High response rate and improved exercise capacity and quality of life with a new regimen of darbepoetin alfa with or without filgrastim in lower-risk myelodysplastic syndromes: a phase II study by the GFM.

Darbepoetin (DAR), with or without granulocyte colony-stimulating factor (G-CSF), has proved effective in treating anemia in patients with lower-risk myelodysplastic syndrome (MDS), but its effects on quality of life (QoL) and exercise functioning are less well established. In this phase II study (no. NCT00443339), lower-risk MDS patients with anemia and endogenous erythropoietin (EPO) level <500 IU/L received DAR 500 µg once every 2 weeks for 12 weeks, with G-CSF added at week 12 in non-responders. Physical performance was assessed with the 6-min walking test and, for fit patients, maximal oxygen consumption (VO2max). QoL was evaluated using SF-36 and FACT-An tests. In 99 patients, erythroid response rate according to IWG 2006 criteria was 48 and 56 % at 12 and 24 weeks, respectively. Addition of G-CSF rescued 22 % of non-responders. In 48 % of the responders, interval between darbepoetin injections could be increased for maintenance treatment. Serum EPO level was the only independent predictive factor of response at 12 weeks, and its most discriminant cutoff value was 100 IU/L. QoL and VO2max showed improvement over time in responders, compared with non-responders. With a median follow-up of 52 months, median response duration was not reached, and 3-year cumulative incidence of acute myeloid leukemia and overall survival (OS) was 14.5 and 70 %, respectively. Baseline transfusion dependence, International Prognostic Score System (IPSS), and Revised IPSS accurately predicted OS from treatment onset. Tolerance of darbepoetin was good. In conclusion, this regimen of darbepoetin every 2 weeks yielded high response rates and prolonged response duration. Objective improvement in exercise testing and in patient-reported QoL confirms the clinical relevance of anemia correction with erythropoiesis-stimulating agents.

Interpretation of lipoprotein-associated phospholipase A2 levels is influenced by cardiac disease, comorbidities, extension of atherosclerosis and treatments.

PURPOSE: Lipoprotein-associated phospholipase A2 (Lp-PLA2) is an inflammatory biomarker secreted in the atherosclerotic plaque. Blood levels of Lp-PLA2 predict future cardiovascular events in patients with ischemic disease and heart failure. This association seems to be independent of traditional cardiovascular risk factors. The aims of our study were (1) to assess relationships between Lp-PLA2 levels, cardiac disease and treatments; (2) to evaluate the association of Lp-PLA2 level with the severity of angiographic coronary artery disease (CAD) and the extracoronary atherosclerosis. METHODS: Between December 2009 and June 2010, 494 subjects were recruited from a population scheduled for diagnostic coronary angiography. Routine clinical (age, gender, BMI and treatment), cardiac (echocardiography, coronarography, carotid ultrasonography) and biochemical parameters were recorded for all patients. Lp-PLA2 mass concentration was assessed in serum with a Plac®-test turbidimetric immunoassay. Control Lp-PLA2 values were specifically obtained in 61 healthy subjects aged 44.5 ± 17.6 years (range: 25 to 59 years) without known cardiovascular risk factors (diabetes, smoking, hypertension, dyslipidemia) or cardiac treatment. RESULTS: In healthy controls, mean Lp-PLA2 level was 163 ± 43 µg/L (166 ± 45 µg/L in men and 159 ± 39 µg/L in women, non significant difference). In our cohort of 494 patients (69.8% men) aged 64.2 ± 16.7 years, the main etiologies of cardiomyopathies were ischemic (40%), valvular (22%), cardiac failure with left ventricular (LV) dysfunction (14%), infection (5%) and aortic aneurysm (7%). Mean Lp-PLA2 levels were 216 ± 17 µg/L. Lp-PLA2 correlated with age, BMI, current smoking, history of hypertension but not with diabetes and gender. The bivariate analysis showed a significant correlation between Lp-PLA2, and BMI (p=0.001) but no correlation with serum creatinine or NYHA status. A multivariate correlation showed that Lp-PLA2 was associated with total cholesterol, LDL-cholesterol and apoB (r=0.95, p<0.0001) but not with Lp(a). We observed that Lp-PLA2 was significantly associated with treatments such as statins and ACEi/ARA2 but not with ß-blockers, antiaggregant drugs or diuretics. Lp-PLA2 levels were significantly higher in patients with CAD than in patients without CAD (223 ± 54 vs. 208 ± 52 µg/L, respectively; p<0.007). Moreover, Lp-PLA2 levels were significantly higher in patients with the most extensive angiographic CAD [single (n=24)=215.2 ± 52 µg/L; two (n=55)=222 ± 53 µg/L and three vessels (n=140)=251.9 ± 53.7 µg/L, respectively; p<0.0001]. Patients with heart failure, sepsis or aortic aneurysm had increased Lp-PLA2 levels: 256.2 ± 46.8; 226.7 ± 47.3; 218.1 ± 38.9 µg/L, respectively, as compared to controls (p<0.0001). In patients with carotid artery disease, Lp-PLA2 significantly increased with the severity of atherosclerosis. Mean Lp-PLA2 levels were 218.8 ± 51 µg/L in the group without any stenosis (n=108), 224 ± 51 µg/L in the group with mild stenosis (n=101), and 231 ± 46 µg/L in the group with severe stenosis (n=22); p=0.004. CONCLUSION: This study clearly shows that interpretation of Lp-PLA2 levels needs a good assessment of cardiac parameters and treatments, especially statins and ACEi/ARA2. Lp-PLA2 levels are significantly associated with coronary heart disease and with the extension of extra coronary disease after adjustment for age and gender.

