Treatment of heart failure with preserved systolic function.

Heart failure is a major public health problem. Heart failure with preserved systolic function (HF-PSF) is a common form, which is difficult to diagnose. Results of recent studies show that HF-PSF has a poor prognosis, with an annual survival rate similar to that of heart failure with left ventricular systolic dysfunction. Despite these findings, the therapeutic management of HF-PSF is not clearly defined. We will discuss in this review of the literature the current therapeutic management of HF-PSF, including the role of precipitating factors such as hypertension, myocardial ischaemia and supraventricular arrhythmias, and the main results of epidemiological registries and randomized controlled clinical trials in this disease. Only four large therapeutic trials have assessed the impact of different classes of drugs (digoxin, angiotensin II converting enzyme inhibitors, angiotensin II receptors type I blockers and beta-blockers) on morbidity and mortality in HF-PSF. Results of these trials are disappointing. Apart from the beta-blockers, the other three classes of drugs did not show benefit on the outcome of the disease. Moreover, the results of the beta-blocker trial are controversial as a mixed population of heart failure with and without preserved systolic function was studied. Finally, the current therapeutic management of patients with HF-PSF is still based on our pathophysiological knowledge: education, low salt diet, diuretics, slowing heart rate and controlling triggering factors. Other large randomized controlled multicenter trials, which may help us in the understanding of HF-PSP and its therapeutic management, are ongoing.

[Microalbuminuria and urinary albumin excretion: French guidelines]. / Microalbuminurie et excrétion urinaire d'albumine: recommandations pour la pratique clinique.

UNLABELLED: Measurement of urinary albumin excretion (UAE) may be done on a morning urinary sample or on a 24 hour-urine sample. Values defining microalbuminuria are: - 24-hour urine sample: 30-300 mg/24 hours - Morning urine sample: 20-200 mg/mL or 30-300 mg/g creatinine or 2.5-25 mg/mmol creatinine (men) or 3.5-35 mg/mol (women). - Timed urine sample: 20-200 mug/min. The optimal use of semi-quantitative urine test-strip is not clearly defined. It is generally believed that microalbuminuria reflects a generalized impairment of the endothelium; however, no definite proof has been shown in humans. In diabetic subjects, microalbuminuria is a marker of increased risk of cardiovascular (CV) and renal morbidity and mortality in type 1 and type 2 diabetic subjects. The increase in UAE during follow-up is also a marker of CV and renal risk in type 1 and type 2 diabetic subjects; its decrease during follow-up is associated with lower risks. In non-diabetic subjects, microalbuminuria is a marker of increased risk for diabetes mellitus, deterioration of the renal function, CV morbidity and all-cause mortality. It is a marker of increased risk for the development of hypertension in normotensive subjects, and is associated with unfavorable outcome in patients with cancer and lymphoma. Persistence or elevation of UAE overtime is associated with deleterious outcome in some hypertensive subjects. Measurement of UAE may be recommended in hypertensive subjects with 1 or 2 CV risk factors in whom CV risk remains difficult to assess, and in those with refractory hypertension: microalbuminuria indicates a high CV risk and must lead to strict control of arterial pressure. Studies focused on microalbuminuria in non-diabetic, non-hypertensive subjects are limited; most of them suggest that microalbuminuria predicts CV complications and deleterious outcome as it is in diabetic or hypertensive subjects. Subjects with a history of CV or cerebrovascular disease have an even greater CV risk if microalbuminuria is present than if it is not; however, in all cases, therapeutic intervention must be aggressive regardless of whether microalbuminuria is present or not. It is not recommended to measure UAE in non-diabetic non-hypertensive subjects in the absence of history of renal disease. Monitoring of renal function (UAE, serum creatinine and estimation of GFR) is annually recommended in all subjects with microalbuminuria. MANAGEMENT: in patients with microalbuminuria, weight reduction, sodium restriction (< 6 g/day), smoking cessation, strict glucose control in diabetic subjects, strict arterial pressure control are necessary; in diabetic subjects: use of maximal doses of ACEI or ARB are recommended; ACEI/ARB and thiazides have synergistic actions on arterial pressure and reduction of UAE; in non-diabetic subjects, any of the five classes of anti-hypertensive medications (ACEI, ARB, thiazides, calcium channel blockers or beta-blockers) can be used.

