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.

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.

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.

[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.

N-terminal pro-brain natriuretic peptide as a diagnostic marker of early pulmonary artery hypertension in patients with systemic sclerosis and effects of calcium-channel blockers.

OBJECTIVE: To evaluate N-terminal pro-brain natriuretic peptide (NT-proBNP) as a marker of early pulmonary artery hypertension (PAH) and to study changes in the levels of this marker following treatment with dihydropyridine-type calcium-channel blocker (DTCCB) in patients with systemic sclerosis (SSc). METHODS: We evaluated 40 consecutive SSc patients who had been hospitalized for followup care (mean +/- SD age 56 +/- 11 years and mean +/- SD duration of cutaneous disease 9 +/- 9 years; 27 with limited cutaneous and 13 with diffuse cutaneous disease) but who had no clinical symptoms of heart failure and had a normal left ventricular ejection fraction. At baseline, 10 patients had PAH, defined as a systolic pulmonary artery pressure (sPAP) >40 mm Hg, as measured by echocardiography. Levels of NT-proBNP were determined at baseline (after discontinuation of DTCCB treatment for 72 hours), after taking 3 doses of DTCCB following treatment reinitiation (assessment 1), and after 6-9 months of continuous DTCCB treatment (assessment 2) in the 20 patients who attended regular appointments (including the 10 patients with PAH at baseline). RESULTS: At baseline, 13 patients had high NT-proBNP values for their ages. High NT-proBNP levels identified patients with PAH with a sensitivity of 90%, a specificity of 90.3%, a positive predictive value of 69.2%, and a negative predictive value of 96%. The NT-proBNP level correlated with the sPAP (r = 0.44; P = 0.006). By assessment 1, the number of patients with PAH and high levels of NT-proBNP had decreased from 9 of 10 to 2 of 10 (P = 0.02). This decrease was partially sustained at assessment 2 (4 of 10 patients; P = 0.06). CONCLUSION: NT-proBNP is a useful biologic marker that can be used to diagnose early PAH in SSc patients without clinical heart failure. Measurement of NT-proBNP may be valuable for the evaluation of treatment with DTCCB and vasodilators in patients with PAH.

Recurrent cerebrovascular events associated with patent foramen ovale, atrial septal aneurysm, or both.

BACKGROUND: Patent foramen ovale and atrial septal aneurysm have been identified as potential risk factors for stroke, but information about their effect on the risk of recurrent stroke is limited. We studied the risks of recurrent cerebrovascular events associated with these cardiac abnormalities. METHODS: A total of 581 patients (age, 18 to 55 years) who had had an ischemic stroke of unknown origin within the preceding three months were consecutively enrolled at 30 neurology departments. All patients received aspirin (300 mg per day) for secondary prevention. RESULTS: After four years, the risk of recurrent stroke was 2.3 percent (95 percent confidence interval, 0.3 to 4.3 percent) among the patients with patent foramen ovale alone, 15.2 percent (95 percent confidence interval, 1.8 to 28.6 percent) among the patients with both patent foramen ovale and atrial septal aneurysm, and 4.2 percent (95 percent confidence interval, 1.8 to 6.6 percent) among the patients with neither of these cardiac abnormalities. There were no recurrences among the patients with an atrial septal aneurysm alone. The presence of both cardiac abnormalities was a significant predictor of an increased risk of recurrent stroke (hazard ratio for the comparison with the absence of these abnormalities, 4.17; 95 percent confidence interval, 1.47 to 11.84), whereas isolated patent foramen ovale, whether small or large, was not. CONCLUSIONS: Patients with both patent foramen ovale and atrial septal aneurysm who have had a stroke constitute a subgroup at substantial risk for recurrent stroke, and preventive strategies other than aspirin should be considered.

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.

[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.

[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.

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.

[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.

Atrial septal aneurysm and patent foramen ovale as risk factors for cryptogenic stroke in patients less than 55 years of age. A study using transesophageal echocardiography.

BACKGROUND AND PURPOSE: An association between atrial septal aneurysm and embolic events has been suggested. Atrial septal aneurysm has been shown to be associated with patent foramen ovale and, in some reports, with mitral valve prolapse. These two latter cardiac disorders have been identified as potential risk factors for ischemic stroke. The aim of this prospective study was to assess the role of atrial septal aneurysm as an independent risk factor for stroke, especially for cryptogenic stroke. METHODS: We studied the prevalence of atrial septal aneurysm, patent foramen ovale, and mitral valve prolapse in 100 consecutive patients < 55 years of age with ischemic stroke who underwent extensive etiological investigations. We compared these results with those in a control group of 50 consecutive patients. The diagnosis of atrial septal aneurysm and patent foramen ovale relied on transesophageal echocardiography with a contrast study and that of mitral valve prolapse, on two-dimensional transthoracic echocardiography. RESULTS: Stepwise logistic regression analysis showed that atrial septal aneurysm (odds ratio, 4.3; 95% confidence interval, 1.3 to 14.6; P = .01) and patent foramen ovale (odds ratio, 3.9; 95% confidence interval, 1.5 to 10; P = .003) but not mitral valve prolapse were significantly associated with the diagnosis of cryptogenic stroke. The stroke odds of a patient with both atrial septal aneurysm and patent foramen ovale were 33.3 times (95% confidence interval, 4.1 to 270) the stroke odds of a patient with neither of these cardiac disorders. For a patient with atrial septal aneurysm of > 10-mm excursion, the stroke odds were approximately 8 times the stroke odds of a patient with atrial septal aneurysm of < 10 mm. CONCLUSIONS: This study shows that atrial septal aneurysm and patent foramen ovale are both significantly associated with cryptogenic stroke and that their association has a marked synergistic effect. Atrial septal aneurysms of > 10-mm excursion are associated with a higher risk of stroke.

