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.

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.

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.

[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.

[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.

[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).

[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.

[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.

[Late ventricular potentials and mitral valve prolapse]. / Potentiels ventriculaires tardifs et prolapsus valvulaire mitral.

In order to determine the predictive value for ventricular arrhythmias of ventricular late potentials (LP) in mitral valve prolapse (MVP) the authors performed high amplification signal-averaging ECG (SA) and 24 hours ambulatory ECG (Holter) monitoring in 68 consecutive patients (34 men, 34 women, average age 48 +/- 17.7 years) with echocardiographically diagnosed MVP. Patients with bundle branch block or associated cardiac disease were excluded. Echocardiography showed 26 patients to have floppy mitral valves (38.2%), 50 patients to have posterior deplacement > or = 5 mm of the mitral valves in systole (73.5%) and 35 patients to have mitral regurgitation (51.4%). Holter monitoring showed 17 patients without ventricular extrasystoles (VES), 15 had Lown Grade I, 6 had Lown Grade II, 3 had Lown Grade III, 15 had Lown Grade IV A and 12 had Lown Grade IV B ventricular arrhythmias. Therefore, 30 patients had complex ventricular arrhythmias (> or = Lown Grade III) and 13 patients had spontaneous non-sustained ventricular tachycardia (NSVT) (one patient had NSVT on resting ECG but not on Holter monitoring). Eighteen patients had LP (26.5%). The incidence of complex ventricular arrhythmias was higher in patients with mitral regurgitation (62.8% versus 27.7%; p < 0.005) whereas the incidence of NSVT was not significantly different (25.7% versus 17.1%; p = 0.15). On the other hand, the frequency of complex ventricular arrhythmias was not significantly different in the presence or absence of LP (61.1% versus 40%: NS) whereas the incidence of NSVT was higher in patients with LP (44.4% versus 10%; p < 0.005).(ABSTRACT TRUNCATED AT 250 WORDS)

[Antioxidative treatment in myocardial reperfusion ischemia]. / Le traitement antioxydant dans l'ischémie reperfusion myocardique.

Perfusion-induced myocardial ischaemia is observed in several situations: chronic coronary insufficiency, unstable angina, myocardial infarction, and during coronary angioplasty or bypass surgery. Oxygen-derived free radicals are liberated in large quantities during myocardial reperfusion ischaemia. Though very toxic in experimental studies, the responsibility of these free radicals in myocardial injury remains to be demonstrated clinically. Oxidant stress, characterised by an imbalance between the free radical attack and insufficient cellular defense seems partially responsible for reperfusion arrhythmias and post-ischemic stunning. On the other hand, its role is less evident in prolonged myocardial ischaemia causing irreversible myocardial lesions such as infarction. Antioxidant therapy is under evaluation in clinical trials. There are several options: some prevent the formation of free radicals by inhibiting the biochemical reactions which may produce them or by limiting the intervention of the neutrophils--the "fulcrum" of free radicals formations. Other antioxidant therapies inactivate free radicals as they are formed by promoting their degradation or their neutralisation. Experimental data is profuse and discordant. The models are very different. The first clinical trials are under way using either specific antioxidant molecules or molecules having other beneficial effects: in the latter case, the benefit of the antioxidant action is more difficult to demonstrate. Antioxidant therapy could play a role in surgical myocardial protection, especially of transplant organs, in very early forms of ischaemia. It could also prevent the pejorative hemodynamic consequences of myocardial stunning of the border zones of infarction, so enabling patients to survive a difficult period. The results of on-going studies should clarify the role of antioxidant therapy in reperfusion-induced myocardial ischaemia.

[Anti-arrhythmic therapy and cardiac failure]. / Médicaments antiarythmiques et insuffisance cardiaque.

In cardiac failure, continuous ambulatory electrocardiographic recording for 24 hours (Holter system) enables detection of 60 to 80% of complex ventricular arrhythmias, 15 to 40% of atrial arrhythmias and sudden death accounts for about 40% of fatalities but its causes are multiple and sometimes unrelated to arrhythmias. Abnormalities of cardiac structure, metabolic and neuro-hormonal changes and some drug therapies are implicated in the genesis of these arrhythmias, the management of which is discussed in two different situations with respect to the functional incapacity: in paucisymptomatic ventricular arrhythmias in patients with cardiac failure, class I antiarrhythmics and d-sotalol should be avoided and betablockers prescribed with caution; the indications of amiodarone have not yet been determined. When the arrhythmia is symptomatic (sustained ventricular tachycardia or ventricular fibrillation), class I antiarrhythmics are not effective enough in the prevention of sudden death; betablockers and amiodarone may give good results but should be compared with implantable defibrillators in the future. The multiplicity and complexity of the mechanisms of arrhythmias in cardiac failure, and the inadequate results obtained with classical antiarrhythmics necessitate the development of new antiarrhythmics based on blockade of non-selective channels probably activated in cardiac failure by the stretching of myocardial fibres.

[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.

[Prevalence of atrial arrhythmia in 48 hypertensive patients: research of predictive criteria]. / Etude de la prévalence des troubles du rythme auriculaire chez 48 patients hypertendus: recherche de critères prédictifs.

