Primary electrical diseases diagnosis, genetic and management.

Primary electrical diseases or channelopathies are inherited genetic alterations of the cell ionic and electrical behavior leading to various cardiac arrhythmias carrying the risk of sudden death. A descriptive review of the successively described channelopathies is made in this article, with emphasis on the clinical manifestations, the genetic background and the currently accepted therapeutic options.

Stepwise sarcomere shortening: analysis by high-speed cinemicrography.

Sarcomere shortening in striated muscle appears to follow a regionally synchronized staircase-like time course not anticipated in some cross-bridge models. The visualization method used has been criticized as subject to Bragg diffraction effects. Two independent optical methods were used to visualize a muscle during contraction; agreement between the stepwise behavior observed with the two methods suggests that the phenomenon is genuine.

Organization in response to massive afflux of war victims in civilian practice - experimental feedback from the November 2015 Paris terrorist attacks.

The arrival of a large number of war-weapon casualties at a civilian trauma center requires anticipation. A plan defining the management principles and the respective roles of the involved physicians and nurses and their interaction with each other is essential. Uni-directional patient flow associated with adequate numbers of staff physicians and nurses under the leadership of a medical director is essential to prevent the overwhelming of the trauma center. Routine and regular interaction between the pre-hospital medical flow control system and the medical director, on one hand, and between surgical teams and the medical director, on the other, are necessary to know when to apply "damage control" surgical techniques. Based on the feedback of a level 1 trauma center that received 53 victims of the November 13, 2015 terrorist attack in Paris, we present the factors of success, and the stumbling blocks.

[Clinical evolution of patients following investigation of atrial vulnerability after a first cerebral ischaemic accident]. / Evolution clinique des patients ayant eu une recherche de vulnérabilité atriale après un premier accident ischémique cérébral.

Atrial vulnerability reflects the ability of the atrium to fibrillate. ISAV (Ischemic stroke and atrial vulnerability) is a French epidemiological registry whose main goal is to assess the evolution modalities of patients in whom an electrophysiological study of the atrium has been performed. A group of 269 patients with a history of non elucidated ischemic stroke and an electrophysiological study of the atrium performed in a mean delay of 3 months after the stroke has been included. Their mean age at the time of the stroke was 55 +/- 15.8 years. The electrophysiological study has measured the effective refractory period of the atrium, the locoregional right intra-atrial conduction time, the index of latent atrial vulnerability and assessed the inductibility. The mean delay between the date of the stroke and the date of the last news was 4.4 +/- 2.8 years. We observed 12 deaths and 11 patients presented during the follow up a spontaneous atrial arrhythmia and 17 a recurrence of stroke. If we consider the occurrence of the 28 combined events (atrial arrhythmia and/or stroke), it is not correlated with the presence of an atrial septal defect nor with the existence of an atrial vulnerability. On the contrary this occurrence is correlated with tobacco consumption and/or arterial hypertension; 82% of patients have these risk factors versus 54% of patients without events (p = 0.004). This association is not significant in patients younger than 55 years.

[Cardiac asystole during acute anterior myocardial infarction: a consequence of endocardiac reflexes]. / Asystolie en phase aiguë d'infarctus du myocarde antéroseptal: une manifestation de la mise en jeu de réflexes endocardiaques.

The authors report a case of paroxysmal, complete atrioventricular block during an anterior acute myocardial infarction, leading to asystolia. The different possible physiopathological mechanisms are discussed, suggesting a paroxysmal nodal conduction defect, secondary to transient parasympathetic stimulation, triggered by a Bezold-Jarish type of cardiac reflex. This reflex is frequently involved in various pathologic situations or diagnostic procedures, usual in cardiology. Although it is frequently observed in inferior myocardial infarction, it can occur during an anterior acute myocardial infarction.

[Ablation of ventricular extra-systole in the left Valsalva sinus. A case report]. / Ablation d'une extrasystolie ventriculaire dans le sinus de Valsalva gauche. A propos d'un cas.