[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.

Patent foramen ovale: is stroke due to paradoxical embolism?

BACKGROUND AND PURPOSE: A patent foramen ovale has been reported to be significantly more frequent in young stroke patients than in matched control subjects, and paradoxical embolism has been suggested as the main mechanism of stroke in this situation. The present study was designed to test this hypothesis. METHODS: Sixty-eight consecutive patients under 55 years of age presenting with an ischemic stroke had an extensive workup, including transesophageal echocardiography with contrast. We compared the prevalence of criteria for the diagnosis of paradoxical embolism in patients with and without a patent foramen ovale. RESULTS: A patent foramen ovale was found in 32 patients (47%). A Valsalva-provoking activity was present at stroke onset in six patients with a patent foramen ovale and in eight patients with no patent foramen ovale (chi 2 = 0.1, nonsignificant). Clinical/radiological features suggestive of an embolic mechanism were not more frequent in patients with a patent foramen ovale. Clinical evidence of deep vein thrombosis was present in one patient with a patent foramen ovale and in none of the others. No occult venous thrombosis was found in a subgroup of patients with a patent foramen ovale and no definite cause for stroke who underwent venography (n = 13). CONCLUSIONS: Our results do not support the hypothesis that paradoxical embolism is the primary mechanism of stroke in patients with a patent foramen ovale.

Effect of airway blood flow on airflow.

Resistance to gas flow of an airway is a function of both airway smooth muscle tone and thickness of the airway wall internal to the outer ring of airway smooth muscle. Schematically, the increase in airway resistance caused by shortening of airway smooth muscle may be potentiated by a concomitant increase in airway wall thickness caused by vasodilation of the bronchial vessels and/or microvascular leakage. Conversely, bronchial vasoconstriction may limit to some extent the increase in resistance to gas flow caused by airway smooth muscle shortening and/or congestion and edema of the airway wall. Many endogenous paracrine mediators, putatively involved in asthma and bronchial hyperresponsiveness, have both bronchomotor and vascular effects. The overall effects on resistance to airflow of endogenous or exogenous agents depend not only upon pre-existing airway smooth muscle tone and pre-existing condition of bronchial vessels but also upon two factors that facilitate microvascular leakage, namely, inflammation of the airway wall and outflow pressure of the bronchial circulation, which is close to left atrial pressure.

[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.

Improvement in exercise performance by inhalation of methoxamine in patients with impaired left ventricular function.

BACKGROUND: Bronchial hyperresponsiveness to cholinergic stimuli such as the inhalation of methacholine is common in patients with impaired left ventricular function. Such hyperresponsiveness is best explained by cholinergic vasodilation of blood vessels in the small airways, with extravasation of plasma due to high left ventricular filling pressure. Because this vasodilation may be prevented by the inhalation of the vasoconstrictor agent methoxamine, we studied the effect of methoxamine on exercise performance in patients with chronic left ventricular dysfunction. METHODS: We studied 19 patients with a mean left ventricular ejection fraction of 22 +/- 4 percent and moderate exertional dyspnea. In the first part of the study, we performed treadmill exercise tests in 10 patients (group 1) at a constant maximal workload to assess the effects of 10 mg of inhaled methoxamine on the duration of exercise (a measure of endurance). In the second part of the study, we used a graded exercise protocol in nine additional patients (group 2) to assess the effects of inhaled methoxamine on maximal exercise capacity and oxygen consumption. Both studies were carried out after the patients inhaled methoxamine or placebo given according to a randomized, double-blind, crossover design. RESULTS: In group 1, the mean (+/- SD) duration of exercise increased from 293 +/- 136 seconds after the inhalation of placebo to 612 +/- 257 seconds after the inhalation of methoxamine (P = 0.001). In group 2, exercise time (a measure of maximal exercise capacity) increased from 526 +/- 236 seconds after placebo administration to 578 +/- 255 seconds after methoxamine (P = 0.006), and peak oxygen consumption increased from 18.5 +/- 6.0 to 20.0 +/- 6.0 ml per minute per kilogram of body weight (P = 0.03). CONCLUSIONS: The inhalation of methoxamine enhanced exercise performance in patients with chronic left ventricular dysfunction. However, the improvement in the duration of exercise at a constant workload (endurance) was much more than the improvement in maximal exercise capacity assessed with a progressive workload. These data suggest that exercise-induced vasodilation of airway vessels may contribute to exertional dyspnea in such patients. Whether or not inhaled methoxamine can provide long-term benefit in patients with heart failure will require further study.