Microalbuminuria and urinary albumin excretion: French clinical practice guidelines.

Urinary albumin excretion (UAE) may be assayed on a morning urinary sample or a 24 h-urine sample. Values defining microalbuminuria are: 1) 24-h urine sample: 30-300 mg/24 h; 2) morning urine sample: 20-200 mg/ml or 30-300 mg/g creatinine or 2.5-25 mg/mmol creatinine (men) or 3.5-35 mg/mmol (women); 3) timed urine sample: 20-200 mug/min. The optimal use of semi-quantitative urine test-strip is not clearly defined. It is generally believed that microalbuminuria reflects a generalized impairment of the endothelium; however, no definite proof has been obtained in humans. IN DIABETIC SUBJECTS: Microalbuminuria is a marker of increased risk of cardiovascular (CV) and renal morbidity and mortality in type 1 and type 2 diabetic subjects. The increase in UAE during follow-up is associated with greater CV and renal risks in type 1 and type 2 diabetic subjects; its decrease during follow-up is associated with lower risks. IN NON-DIABETIC SUBJECTS: Microalbuminuria is a marker of increased risk for diabetes mellitus, deterioration of renal function, CV morbidity and all-cause mortality. It is a marker of increased risk for the development of hypertension in normotensive subjects, and is associated with unfavorable outcome in patients with cancer and lymphoma. Persistence of elevated UAE during follow-up is associated with poor outcome in some hypertensive subjects. Measurement of UAE may be recommended in hypertensive medium-risk subjects with 1 or 2 CV risk factors in whom CV risk remains difficult to assess, and in those with refractory hypertension: microalbuminuria indicates a high CV risk and must lead to strict control of arterial pressure. Studies focused on microalbuminuria in non-diabetic non-hypertensive subjects are limited; most of them suggest that microalbuminuria predicts CV complications and deleterious outcome. Subjects with a history of CV or cerebrovascular disease have an even greater CV risk if microalbuminuria is present than if it is not; however, in all cases, therapeutic intervention must be aggressive regardless of whether microalbuminuria is present or not. It is not recommended to measure UAE in non-diabetic non-hypertensive subjects in the absence of history of renal disease. Monitoring of renal function (UAE, serum creatinine and estimation of GFR) is recommended annually in all subjects with microalbuminuria. MANAGEMENT: In patients with microalbuminuria, weight reduction, sodium restriction (<6 g per day), smoking cessation, strict glucose control in diabetic subjects, strict arterial pressure control are necessary; in diabetic subjects: use of maximal doses of angiotensin-converting enzyme inhibitors (ACEI) or angiotensin receptor blockers (ARB) are recommended; ACEI/ARB and thiazides have synergistic actions on arterial pressure and reduction of UAE; in non-diabetic subjects, any of the five classes of anti-hypertensive medications (ACEI, ARB, thiazides, calcium channel blockers or beta-blockers) can be used.

Oxidative stress implication in a new phenotype of amyotrophic quadricipital syndrome with cardiac involvement due to lamin A/C mutation.

This study aimed at evaluating OS in an amyotrophic quadricipital syndrome with cardiac impairment in a family of 80 members with a mutation in lamin A/C gene. Twelve patients had cardiac involvement (5 cardiac and skeletal muscles impairment). OS was evaluated in blood samples (thiobarbituric acid-reactive substances (TBARS), carbonylated proteins (PCO)) 6 "affected patients" with phenotypic and genotypic abnormalities without heart failure and 3 "healthy carrier" patients. OS was higher in affected patients than in healthy, as shown by the higher TBARS and PCO values. Patients with cardiac and peripheral myopathy exhibited a higher OS than patients with only cardiac disease (TBARS: 1.73 +/- 0.05 vs. 1.51 +/- 0.04 mmol/l (p = 0.051), PCO: 2.73 +/- 0.34 vs. 0.90 +/- 0.10 nmol/mg protein (p = 0.47)), and with healthy carriers patients (TBARS: 1.73 +/- 0.05 vs. 1.16 +/- 0.14 mmol/l (p = 0.05), PCO: 2.73 +/- 0.34 vs. 0.90 +/- 0.20 nmol/mg protein (p = 0.47)). OS may thus contribute to the degenerative process of this laminopathy. ROS production occurs, prior to heart failure symptoms. We suggest that the extent activation may also promote the variable phenotypic expression of the disease.