We report the case of a patient presenting with unrelenting isolated or repetitive monomorphic ventricular extra-systoles, with left block and right axis deviation, which appeared to arise from the right ventricular chamber, but for which ablation was finally performed in the left Valsalva sinus. The ECG and endocavity electro-physiological features which led us to suspect this atypical, although not exceptional, situation are reported, as well as the techniques for ablation available in this case.

[Prescriptions following acute coronary syndrome]. / Ordonnance de sortie après syndrome coronaire aigu.

Each year in France, 150,000 to 180,000 new patients are the subject of prescriptions following acute coronary syndrome with or without ST segment elevation. There are two targets of the treatment, atherosclerosis, a diffuse, evolving trouble which, in this situation, is coming out of an unstable phase, and the myocardium, which has often been revascularised and has suffered deterioration of its contractile and electrophysiological characteristics to a greater or lesser extent. Prescriptions, based on proven factors and always centred on hygiene and dietary advice and the use of a combination of statins and aspirin, are adapted to suit the atherosclerotic and myocardial risk assessed for the individual patient. The prescription starts off the secondary preventive phase. It marks the first stage of the follow up, which is inevitable though of variable duration, for a disease which may evolve. It is the first step in the accompaniment of an attentive, informed patient whose confidence has been restored and who must now avoid falling into the double trap of not taking the treatment sufficiently seriously or of obsessively over-reacting.

The use of metallochromic Ca indicators in skeletal muscle.

Absorbance signals recorded with metallochromic indicators in skeletal muscle fibers show rapid time courses that probably closely track the fast kinetic process of Ca++ release and retrapping by the sarcoplasmic reticulum. However, the formation of more than one complex in cuvette calibrations, both for Arsenazo III (ArIII) and Antipyrylazo III (ApIII), suggest that care needs to be taken in the deconvolution of in vivo absorbance signals. Since the kinetic rate constants have not yet been obtained for these probes, attempts to deconvolute absorbance signals should be considered approximate. The evidence suggesting that more than one complex is formed during a skeletal muscle transient with ArIII is more compelling than for the case of ApIII. The differences between the ArIII and ApIII signals may not be readily explained assuming 1:1 dye:Ca complexation and kinetic differences between the probes. Competition for Ca++ with cell Ca buffers and/or multiple complex formation by at least one of these probes needs to be invoked. Based on a simple model to simulate the behavior of the Ca signals in muscle, it may be suggested that an ApIII-like probe would more closely track pCa changes in the fiber than would an ArIII-like probe, which would show more interference with intracellular buffers; an even higher affinity probe would tend to sense the total release of Ca by the SR.

[Cardiotoxicity of continuous intravenous infusion of 5-fluorouracil: clinical study, prevention and physiopathology. Apropos of 13 cases]. / Cardiotoxicité du 5-fluorouracile perfusion intraveineuse continue: étude clinique, prévention, physiopathologie. A propos d'une série de 13 cas.

5-Fluorouracil (5FU) cardiotoxicity is thought to be an infrequent toxic effect, usually related to coronary vasospasm. Among 198 patients (pts) receiving 5FU as a continuous infusion (CI) over 96 or 120 h, at a daily dose of 1,000 mg/m2, 13 new cases of 5FU--cardiotoxicity are reported. In all cases but 1, cardiovascular symptoms occurred at the first 5FU-CI course, with mean time of onset of 3 d. Chest pain was the prominent inaugural symptom with angor pectoris (6 pts) and pericarditis (3 pts). Five pts developed cardiogenic shock, which was irreversible in 3 cases. The severity of such an evolution requires prompt 5FU discontinuation, if symptoms occur, and careful hemodynamic supervision during 5FU therapy. One patient experienced typical myocardial infarction, another one epicardo myocardiopathic process with adiastolia. Disorders of repolarisation on electrocardiographic tracing were the prominent abnormalities, associated with a significant increase of QT segment in 3 cases. Re-introduction of 5FU-CI resulted in chest pain recurrence in 2 out of 4 pts, despite calcium antagonist "prevention". In our retrospective study, the incidence of 5FU-CI cardiotoxicity is 6.5%, which is consistent with recent reports (10%). Whether 5FU-induced cardiotoxicity mechanism is related to vasospastic or direct effect remains unclear. However, our series suggests a 5FU-induced post ischaemic myocardial dysfunction as described in the "stunned myocardium" syndrome.

