[New guidelines on the diagnostic and therapeutic management of hypertrophic cardiomyopathy]. / Nouvelles recommandations pour le diagnostic et la prise en charge thérapeutique de la cardiomyopathie hypertrophique.

Hypertrophic cardiomyopathy is a disease characterized by left ventricular hypertrophy (with or without right ventricular hypertrophy) not explained by loading conditions, the origin of which may be genetic and whose phenotypic expression is highly variable. The novelties in terms of diagnosis, clinical development, and management have been the subject of an update of the recommendations of the European Society of Cardiology (ESC).

La cardiomyopathie hypertrophique est une maladie caractérisée par une hypertrophie ventriculaire gauche (avec ou sans hypertrophie ventriculaire droite) non expliquée par les conditions de charge, dont l'origine peut être génétique et dont l'expression phénotypique est très variable. Les nouveautés en termes diagnostique, de mise au point, et de prise en charge ont fait l'objet d'une mise à jour des recommandations de la Société Européenne de Cardiologie (ESC).

[Aortic stenosis : new developments in management]. / Sténose aortique : nouveautés thérapeutiques et diagnostiques.

Aortic stenosis (AS) is the most common valve disease in our countries; most often of degenerative origin, its prevalence is constantly increasing due to the aging of the population. Its development is a continuum ranging from aortic sclerosis to severe aortic stenosis, the diagnosis of which is essentially based on transthoracic echocardiography, which will allow classification into subcategories. Even if today no treatment makes it possible to prevent the progression of the disease, the management has clearly evolved with an increasingly important place for new approaches to valve replacement by the percutaneous route, and an indication of management at an increasingly early stage.

La sténose aortique (SA) est la valvulopathie la plus fréquente dans les pays occidentaux, le plus souvent d'origine dégénérative, et sa prévalence augmente constamment étant donné le vieillissement de la population. Son développement est un continuum allant de la sclérose aortique vers la sténose aortique serrée, dont le diagnostic repose essentiellement sur l'échocardiographie trans-thoracique qui permettra une classification en sous-catégories. Même si aujourd'hui aucun traitement ne permet d'empêcher la progression de la maladie, sa prise en charge a nettement évolué avec une place de plus en plus importante pour les techniques de remplacement valvulaire par voie percutanée, et une indication de prise en charge qui sera posée de façon de plus en plus précoce.

Cardiac Damage and Conduction Disorders after Transcatheter Aortic Valve Implantation.

Recently, a staging system using 4 grades has been proposed to quantify the extent of cardiac damage associated with aortic stenosis (AS), namely AS-related cardiac damage staging (ASCDS). ASCDS is independently associated with all-cause mortality and important clinical outcomes. To evaluate whether it might be associated with the occurrence of conduction system disorders after TAVI, a total of 119 symptomatic patients with severe AS who underwent a TAVI were categorized according to ASCDS: group 1 (13.5%): no or LV damage; group 2 (58.8%): left atrial/mitral valve damage, atrial fibrillation (AF); group 3 (27.7%): low-flow state, pulmonary vasculature/tricuspid valve/RV damage. After TAVI, 34% of patients exhibited LBBB and 10% high-degree atrioventricular block (HD-AVB). No patient in group 1 developed HD-AVB whereas new LBBB was frequent in groups 2 and 3. Twenty-one patients presented with paroxysmal AF with a higher rate for each group increment (group 1: n = 0, 0%; group 2: n = 11, 15.7%; group 3: n = 10, 30.3%) (p = 0.012). Patients in group 3 had the higher rate of permanent pacemaker implantation (PPMI) (group 1: n = 1, 6.3%; group 2: n = 7, 10%; group 3: n = 9, 27.3%) (p = 0.012). In conclusion, ASCDS might help identify patients at higher risk of conduction disorders and PPMI requirement after TAVI.

Comparison of the Full Outline of UnResponsiveness and Glasgow Liege Scale/Glasgow Coma Scale in an intensive care unit population.