Manganese superoxide dismutase dimorphism relationship with severity and prognosis in cardiogenic shock due to dilated cardiomyopathy.

The aim was to determine (a) Ala-16Val-SOD2 dimorphisms; (b) allelic frequency and phenotype of a common Pro-Leu polymorphism in GPx1, in a cohort of patients with a cardiogenic shock (CS) due to dilated cardiomyopathy without acute coronary syndrome. Consecutive patients with de novo CS that worsened a dilated (DCM) or ischemic (ICM) cardiomyopathy. Congenital heart disease, pacemaker and other shock aetiologies were excluded. To determine oxidative stress (OS), this study evaluated lipid peroxidation, protein oxidation and erythrocyte GPx, SOD and catalase activities. Ala16Val-SOD2 (dbSNP: rs4880) and Pro198Leu-GPx1 (dbSNP: rs1050450) polymorphisms were studied by allelic discrimination using fluorogenic probes and the 5'nuclease (TaqMan) assay. Twenty-four patients (with ICM (n = 8) or DCM (n = 16), age = 57.5 ± 10.7 years, LVEF = 25.3 ± 8.5%, NT-proBNP levels = 8540 ± 1703 ng/L) were included during a 15 month follow-up. OS parameters were significantly higher in patients than in controls. Distribution of MnSOD genotypes was 47% Val/Val-variant, 29.5% Ala/Val and 23.5% Ala/Ala-variants. Severity of CS was more important in patients with Val/Val-variant and can be put in parallel with NT-proBNP levels (Val/Val-variant: 11 310 ± 3875 ng/L vs Ala/Ala-variant: 6486 ± 1375 ng/L and Ala/Val-variant: 6004 ± 2228 ng/L; p < 0.05) and hemodynamic support duration (144.6 vs Ala/Val-variant: 108.8 h and Ala/Ala-variant: 52.5 h; p < 0.05) with a positive correlation (Spearman rho = 0.72, p < 0.05). Moreover, Val/Val-variant significantly influenced the mortality (Spearman rho = 0.67, p < 0.05), but not the morbidity (p = 0.3). Distribution of GPx genotypes was 64% Pro/Pro, 18% Pro/Leu and 18% Leu/Leu. GPx-variants influenced neither GPx activities nor cardiac events. In conclusion, CS was associated with markers of increased OS. GPx polymorphism did not influence the GPx activity. Only the Val-encoding MnSOD allele was significantly correlated with the severity and prognosis of CS.

B-type natriuretic peptide, a marker of asymptomatic left ventricular dysfunction in type 2 diabetic patients.