[Diagnosis of adrenergic ventricular tachycardia in two homozygotic twins]. / Dépistage d'une tachycardie ventriculaire catécholergique chez 2 jumeaux monozygotes.

The authors report the first case of adrenergic ventricular tachycardia observed in two 11 year old homozygotic twins. Syncope on exercise led to the investigation of the first twin with confirmation of the diagnosis by exercise stress testing. The same investigations led to the demonstration of the condition in the second asymptomatic twin. The two patients were treated with nadolol and there has been no recurrence of syncope with a 2 year follow-up. Adrenergic ventricular tachycardia is a rare arrhythmia with a risk of sudden death which may be prevented by betablocker therapy. An exhaustive familial inquiry is essential, but is only positive in one out of three cases. Mutations in the ryanodine receptor gene (hRyRZ) and also in the calsequestrin gene (CASQ2) have recently been reported. Progress in the understanding of the genetics of this condition should improve primary preventive measures.

[Clinical, angiographic aspects and 18-month follow-up of 134 cases of left coronary trunk stenosis]. / Caractères cliniques, angiographiques et suivi à 18 mois de 134 cas de sténoses du tronc commun de la coronaire gauche.

A series of 134 patients with left main coronary stenosis was followed up for an average of 18 months. The majority of patients presented with unstable angina and per-critical ECG changes. Coronary angiography showed that left main coronary stenosis is rarely an isolated entity but generally associated with diffuse coronary atherosclerosis. Left ventricular function is usually normal. Treatment is surgical whenever the coronary lesions, left ventricular function and general condition of the patient allow it. The medium-term surgical results are excellent on clinical symptoms and quality of life at the price of low perioperative mortality and morbidity.

[Systemic fibrinolytic treatment in the acute phase of myocardial infarction. Effects on coronary repermeation and left ventricular function]. / Traitement fibrinolytique par voie générale à la phase aiguë de l'infarctus du myocarde. Effets sur la reperméabilisation coronaire et sur la fonction ventriculaire gauche.

Coronary angiography and 2D echocardiography were performed at the 24th hour and 21st day of acute myocardial infarction in 18 patients to study the effects of peripheral fibrinolytic therapy on coronary repermeabilisation and left ventricular function. The treatment was the same for all patients included in the study. The left ventricular ejection fraction and sequential left ventricular contractility were analysed. The first coronary angiographic control study (24th hour) showed coronary repermeabilisation in 18 cases, occurring in significantly stenosed vessels in 15 cases and in angiographically normal vessels in 3 cases, demonstrating appearances of thrombosis. Transluminal coronary angioplasty was performed in 8 cases. Coronary angiography at the 21st day showed 4 secondary occlusions in patients who had not undergone angioplasty. The comparative study of left ventricular function did not show a statistically significant difference between the 24th hour and the 21st day studies. Biochemical studies showed on early massive elevation of the specific myocardial enzyme CPK MB and a rapid fall in the fibrinolytic activity of all patients. The treatment was well tolerated and mortality was nil.

[Myocardial infarct of effort with normal coronaries. Study of in vivo platelet activation]. / Infarctus du myocarde d'effort à coronaires saines. Etude de l'activation plaquettaire in vivo.