UNLABELLED: We studied atrial arrhythmias during a continue prospective work in 48 hypertensive patients referred to the OMS criteria. Hypertension was confirmed by a blood pressure ambulatory monitoring and stress testing blood pressure trend. All cardiovascular drugs were stopped at admission. Patients with associated valvular or coronary artery disease were excluded from analysis. In all patients, we realized a twelve lead-ECG, stress testing, 24 hour Holter monitoring, a blood pressure ambulatory monitoring, two-dimensional echocardiography with Doppler study and cardiac radio-nuclide angiography with diastolic function study. Atrial arrhythmias were considered significant if more than 100 premature atrial beats (PAB) and/or more than three successive PAB were present during Holter monitoring. Significant atrial arrhythmias were found in 39.5% of patients (group II, n = 19), not significant in 60.5% of patients (group I, n = 29). The duration of hypertension was longer in group II (140 vs 66 months, p < 0.05). There was no difference between the two populations considering left atrial size or blood pressure level. Furthermore, we were surprised to find a normal E/A ratio on mitral Doppler recording in patients with atrial arrhythmias (1.23 vs 0.9; p < 0.05). Others diastolic parameters didn't significantly differ. Left ventricular mass index was similar in the two groups but patients with atrial arrhythmias had more asymmetric hypertrophy (1.23 vs 1.13 septum/posterior wall ratio: p < 0.05). CONCLUSION: atrial arrhythmias in our study seem to be more dependent from duration of HTA and left ventricular asymmetric structure than from left atrial size.(ABSTRACT TRUNCATED AT 250 WORDS)

[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.

[Contact dermatitis following cardiac pacemaker implantation]. / Dermite de contact après implantation d'un stimulateur cardiaque.

The authors report a case illustrating the causality between the appearance of contact eczema and the implantation of a cardiac pacemaker. To the authors' knowledge, only 11 cases of eczema secondary to the implantation of a pacemaker have been previously reported, all in dermatological journals. In 60% of caes, the lesions were observed over the pacemaker implantation and the appearance of the rash varied from 2 days to 24 months. The causal allergens were mainly the metallic and plastic components. The physiopathological mechanisms, though not completely understood, are related to cellular immunity and therefore, to delayed hypersensitivity reactions. From the therapeutic point of view, locak steroid applications to limited excema lesions have been suggested but recurrence is common. The only effective treatment is removal of the allergen.

[Value of tomoscintigraphy with Fourier analysis in the diagnosis of arrhythmogenic right ventricular cardiomyopathy]. / Intérêt de la tomoscintigraphie cavitaire avec analyse de Fourier dans le diagnostic de cardiomyopathie arythmogène du ventricule droit.

ECG gated blood pool tomography has been performed in sixteen patients with right ventricular arrhythmias in whom the diagnosis of arrhythmogenic right ventricular cardiomyopathy was made based on the finding of abnormalities on contrast angiography. They were compared both to control subjects and to patients with primary dilated cardiomyopathy. Thick slices of ventricles were obtained throughout the cardiac cycle in three orthogonal planes: horizontal long axis and short axis thick slices for analysis of right and left ventricular regional wall motion abnormalities and analysis of the spread of the contraction by means of Fourier phase imaging, vertical long axis slices (one for each ventricle) for ejection fractions, because of easy and reproducible determination of valvular planes and analysis of all right ventricular segments, especially the pulmonary infundibulum. Five typical right ventricular abnormalities were seen: decreased ejection fraction (32 +/- 15% vs 55 +/- 3% in control; p < 0.001), increased diameter (ratio of right to left diameters = 1.2 +/- 0.3 vs 0.9 +/- 0.1; p < 0.01), global delayed contraction versus that of the left ventricle (22 +/- 20 degrees vs -2 +/- 6%; p < 0.01), increased dispersion of contraction (32 +/- 16 degrees vs 13 +/- 4 degrees; p < 0.01) and presence of segments with decreased and/or delayed contraction. Right ventricular disease was observed in all the patients: localized form (56%), diffused form (44%). This method provides accurate functional data for diagnosis and follow-up of patients. In future, this wall motion evaluation method may replace planar nuclear angiography as myocardial SPECT have replaced myocardial planar scintigraphy.

[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.

[A study of 36 cases of mitral valve prolapse by isotopic ventricular tomography]. / Etude par tomographie cavitaire isotopique de 36 cas de prolapsus valvulaire mitral.

Left and right ventricular wall motion was studied in mitral valve prolapse with or without ventricular arrhythmias. Regional and global ventricular wall motion was evaluated by isotopic methods, based in ejection fraction and Fourier phase analysis representing the progression of wall contraction. The synchronisation of the ventricles was characterized by the difference of the mean phase of each ventricle. The heterogeneity of contraction of each ventricle was defined by the dispersion around the mean (standard deviations of the phases). Fifteen of the 36 patients had complex ventricular arrhythmias (Lown grade > or = III). 12 had LVP and 16 had mitral regurgitation. In mitral valve prolapse, the RV EF was decreased compared with normal controls (30 +/- 9% vs 40 +/- 10% ; p < 0.001), especially in patients with mitral regurgitation (26 +/- 7% vs 30 +/- 10%; p = NS) and complex ventricular arrhythmias (26 +/- 7% vs 32 +/- 10%; p < 0.01). The SDP of the LV was greater than those of controls (18 +/- 11 degrees vs 11 +/- 5 degrees ; p = NS) whereas the SDP of the RV was greater (27 +/- 17 degrees vs 12 +/- 5 degrees ; p < 0.05) especially in those with complex ventricular arrhythmias (36 +/- 21 degrees vs 21 +/- 10 degrees : p < 0.01). The SDP of LV and RV were greater in patients with mitral regurgitation: 20 +/- 11 degrees versus 17 +/- 10 degrees (NS) and 35 +/- 21 degrees versus 20 +/- 8 degrees (p < 0.01). Heterogenous ventricular contraction, more marked in the right ventricle in mitral valve prolapse suggests severe myocardial disruption in this valvular disease, reflected by the high incidence of LVP and complex ventricular arrhythmias.

[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.