AIM: To evaluate BNP in assessing LV functions in asymptomatic type 2 diabetic patients. METHODS: BNP was measured in 91 consecutive patients with type 2 diabetes mellitus. According to Doppler echocardiography, patients were first separated into three categories: normal LV function, or isolated diastolic or systolic LV dysfunction. As some patients with diastolic dysfunction were treated for hypertension, the population was divided into four groups: groups 1, 2 and 3 all had no antihypertensive treatment, and had normal LV function, and isolated diastolic and systolic LV dysfunction, respectively; and group 4 were being treated with antihypertensive drugs and had diastolic LV dysfunction. RESULTS: In group 1, BNP levels (13+/-2 ng/L) were lower than in group 2 (87+/-20 ng/L, P<0.0001) or group 3 (213+/-32 ng/L, P<0.0001), but were similar to those of group 4 (32+/-6 ng/L, P=0.14). ROC analysis revealed a rule-out value of 23 ng/L for group 1 versus group 2, and of 239 ng/L for group 2 versus group 3. In groups 1, 2 and 3 taken together, BNP levels were correlated with urinary albumin excretion rate (r=0.80, P<0.0001) and pulse pressure (r=0.65, P<0.0001). In group 4, patients receiving ACE inhibitors had lower BNP levels than those receiving ss-blockers. CONCLUSION: BNP can be used to pre-screen asymptomatic type 2 diabetic patients with LV dysfunction, and may reveal vascular remodelling in type 2 diabetes mellitus.

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.

[Evaluation after 20 years of a case of Takayasu's disease that presented with aortic regurgitation]. / Evolution après 20 ans d'une maladie de Takayasu Révélée par une insuffisance aortique.

Takayasu's disease is a segmental multifocal affection of medium and large arteries. The diagnosis is based on the association of stenotic and aneurismal lesions of the aorta and its branches secondary to an inflammatory infiltration of the media and adventitia. Cases of aortic regurgitation associated with aneurismal dilatation of the ascending aorta as the presenting features of Takayasu's disease, as in this case, are rare. Histological examination of the aortic wall may help establish the diagnosis by showing signs of aortitis. The other usual arterial lesions are sometimes missing at the initial phase of the disease. A late histological diagnosis may be difficult as the inflammatory lesions tend to be progressively replaced by fibrotic lesions or a banal atheroma.

Expanding the phenotype of LMNA mutations in dilated cardiomyopathy and functional consequences of these mutations.

AIMS: Mutations in the lamin A/C gene (LMNA) have been reported to be involved in dilated cardiomyopathy (DCM) associated with conduction system disease and/or skeletal myopathy. The aim of this study was to perform a mutational analysis of LMNA in a large white population of patients affected by dilated cardiomyopathy with or without associated symptoms. METHODS: We performed screening of the coding sequence of LMNA on DNA samples from 66 index cases, and carried out cell transfection experiments to examine the functional consequences of the mutations identified. RESULTS: A new missense (E161K) mutation was identified in a family with early atrial fibrillation and a previously described (R377H) mutation in another family with a quadriceps myopathy associated with DCM. A new mutation (28insA) leading to a premature stop codon was identified in a family affected by DCM with conduction defects. No mutation in LMNA was found in cases with isolated dilated cardiomyopathy. Functional analyses have identified potential physiopathological mechanisms involving identified mutations, such as haploinsufficiency (28insA) or intermediate filament disorganisation (E161K, R377H). CONCLUSION: For the first time, a specific phenotype characterised by early atrial fibrillation is associated with LMNA mutation. Conversely, mutations in LMNA appear as a rare cause of isolated dilated cardiomyopathy. The variable phenotypes observed in LMNA-DCM might be explained by the variability of functional consequences of LMNA mutations.

[Conduction defects as the presenting feature of sarcoidosis or observed during the course of the disease: regression with corticoid steroid therapy]. / Troubles conductifs révélant une sarcoïdose ou apparaissant au cours de son évolution: régression sous corticoïdes.