Platelet activation may play a part in causing myocardium infarction with angiographically normal coronary arteries. We investigated this possibility by performing ergometric stress testing in a series of 9 patients (Group A) who had suffered myocardial infarction after a violent effort with angiographically documented coronary insufficiency responsible for a stable effort angina (Group B) and 11 healthy subjects (Group C). Blood samples were taken separately before exercise, at the peak of exercise, and during the recovery period. Platelet morphology, a sensitive indication of the degree of platelet activation, was studied by phase contrast microscopy after immediate fixation of the blood. The percentage of non-discoidal platelets presenting with one or several spicules was measured. At the same time, the plasma concentrations of platelet factor 4 (PF4) and beta-thromboglobulin (beta-TG) were measured. At rest, there was no difference in platelet morphology or specific platelet proteins between the 3 groups. At the peak effort, there was a significant increase of the number of morphologically modified platelets in Groups A and B but not in healthy subjects. This platelet activation could not be linked to the presence of myocardial ischaemia because it was found both in patients with a negative maximal exercise stress test (Group A). Finally, no increase of the plasma concentrations of the platelet protein was observed in any of the groups.

[Cardiocirculatory arrest after administration of combined mifepristone (Mifegyne) and sulprostone (Nalador) for induced abortion. Possible role of coronary vasospasm]. / Arrêt cardiocirculatoire après administration de l'association mifépristone (Mifégyne) sulprostone (Nalador) pour interruption de grossesse. Rôle évoqué du spasme coronaire.

Reversible ventricular fibrillation occurred in a 35 year old multiparous woman after the administration of the association mifepristone (Mifegyne) and sulprostone (Nalador) for therapeutic abortion. A coronary spasm induced by the analogue of the prostaglandin PGE 2x was though to be the causative mechanism and was reproduced by the Ergonovine test. The evolution of the ECG and myocardial scintigraphy suggested a slowly regressive stunned myocardium.

[Simultaneous thrombosis of the left anterior interventricular and right coronary arteries in a 27 year-old patient with protein S deficiency]. / Thrombose simultanée de l'interventriculaire antérieure et de la coronaire droite au cours d'un déficit en protéine S chez un sujet de 27 ans.

The authors report a case of myocardial infarction in a 27 year old patient by simultaneous thrombosis of the left anterior descending and right coronary arteries in an angiographically normal coronary circulation. The young age of the patient, the absence of the usual risk factors and a normal angiographic network after arterial recanalisation by angioplasty led to the search for a risk factor of thrombosis. This showed a qualitative deficiency of protein S and the absence of any other abnormality of coagulation or fibrinolysis. This case raises the question of a causal relationship between a hereditary protein S deficiency and thrombotic arterial occlusion.

[Severe persistent sub-shift of the ST segment following cardioversion in ventricular tachycardia]. / Grand sus-décalage du segment ST durable après cardioversion pour tachycardie ventriculaire.

The authors report a case of severe ST elevation after defibrillation which persisted for 6 days, suggesting acute myocardial infarction. The possible mechanisms of ST elevation after cardioversion and the unusually long duration of the ECG changes in this case are reviewed. The protective role of verapamil against myocardial damage is discussed.

[Prognostic indices of the normalization of left ventricular function in chronic aortic insufficiency following valve replacement]. / Indices prédictifs de normalisation de la fonction ventriculaire gauche dans les insuffisances aortiques chroniques après remplacement valvulaire.

The aim of this study of 31 patients was to identify M mode echocardiographic parameters predictive of normalisation of left ventricular function after valvular replacement for chronic aortic incompetence in order to determine the optimal time for surgery. Only patients with chronic, pure aortic incompetence (ventriculo-aortic pressure gradient less than or equal to 30 mmHG) were considered. At the time of investigation 4 patients were in functional Class I, 6 in Class II, 10 in Class III and 11 in Class IV (NYHA). M mode echocardiography was performed on an Echovideorex or an Irex System II echocardiograph. The following measurements were made and corrected for body surface area according to the recommendations of the American Society of Echocardiography; end systolic and end diastolic dimensions (mm), fractional shortening (%), end systolic and end diastolic wall thickness (mm), diastolic radius to wall thickness ratio, short axis myocardial surface area (cme), wall stress, end systolic stress (mmHg). The study comprised pre and postoperative studies with an interval of 22.7 +/- 12.5 months (range 5 to 46 months); the data obtained was compared with a control group of 10 normal subjects. The results showed that preoperative fractional shortening less than 28% was associated with an increased risk of persistent postoperative left ventricular dysfunction.

