rate and contractility which, to some extent, counterbalances the alteration of left ventricular function, but rapidly reaches its limits: the excessive shortening of diastoles and, mostly, the increase of myocardial oxygen demand neutralize the beneficial haemodynamic effect of beta-adrenergic stimulation, especially when ischaemia is the cause of the heart failure; the chronic exposure of adrenergic receptors to noradrenaline in high concentrations leads to desensitization, to a "down regulation" which primarily affects the beta 1 receptors and spares, at least partly, the myocardial beta 2 receptors which seem to play a quantitatively important inotropic role, particularly in chronic heart failure. These new data on the physiology of the cardiac noradrenergic system have major therapeutic consequences: in practice, the positively inotropic beta-stimulants can only be used for a short period in acute episodes of heart failure; - the use of beta-blockers in low doses is now considered in the treatment of some forms of heart failure; the mechanism of their therapeutic action remains controverted, and their long-term effectiveness in a large patient population is under study; - a new pharmacological class, beta-adrenoceptor partial agonists, seems to give satisfactory clinical and haemodynamic results in mode-rate heart failure, A wider clinical evaluation is needed to determine the therapeutic role of theses new pharmacodynamic agents.

Delayed-enhanced cardiac magnetic resonance imaging features in peripartum cardiomyopathy.

Peripartum cardiomyopathy (PPCM) is a rare disorder in which left ventricular systolic dysfunction and symptoms of heart failure occur in the peripartum period. Although cardiac magnetic resonance (CMR) is largely used for diagnosis and prognosis assessment in cardiomyopathies, its interest in PPCM is unknown. We reported two cases of patients with PPCM who underwent CMR. One patient had no CMR abnormality, while the second patient had several areas of myocardial delayed enhancement (MDE) on CMR images. During follow up, the patient with normal CMR was asymptomatic and had full recovery of cardiac function, whereas the patient with MDE was still symptomatic with persistence of a left ventricular dysfunction. CMR could have prognosis value in PPCM as demonstrated in other cardiomyopathies.

Pharmacological and therapeutic basis for combined administration of beta blockers and calcium channel blockers in the treatment of stable chronic angina.

Pharmacodynamics of beta-adrenergic blockers and dihydropyridines are potentially synergic in the treatment of angina pectoris. The anti-ischaemic effect of beta blockers is mainly the consequence of reductions in heart rate and inotropism, while DHPs promote afterload reduction and coronary vasodilation. Furthermore, beta blockers antagonise the possible dihydropyridines-induced reflex sympathetic activation. Despite these mechanistic considerations the results of clinical trials are not homogeneous. Differences in the assessment of the beta blocker-dihydropyridines connection are due to differences in trial design, dosage and formulation of both dihydropyridines and beta-blockers, and in baseline characteristics of the study population. The predominant finding is that a combination of a dihydropyridines and a beta blocker is not superior to either drug alone as a first step treatment of unselected patients with stable or unstable angina. In contrast, combination therapy is often efficacious when residual ischaemia is present under dihydropyridines or beta blocker monotherapy. That this combination is usually well tolerated thus appears to represent a useful treatment of severe angina pectoris. Combination of a non-dihydropyridines calcium antagonist such as diltiazem or verapamil with a beta blocker offers similar synergistic anti-ischaemic effects, but the addition of their negative chronotropic action may lead to severe bradycardia and thus limit its usefulness, especially in elderly patients with conduction disturbances.