Cardiac sarcoidosis is often unrecognised because of the absence of specific clinical and electrical signs. The consequences are serious, the main risk being sudden death due to conduction defects (24 to 31% of cases) or ventricular arrhythmias. Any conduction defect without an obvious cause in a young patient should suggest a possible diagnosis of sarcoidosis. The confirmation is histological when giant cell non-caseuting epithelioid granuloma is demonstrated but myocardial biopsies are only positive in 20% of cases. Therefore, biopsy of accessible organs such as salivary glands is recommended. Diagnostic strategy consists in searching for signs of systemic sarcoidosis, and, when the diagnosis has been established, perform a complete work-up with echocardiography, dipyridamole myocardial scintigraphy, cardiac MRI and 24 hour ambulatory ECG recordings (Holter). The only proven treatment is steroid therapy with occasional spectacular observations of reversibility of arrhythmias or conduction defects.

[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.

Breast adenocarcinoma complicating Kallmann's syndrome.

Carcinoma of the breast is relatively rare among men, accounting for less than 1% of all malignancies. An increased risk has been associated with benign breast diseases, such as gynecomastia, Klinefelter's syndrome, testicular disorders, exogenous estrogen use, radiation, or a family history of male or female breast cancer. To date, hypogonadotrophic hypogonadism has never been associated with male breast cancer. We report here the first case of breast cancer coexisting with Kallmann's syndrome in a 66-year-old man.

[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.

[Cardiac abnormalities in a prospective series of 40 patients with type 2 diabetes]. / Anomalies cardiaques d'une série prospective de 40 patients diabétiques de type 2.

Cardiovascular mortality, the principal cause of early death in diabetics, is multifactorial. A prospective study was undertaken to analyse the different factors of excess cardiac complications in 40 patients with type 2 diabetes, whatever the symptomatology, by making an inventory of the cardiac abnormalities (systolic and diastolic left ventricular function, left ventricular hypertrophy, abnormalities of myocardial perfusion, heart rate variability and arrhythmias). Patients underwent 24 hour Holter monitoring, high amplification signal averaged electrocardiography, echocardiography, Thallium scintigraphy with a dipyridamole test followed by coronary angiography when positive. Patients were aged 60 +/- 8 years, diabetics for 11.8 +/- 6.8 years, and had associated cardiovascular risk factors: 85% were obese, 75% were hypertensive, 62.5% had hypercholesterolaemia and 60% were smokers. The HbA1C was 9.2 +/- 19%. An increased left ventricular mass was observed in 34.2% of patients. The left ventricular ejection fraction was normal (59.1 +/- 6.8%); 69.7% of patients had left ventricular diastolic dysfunction. Reduced heart rate variability was observed in 51.8% of cases. Late ventricular potentials were recorded on high amplification signal averaging in 39.5% of patients; 25.6% had significant ventricular extrasystoles and 52.2% had atrial extrasystoles. Twelve patients (45%) underwent Thallium myocardial scintigraphy with a positive dipyridamole test, 8 of whom had coronary lesions on angiography. The excess cardiac complications of diabetes is mainly due to ischaemic heart disease aggravated by autonomic neuropathy, left ventricular diastolic dysfunction, arrhythmias and left ventricular hypertrophy. In future, larger series are required to demonstrate that this detection can guide therapeutic intervention and reduce cardiac morbidity and mortality of diabetics.

[Cardiac side effects of anti-HIV agents]. / Complications cardiaques des médicaments au cours de l'infection par le VIH.