Heart failure with preserved systolic function: a diagnostic algorithm for a pragmatic definition.

Heart failure is a major health problem which often concerns the elderly. Prevalence of heart failure with preserved systolic function is increasing and varies from 40 to 50%. In the literature, and in the large epidemiological studies, it is commonly designed with the term of "diastolic heart failure", even if a precise analysis of diastolic function is not performed. A diagnostic algorithm is proposed in order to better define the concept of heart failure with preserved systolic function. It consists of seven steps from symptoms and clinical signs to the echocardiographic analysis of diastolic function, in order to confirm the definition of heart failure with preserved systolic function.

[Neurological aspects of Fabry's disease]. / Manifestations neurologiques de la maladie de Fabry.

Fabry disease is a rare X-linked disorder caused by deficient activity of the lysosomal enzyme alpha-galactosidase A. Progressive accumulation in lysosomes of the undegraded glycosphingolipids leads to a multi-system disease with dermatological, ocular, renal, cardiac, and neurological manifestations. Peripheral nerve involvement, neuropathic pain and chronic acroparesthesiae, are frequent and early-onset signs revealing the disease. They are due to the involvement of small nerve fiber, thus explaining the normality of electroneuromyography. Cochleo-vestibular and autonomic nervous system involvement is frequent. Besides rare aseptic meningitis, central nervous system involvement is essentially represented by cerebrovascular events (stroke, transient ischemic attack). Affecting essentially the posterior circulation, their etiologies have to be clarified: progressive stenosis of small vessels with globotriasocylceramide deposits, arterial remodeling, endothelial dysfunction, pro-thrombotic state, cerebral hypoperfusion consecutive to dysautonaumy, cardiac embolism. MRI shows numerous silent lesions, increasing with age, mainly in small perforant arteries (periventricular white matter, brainstem, cerebellum, basal ganglia). Pulvinar calcifications, due to an increase in cerebral hyperperfusion, could be specific of Fabry disease. Positon tomography analysis shows a reduced cerebral flow velocity and impaired cerebral autoregulation, secondary to the glycosphingolipid storage in vascular endothelial cells. Enzyme replacement therapy has to be carefully monitored.

[Has the obligation to inform patients changed our mode of practice?]. / L'information du patient a-t-elle modifié notre comportement?

From January 2000, the Council of State has harmonised the jurisprudence with the Court of Appeal, changing the responsibility of medical practitioners by requiring them to provide proof that information was both given and understood by their patients. This obligation to inform patients raises several questions: who should give the information? to whom should the information be addressed? how can proof of this information be provided? what should the information be? The authors sent a questionnaire to practicing cardiologists by the internet site of the French Society of Cardiology from the 1st December 2002 to 15th January 2003. Three hundred and thirty-two replies were received of which 305 could be exploited. The activities of the cardiologists who replied were mainly in public hospitals (51.8%), private (18.2%) or mixed (30%). Patient information was mainly performed before invasive procedures, especially coronary angiography (90%) or cardiac pacing (77.3%). On the other hand, it was less commonly undertaken before exercise stress tests (63.2%) or transoesophageal echocardiography (61.4%), although these percentages are much higher than those recorded during previous enquiries in 2000 and 2001. The information given was, in the large majority of cases, that proposed by the French Society of Cardiology and it was usually the practitioner who ordered the investigation who informed the patient (45.4%). In 2002, the role of the nurse was much greater as the nurse informed the patient in 27.2% of cases. The patient was generally given the information the day before the procedure was carried out (74.1%) with complementary information (90.7%), and less than 1% of patients declined the investigation under these conditions. In order to provide proof of patient information, the practitioner usually required the patient's signature (58.3% of cases); less commonly, the referring physician was informed by letter (13.9% of cases) or a note was made in the patient's file (33.9% of cases). The new requirements for patient information have changed medical practice in nearly 53.5% of cases. Finally, although patient information is considered to be part of the normal patient-doctor relationship in most cases (42.7%), doctors thought that patients interpreted this procedure as a cover for the medical team in 18.2% of cases. The information bases most commonly used to determine the methods of informing patients and the nature of the information to be provided were medical reviews (38.9%) or the internet (30.5%). The authors conclude that patient information is carried out before complementary cardiological investigations. The new laws of the Code of Public Health are not well known. Finally, the proof of patient information is not easily provided and the majority of cardiologists request written patient consent, which is not a legal requirement.