BACKGROUND: The Full Outline of UnResponsiveness (FOUR) has been proposed as an alternative for the Glasgow Coma Scale (GCS)/Glasgow Liège Scale (GLS) in the evaluation of consciousness in severely brain-damaged patients. We compared the FOUR and GLS/GCS in intensive care unit patients who were admitted in a comatose state. METHODS: FOUR and GLS evaluations were performed in randomized order in 176 acutely (<1 month) brain-damaged patients. GLS scores were transformed in GCS scores by removing the GLS brainstem component. Inter-rater agreement was assessed in 20% of the studied population (N = 35). A logistic regression analysis adjusted for age, and etiology was performed to assess the link between the studied scores and the outcome 3 months after injury (N = 136). RESULTS: GLS/GCS verbal component was scored 1 in 146 patients, among these 131 were intubated. We found that the inter-rater reliability was good for the FOUR score, the GLS/GCS. FOUR, GLS/GCS total scores predicted functional outcome with and without adjustment for age and etiology. 71 patients were considered as being in a vegetative/unresponsive state based on the GLS/GCS. The FOUR score identified 8 of these 71 patients as being minimally conscious given that these patients showed visual pursuit. CONCLUSIONS: The FOUR score is a valid tool with good inter-rater reliability that is comparable to the GLS/GCS in predicting outcome. It offers the advantage to be performable in intubated patients and to identify non-verbal signs of consciousness by assessing visual pursuit, and hence minimal signs of consciousness (11% in this study), not assessed by GLS/GCS scales.

A French validation study of the Coma Recovery Scale-Revised (CRS-R).

PRIMARY OBJECTIVE: The aim of the present study was to explore the concurrent validity, inter-rater agreement and diagnostic sensitivity of a French adaptation of the Coma Recovery Scale-Revised (CRS-R) as compared to other coma scales such as the Glasgow Coma Scale (GCS), the Full Outline of UnResponsiveness scale (FOUR) and the Wessex Head Injury Matrix (WHIM). RESEARCH DESIGN: Multi-centric prospective study. METHOD AND PROCEDURES: To test concurrent validity and diagnostic sensitivity, the four behavioural scales were administered in a randomized order in 77 vegetative and minimally conscious patients. Twenty-four clinicians with different professional backgrounds, levels of expertise and CRS-R experience were recruited to assess inter-rater agreement. MAIN OUTCOMES AND RESULTS: Good concurrent validity was obtained between the CRS-R and the three other standardized behavioural scales. Inter-rater reliability for the CRS-R total score and sub-scores was good, indicating that the scale yields reproducible findings across examiners and does not appear to be systematically biased by profession, level of expertise or CRS-R experience. Finally, the CRS-R demonstrated a significantly higher sensitivity to detect MCS patients, as compared to the GCS, the FOUR and the WHIM. CONCLUSION: The results show that the French version of the CRS-R is a valid and sensitive scale which can be used in severely brain damaged patients by all members of the medical staff.

The locked-in syndrome : what is it like to be conscious but paralyzed and voiceless?

The locked-in syndrome (pseudocoma) describes patients who are awake and conscious but selectively deefferented, i.e., have no means of producing speech, limb or facial movements. Acute ventral pontine lesions are its most common cause. People with such brainstem lesions often remain comatose for some days or weeks, needing artificial respiration and then gradually wake up, but remaining paralyzed and voiceless, superficially resembling patients in a vegetative state or akinetic mutism. In acute locked-in syndrome (LIS), eye-coded communication and evaluation of cognitive and emotional functioning is very limited because vigilance is fluctuating and eye movements may be inconsistent, very small, and easily exhausted. It has been shown that more than half of the time it is the family and not the physician who first realized that the patient was aware. Distressingly, recent studies reported that the diagnosis of LIS on average takes over 2.5 months. In some cases it took 4-6 years before aware and sensitive patients, locked in an immobile body, were recognized as being conscious. Once a LIS patient becomes medically stable, and given appropriate medical care, life expectancy increases to several decades. Even if the chances of good motor recovery are very limited, existing eye-controlled, computer-based communication technology currently allow the patient to control his environment, use a word processor coupled to a speech synthesizer, and access the worldwide net. Healthy individuals and medical professionals sometimes assume that the quality of life of an LIS patient is so poor that it is not worth living. On the contrary, chronic LIS patients typically self-report meaningful quality of life and their demand for euthanasia is surprisingly infrequent. Biased clinicians might provide less aggressive medical treatment and influence the family in inappropriate ways. It is important to stress that only the medically stabilized, informed LIS patient is competent to consent to or refuse life-sustaining treatment. Patients suffering from LIS should not be denied the right to die - and to die with dignity - but also, and more importantly, they should not be denied the right to live - and to live with dignity and the best possible revalidation, and pain and symptom management. In our opinion, there is an urgent need for a renewed ethical and medicolegal framework for our care of locked-in patients.