Both nature and prognosis of cardiac complications occurring in patients infected by the Human Immunodeficiency Virus-1 (HIV-1) have changed considerably since the introduction of highly acive and anti-retroviral triple therapy ("HART"). Opportunist cardiac infections have thus been displaced and side effects of drugs now occupy the primary aetiological role. Torsades de pointe may be exceptionally triggered by anti-infectious agents such as pentacarinat or trimethoprime-sulfamethoxazole, as are those induced by the association of ketoconazole and terfenadine or cisapride, the dangers of which are well known and the prevention more effective, especially with the association with HIV antiproteases which inhibit the cytochrome P450. The diagnosis of iatrogenic myocardial dysfunction is more difficult, except when it occurs acutely as with phosphonoformate (Foscarnet), or interleukine-2. Progressive cardiomyopathy caused by -interferon and dideoxynucleosides (zidovudine, didanosine and zalcitabine), reversible on withdrawal of the drug responsible in half the cases, should be distinguished from those due to the HIV itself (therapeutic relay) or to another associated cause (alcohol, coronary artery disease). The coronary complications of diseases treated by antiproteases usually occur in smokers whose cholesterol and triglyceride levels are rapidly increased with HAART. In a series of 9 patients (amongst 700 treated with the antiproteases), after the acute phase of myocardial infarction during which the interventional approach is often preferred, the medium-term prognosis is relatively good, on condition that the patients correct the hyperlipidaemia and give up smoking.

[Mid-term complications of automatic implantable cardiac defibrillators]. / Complications à moyen terme du défibrillateur cardiaque automatique implantable.

The incidence and the nature of medium-term complications of automatic implantable cardiac defibrillators (AICD) were studied. Seventy-nine AICD were implanted in 50 consecutive patients (42 men, aged 54.5 +/- 13.7 years). Forty-six patients had spontaneous ventricular arrhythmia. These arrhythmias were resistant to treatment (N = 9), reproducible with treatment (N = 28). In 4 patients, the indication was prophylactic, in 2 a Brugada syndrome, in 2 syncope with reinducible ventricular tachycardia and in 1 patient, torsades with a short coupling interval. Forty-six patients had underlying cardiac disease (ischaemic, N = 28, primary dilated cardiomyopathy, N = 10, others, N = 8). The ejection fraction was > 40% in 32 patients. The average follow-up was 41.3 +/- 34.9 months. Eight patients died, 2 from cardiac failure. Twenty-one patients (42%) had 1 or more complications related to their AICD. These occurred: in the operative period (N = 3): 1 post-shock atrioventricular block, 1 ruptured electrode and 1 increased threshold with amiodarone; in the postoperative period (N = 6): infection in 3 cases, cerebrovascular accident in 1 case, deep venous thrombosis of the left arm in 1 case, pneumothorax in 1 case. In the medium-term, the complications were mainly inappropriate electrical shocks observed in 14 patients related to atrial arrhythmias in 7 cases, sinus tachycardia in 1 case, over-detection of myopotentials in 2 cases and electrode dysfunction in 4 cases. In addition, the authors observed complications related to the material: AICD failure in 1 case, electrode displacement in 1 case, and electrode rupture in 3 cases. The authors conclude that AICD are effective for the treatment of malignant ventricular arrhythmias which justify strict specialist follow-up given the incidence and diversity of their complications.

[Mitral valve prolapse, arrhythmias and sudden death]. / Prolapsus valvulaire mitral, troubles du rythme et mort subite.

Some of the classical concepts of mitral valve prolapse (MVP) should be reviewed in the light of recent publications. It is a condition, according to strict echocardiographic criteria excluding near physiological abnormalities, which affects 2 to 3% of the adult population in the industrialised world. Only repetitive atrial arrhythmias and complex ventricular arrhythmias are more common in this condition than in control groups, the differences being more pronounced in cases of mitral regurgitation. The risk of syncope or sudden death is 0.1% per year, hardly any different to that of the rest of the general adult population (0.2%). However, this risk may attain 0.9 to 2% in cases with mitral regurgitation. The causes of sudden death are unclear (haemodynamic, neurohumoral, arrhythmic, etc...), although there is evidence in favour of malignant ventricular arrhythmias. Detailed clinical, electrophysiological, isotopic and anatomopathological studies have raised doubts as to the direct responsibility of the vascular malformation (or its eventual consequences on the atrial and ventricular chambers) in this mode of fatal outcome. On the other hand, localised or diffuse myocardial disease is often observed, usually a- or pauci-symptomatic, associated with MVP, the responsibility of which is more plausible. Therefore, the physician should adopt a flexible attitude towards these patients, reassuring those with benign symptoms at low risk and following up or actively treating the rarer malignant forms (especially familial, syncopal with mitral regurgitation and/or severe arrhythmias).