[Repolarisation anomalies simulating anterior myocardial infarction during ischemic cerebrovascular accident]. / Anomalies de la repolarisation simulant un infarctus du myocarde antérieur au cours d'un accident vasculaire cérébral ischémique.

The authors report the case of a woman admitted to hospital for minor trauma of the left hip and who presented major ST segment elevation on the second day suggesting an acute anterior wall myocardial infarction at the same time as a cerebrovascular accident. Complementary investigations and follow-up excluded the diagnosis of myocardial infarction with normalisation of the ECG on the 5th day. ECG changes during cerebrovascular accidents may be very variable ranging from extrasystoles to ST elevation mimicking myocardial infarction. They carry a poor prognosis with an increased risk of sudden death necessitating continuous ECG monitoring until the ECG reverts to normal. The cardiac involvement is not ischaemic but due to disease of the insular cortex of the brain which induces myocytolysis (centered around the intra-cardiac nerve endings) due to the sudden liberation of catecholamines. These lesions may be treated by propranolol or phentolamine.

[Role of HDL cholesterol in the prediction of exercise stress test abnormalities in asymptomatic high-risk patients]. / Rôle du HDL cholestérol dans la prédiction des anomalies à l'épreuve d'effort chez le sujet asymptomatique à haut risque.

The diagnosis of coronary artery disease in asymptomatic patients is useful in order to target therapeutic intervention in the patients at highest risk. Systematic testing of all asymptomatic adults with coronary risk factors is not feasible. The aim of this study, carried out in 950 healthy subjects, was to assess the predictive value of classical risk factors for positive exercise stress tests (EE). All subjects underwent stress testing using the Bruce protocol. Statistical analysis was performed by multiple logistic regression on half the samples, then by CART (Classification and Regression Trees) analysis on all subjects. Age, HDL-cholesterol and interaction between lipid lowering treatment and LDL-cholesterol were significantly correlated (p < 0.05) to a positive exercise stress test. In both groups, treated or untreated by lipid lowering drugs. CART identified HDL-cholesterol (< 0.40 g/l) as a predictive factor for positive stress testing. Subgroups of elderly patients (> or = 60 years) with probabilities of 20 to 28% for a positive stress test were identified. The authors conclude that the diagnosis of coronary artery disease by systematic exercise stress testing is potentially valuable in elderly patients with low HDL-cholesterol values.

[Value of continuous measurement of mixed venous oxygen saturation (SVO2) during coronary angioplasty]. / Intérêt de la mesure en continu de la saturation en oxygène du sang veineux mêlé (SVO2) pendant l'angioplastie coronaire.

Mixed venous oxygen saturation (SVO2) is an haemodynamic parameter of cardiovascular function. The object of this study was to measure SVO2 during percutaneous transluminal coronary angioplasty (PTCA) with prolonged balloon inflation in order to evaluate haemodynamic tolerance more precisely in two groups of patients. Twenty-six patients undergoing PTCA were divided into two groups: Group I, N = 15, with single vessel disease and good left ventricular function (EF: 61.63 +/- 10.1%); Group II, N = 11, with triple vessel disease and poor left ventricular function (EF: 48.05 +/- 14%; p < 0.05). Continuous monitoring of SVO2 with an oximetrix fiber optic catheter was performed in all patients during PTCA. Irrespective of the duration of balloon inflation, a significant correlation was observed between the changes in cardiac output and VSO2. Given that arterial oxygen pressure, global oxygen consumption and haemoglobin concentrations remained constant during balloon inflation, SVO2 was an indicator of cardiac output.