Heart enlargement after thrombolysis for unsuccessful resuscitation.

Thrombolysis has been recently reported for the treatment of unsuccessful out-of-hospital cardiopulmonary resuscitation. This case report presents the first case of mediastinal hematoma following thrombolysis in this setting. Chest X-ray and hemodynamic pattern mimicked pericardial effusion with cardiac-filling compromise. Diagnosis was established by transesophageal echocardiography and chest computerized tomodensitometry.

NGAL Usefulness in the Intensive Care Unit Three Hours after Cardiac Surgery.

Objective. Neutrophil gelatinase-associated lipocalin (NGAL) measured by a research ELISA is described as an early marker of acute kidney injury (AKI). The aim of this study is to define the usefulness of plasma NGAL (pNGAL) and urine NGAL (uNGAL) measured with platform analysers to detect AKI 3 hours after cardiac surgery in fifty adult patients. Methods and Main Results. pNGAL and uNGAL were measured before and 3 hours after cardiac surgery. AKI, defined following the acute kidney injury network definition, was observed in 17 patients. pNGAL was >149 ng/mL in 8 patients with AKI, two of them died in the follow-up. We also observed elevated pNGAL in 8 patients without AKI. Only one uNGAL was >132 ng/mL among the 15 AKI patients. Sensitivity of pNGAL for prediction of AKI is 47% and specificity is 75.7%. The positive likelihood ratio (LR+) is 1.9 and negative likelihood ratio (LR-) is 0.7. uNGAL performance is slightly improved when reported to urinary creatinine. Following this study, a ratio >62 ng/mg assure a sensitivity of 66.6% and a specificity of 78.5%. LR+ is 3 and a LR- is, 0.42. Conclusions. Three hours after cardiac surgery, pNGAL predicts AKI with a low sensitivity and specificity.

Locked-in syndrome in children: report of five cases and review of the literature.

The locked-in syndrome is a rare neurologic disorder defined by (1) the presence of sustained eye opening; (2) preserved awareness; (3) aphonia or hypophonia; (4) quadriplegia or quadriparesis; and (5) a primary mode of communication that uses vertical or lateral eye movement or blinking. Five cases are reported here, and previous literature is reviewed. According to the literature, the most common etiology of locked-in syndrome in children is ventral pontine stroke, most frequently caused by a vertebrobasilar artery thrombosis or occlusion. In terms of prognosis, 35% of pediatric locked-in syndrome patients experienced some motor recovery, 26% had good recovery, 23% died, and 16% remained quadriplegic and anarthric. These findings raise important ethical considerations in terms of quality of life and end-of-life decisions in such challenging cases.

Cerebral processing of auditory and noxious stimuli in severely brain injured patients: differences between VS and MCS.

We review cerebral processing of auditory and noxious stimuli in minimally conscious state (MCS) and vegetative state (VS) patients. In contrast with limited brain activation found in VS patients, MCS patients show activation similar to controls in response to auditory, emotional and noxious stimuli. Despite an apparent clinical similarity between MCS and VS patients, functional imaging data show striking differences in cortical segregation and integration between these two conditions. However, in the absence of a generally accepted neural correlate of consciousness as measured by functional neuroimaging, clinical assessment remains the gold standard for the evaluation and management of severely brain damaged patients.