[Patient information and the cardiologist]. / L'information du patient et le cardiologue.

AIM OF THE STUDY: The patient's information prior to paraclinical testings is a part of the medical deontology and takes on increasing legal importance since new laws. METHODS: From December 2001 to January 2002, we administered to cardiologists through the website of the French Society of Cardiology a questionnaire in order to determine the way the information is dispensed to patients and to compare the results to the survey performed in 2000. RESULTS: Among the 293 answers obtained, 243 were utilizable. The answers were obtained from cardiologists working on private medicine (27.5%), public medicine (52.8%) or mixed (19.7%). Information was more frequently dispensed for invasive procedures: coronary angiography (92.2%), cardiac pacing (76.8%) than non invasive assessments: transesophageal echocardiography (47.6%) and treadmill test (44.7%). The most frequent information document given to patients was the one edited by the French Society of Cardiology (71.6%). In the great majority of cases, there is the prescribing cardiologist (35.9%) and/or the one performing the assessment who dispenses the information, generally the day prior the examination (73.5%) with additive explanations (91.4%). Few patients refuse the examination after information. The situation where the assessment is performed on a patient without the faculty of understanding modalities and the necessity of that examination is in emergency (45%). In 63.4% of cases, the cardiologist requires the patients signature on the information document. CONCLUSION: Information dispensation prior to an examination is generally well done by cardiologists. The evidence of the information's dispensation is not at ease and most of cardiologists require written document from their patients, which is not legally necessary.

[Valvular locations in Whipple's disease: report of two cases and review of the literature]. / Atteinte valvulaire de la maladie de Whipple: deux observations et revue de la littérature.

Whipple's disease is a consequence of an infection due to a bacteria called Tropheryma whippelii. Endocarditis is frequently described in post mortem studies on this disease but the diagnosis in living patients is uncommon.

Meta-modelling: the appropriate solution for a family of applications.

The aim of this paper is to present an appropriate framework able to generate models and to implement them, in the objective of computerizing a family of medico-technical reports. The accelerated rate of technical development makes it necessary to design computerized applications independently of data-processing technology. This apparent paradox is a quite real challenge which needs research and development software environments to support frameworks. In this article, we present a meta-model (i.e. a generic structure - supported by the Méta-Gen software tool) ) which is able to generate various models of medico-technical reports. These models in turn are able to generate various types of instances. This meta-model is a "Meta-medical record", it is constituted of basic concepts : " User Semantic Group " to which are attached a set of " sentence-type ", a set of several corpus of variables with a set of graphs ("navigators"). Five models (echocardiography for hospital "A", echocardiography for hospital "B", gastroscopy, fibercoloscopy A.E.P.). were already generated from this "Meta-Medical Record". A beginning of implementation in echocardiography report is presented here. The advantages are a very thorough personalization of the document for the user, and a greater independence of the design diagram from the technological platform.

[Patient information. First evaluation]. / L'information au patient. Premier bilan.

