[Mavacamten (Camzyos ®) : first myosin modulator for obstructive hypertrophic cardiomyopathy treatment]. / Le médicament du moisMavacamten (Camzyos®) : premier inhibiteur réversible de l'ATP-ase de la myosine pour le traitement des cardiomyopathies hypertrophiques obstructives.

Mavacamten (Camzyos®) is a myosin modulator which reduces the interactions between myosin and actin. These are overly activated in hypertrophic cardiomyopathy (HCM), a source of exaggerated ventricular contractility, energy loss, and impairment of diastolic function (relaxation). The Food and Drug Administration (FDA) and the European Medication Agency (EMA) approved mavacamten for the treatment of symptomatic obstructive HCM (NYHA class II or III) in adult patients in 2022 and 2023, respectively. The medication is not yet reimbursed in Belgium. As seen in its clinical development studies, mavacamten reduces the intraventricular gradient, improves functional capacity and reduces symptoms. It also seems to be an innovative alternative to septal reduction. Mavacamten is usually very well tolerated knowing that, through its mechanism of action, it causes a dose-dependent and reversible reduction in left ventricular ejection fraction, which must therefore be closely monitored. The good tolerance and the effectiveness of mavacamten seem to be maintained over time. Consequently, the recent European Society of Cardiology Updated Guidelines on cardiomyopathy (ESC 09/2023) already recommend mavacamten in the pharmacological management of obstructive HCM.

Le mavacamten (Camzyos®) est un modulateur de la myosine qui diminue les interactions entre la myosine et l'actine. En effet, celles-ci sont trop activées dans la cardiomyopathie hypertrophique (CMH), source de contractilité ventriculaire exagérée, de déperdition énergétique et de troubles de la fonction diastolique (relaxation). Le mavacamten est approuvé par la Food and Drug Administration (FDA 2022) et l'European Medication Agency (EMA 2023) pour le traitement de la CMH obstructive (CMHO) symptomatique (classe NYHA II ou III) chez les patients adultes. Il n'est pas encore remboursé en Belgique. Les études pivots de son développement clinique ont montré que le mavacamten réduit le gradient intraventriculaire, améliore la capacité fonctionnelle et diminue les symptômes. Il semble aussi représenter une alternative innovante à la réduction septale. Le mavacamten est généralement très bien toléré, sachant que, par son mécanisme d'action, il entraîne une diminution dose-dépendante et réversible de la fraction d'éjection ventriculaire gauche, qui devra donc être surveillée étroitement. Sa bonne tolérance et son efficacité semblent se maintenir au cours du temps. En conséquence, les récentes recommandations de la Société Européenne de Cardiologie (ESC 2023) à propos des cardiomyopathies recommandent déjà le mavacamten dans l'arsenal pharmacologique de la prise en charge des CMHO.

[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.

[Cardio-oncology and cardio-obstetrics : crucial role of a multidisciplinary approach]. / Cardio-oncologie et cardio-obstétrique : importance d'une approche multidisciplinaire.

In this clinical case, we describe the cardio-oncological history and the complexity of the management of a patient presenting a breast cancer diasgnosed during pregnancy followed by a postpartum cardiomyopathy. A multidisciplinary approach is mandatory.

Dans ce cas clinique, nous décrivons l'histoire cardio-oncologique et la complexité de prise en charge d'une patiente présentant un cancer mammaire découvert lors d'une grossesse, puis, une cardiomyopathie du post-partum. Une approche multidisciplinaire s'avère indispensable.

[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).

Cardiac amyloidosis: a review of the literature.

Cardiac amyloidosis is a rare disease associated with severe morbidity and mortality. There are three main types of amyloidosis associated with cardiac involvement: light chain (AL), familial or senile (ATTR) and secondary amyloidosis (AA). Cardiac amyloidosis often results in heart failure with preserved left ventricular ejection fraction, may display echocardiographic features of restrictive cardiomyopathy associated with left ventricular hypertrophy or mimic hypertrophic obstructive cardiomyopathy. However, left ventricular systolic dysfunction and normal wall thickness can sometimes be encountered. Imaging studies (echocardiography, bone scintigraphy, cardiac magnetic resonance) and blood and urine analysis are usually the main tools for the diagnosis. Sometimes, a tissue biopsy may be necessary. Treatment, which is constantly improving, will be carried out on two fronts: treatment of the symptoms and complications that the disease already caused and prevention of additional amyloid deposits while managing the concomitant complications. The purpose of this article is to review the management of cardiac amyloidosis.

Prognostic Value of Non-Invasive Global Myocardial Work in Asymptomatic Aortic Stenosis.

This study aimed to evaluate the modification of non-invasive myocardial work (MW) indices related to aortic stenosis (AS) stages of cardiac damage and their prognostic value. The echocardiographic and outcome data of 170 patients, with asymptomatic moderate-to-severe AS and left ventricular ejection fraction (LVEF) ≥ 50%, and 50 age- and sex-comparable healthy controls were analysed. Primary endpoints were the occurrence of all-cause and cardiovascular death. Increased values of the global work index (GWI), global constructive work (GCW), and global wasted work (GWW) were observed in AS patients compared to controls (GWI: 2528 ± 521 vs. 2005 ± 302 mmHg%, GCW: 2948 ± 598 vs. 2360 ± 353 mmHg%, p < 0.001; GWW: 139 ± 90 vs. 90 ± 49 mmHg%, p = 0.005), with no changes in the global work efficiency. When patients were stratified according to the stages of cardiac damage, the GWI showed lower values in Stage 3−4 as compared to Stage 0 and Stage 2 (p = 0.024). During a mean follow-up of 30 months, 27 patients died. In multivariable Cox-regression analysis, adjusted for confounders, GWI (HR: 0.998, CI: 0.997−1.000; p = 0.034) and GCW (HR:0.998, CI: 0.997−0.999; p = 0.003) were significantly associated with excess mortality. When used as categorical variables, a GWI ≤ 1951 mmHg% and a GCW ≤ 2475 mmHg% accurately predicted all-cause and cardiovascular death at 4-year follow-up. In conclusion, in asymptomatic patients with moderate-to-severe AS, reduced values of GWI and GCW are associated with increased mortality. Therefore, the evaluation of MW indices may allow for a better identification of asymptomatic patients with moderate to severe AS and preserved LVEF whom are at increased risk of worse prognosis during follow-up.