The recent harmonisation of the jurisprudence between the Court of Appeal and State Council has affected medical responsibility because it is now the physician's obligation to prove that the information to the patient has been properly given: it is, therefore, a current issue. A first evaluation was undertaken to determine the modalities of patient information in cardiology by an enquiry of cardiologists working in the public and private sectors. The results show that information to patients was given concerning complementary investigations such as exercise stress testing, transoesophageal echocardiography, coronary angiography and cardiac pacing; the information was more often given for invasive procedures. In the great majority of cases (92%), it is the prescribing or operating physician who gives this information, usually the day before the procedure, with complementary oral explanations in about 90% of cases. Patient information, therefore, seems to be well done by cardiologists. However, the proof of information is not always easy, written consent, signed by the patient, not being compulsory at present.

[Reversible hypokinetic cardiomyopathy revealing severe hypocalcemia]. / Cardiomyopathie hypokinétique réversible révélatrice d'une hypocalcémie profonde.

Dilated cardiomyopathy may be primary or secondary. Although some causes are well known, such as toxic substances (alcohol, chemotherapy...) or viral infections, biochemical abnormalities are much less common. The authors report the case of a 58 year old woman with no previous history admitted to hospital for an inaugural episode of cardiac failure. The ECG showed sinus tachycardia with a long QT interval (560 mm) and a dilated hypokinetic cardiomyopathy with a left ventricular ejection fraction of 20%. The aetiological investigation showed severe hypocalcaemia (0.66 mmol/L) related to primary hypoparathyroidism. This is an important cause to remember because its treatment leads to correction of the cardiac disease, usually within weeks.

[Cardiogenic shock presenting a cardiac sarcoidosis. Apropos of a case]. / Choc cardiogénique révélant une sarcoïdose cardiaque. A propos d'un cas.

The authors report a case of cardiac sarcoidosis in a 38 year old patient presenting initially with cardiogenic shock. The diagnosis was made by myocardial biopsy. The patient underwent cardiac transplantation for terminal, refractory cardiac failure but postoperative complications led to the death of the patient a few weeks later. This rare observation should be noted because the causal disease may benefit from specific therapy.

Anomalous course of the left anterior descending coronary artery between the aorta and pulmonary trunk: a rare cause of myocardial ischaemia at rest.

When the left anterior descending coronary artery follows an anomalous course between the aorta and pulmonary artery it can cause myocardial ischaemia or sudden death during exercise in young people. Coronary arteriography in a 27 year old man with angina pectoris at rest showed a left anterior descending coronary artery arising from a common right trunk and running from the aorta to the pulmonary artery. Follow up after revascularisation was uneventful.

Oxidative stress in patients with unstable angina.

The aim of this study was to determine whether oxidative stress occurs in unstable angina. Thirty patients with unstable angina class B (Braunwald classification) were prospectively studied. Control groups consisted of 23 patients presenting with stable angina and of 21 age-matched healthy volunteers. Upon admission and every 8 h for 24 h, blood samples were drawn for the determination of plasma malondialdehyde (MDA) levels, Se-glutathione peroxidase (GPX) activity, erythrocyte reduced glutathione (GSH) concentrations, erythrocyte GPX and superoxide dismutase (SOD) activities. Coronary angiograms were performed within 4 days of admission in 26 out of the 30 patients included in the study. Nine of these 30 patients were subsequently identified as presenting a non-Q wave myocardial infarction and were separately examined. On admission, only plasma MDA levels and erythrocyte GSH concentrations differed among groups. Plasma MDA levels of patients presenting with unstable angina (P < 0.01) and acute myocardial infarction (P < 0.05) were higher than those of patients with stable angina and of normal volunteers, whereas there was no difference in these parameters between unstable angina and non-Q wave myocardial infarction groups. Erythrocyte GSH concentration was lower in all patient groups as compared to normal subjects. ANOVA for repeated measures showed no difference between admission and subsequent levels for all parameters. Finally, no difference was observed for any of the parameters when anti-ischaemic or anti-aggregant treatment before admission, or the number of affected vessels on coronary angiograms, were considered. We conclude that an oxidative stress can be evidenced in patients with unstable angina or acute myocardial infarction.