No abstract

La maladie thromboembolique veineuse idiopathique peut être la première manifestation d'un cancer non encore diagnostiqué. La découverte précoce d'une néoplasie pourrait permettre une prise en charge plus rapide, et ainsi en réduire potentiellement la mortalité. Cependant, à l'heure actuelle, la nature et l'exhaustivité du dépistage à la recherche d'un cancer occulte chez ce groupe de patients restent encore débattues. L'objectif de cet article est de faire le point sur l'utilité du dépistage de cancer occulte en cas de maladie thromboembolique veineuse idiopathique. Unprovoked venous thromboembolism :should we look for occult cancer ?Idiopathic venous thromboembolic disease may be the first manifestation of a cancer yet undiagnosed. Early detection of neoplasia may allow a timely management and a reduction of mortality. However, the nature and extent of screening for hidden cancer is still debated. The purpose of this article is to review the usefulness of occult cancer screening in patients with idiopathic venous thromboembolism.

This is a 72 year-old man known for idiopathic HES with endomyocardial fibrosis and moderate aortic regurgitation, treated with corticosteroids and antithrombotics. Two months prior to admission, he develops progressive exertional dyspnea reaching NYHA 3 class, with concomitant peripheral edema and rise in NT-proBNP. Cardiac transthoracic work-up shows a preserved left ventricular ejection fraction, but more marked dilatation with decrease in left ventricular volume due to a large echodense mass in the apex. The transmitral flow is restrictive, and the aortic regurgitation moderate. In view of this rapid evolution, resection of the left ventricular mass with concomitant aortic valve replacement is performed. Pathology confirmed eosinophilic infiltration. First described in 1975, hypereosinophilic syndrome (HES) is a rare pathology that is defined as an absolute eosinophil count (>1,5 G/L) in the peripheral blood with eosinophil-mediated organ damage and /or dysfunction. We know three mains categories; primary (or neoplastic), secondary (or reactive) and idiopathic. Cardiac involvement is uncertain and cardiac injury does not clearly correlate with degree of peripheral eosinophilia. Heart tissue damage evolves through three stages (necrotic, intermediate and fibrotic). Endomyocardial fibrosis and eosinophilic myocarditis, also known as "Loeffler’s endocarditis", are major causes of morbidity and mortality among patients with HES. Echocardiography plays a crucial role in initial diagnosis of endomyocardial fibrosis, displaying myocardial wall thickening, signs of restriction (mitral inflow with large E wave and small A wave; E/A ratio greater than 2, short deceleration time less than 150 ms) and intracardiac thrombus (fixed mass embedding left ventricular apex). Valve regurgitation is another possible complication due to entrapment of the chordae tendinae and/or leaflets during the fibrotic stage of this disease. Cardiac MRI is another important diagnostic tool for precising cardiac involvement. Despite of steadily improving noninvasive diagnostic imaging methods, endomyocardial biosy still remains the gold standard exam and ultrasounds work-up is crucial for follow-up. In conclusion, endomyocardial fibrosis is a rare cause of restrictive cardiomyopathy characterized by echocardiographic sign of elevated filling pressure, progressive endomyocardial thickening, valve regurgitation and possible intracardiac thrombus formation. This case underlines the importance of echocardiography in initial diagnosis and regular follow-up of this type of patients, in order to adapt medical treatment and monitor hemodynamic evolution of the restrictive physiology and of valvular damage. Abstract P703 Figure. HES-3

No abstract

Background Loeffler endocarditis is a rare restrictive cardiomyopathy, characterized by hypereosinophilia and fibrous thickening of the endocardium causing progressive onset of heart failure and appearance of thrombi on the walls of the heart chambers. Case summary A 72-year-old man known for hypertension and dyslipidaemia consults for progressive dyspnoea up to New York Heart Association (NYHA) Classes 2–3 over 3 weeks. The biological balance sheet shows a high eosinophil level and an echocardiography shows a mild echodensity fixed to the left apex. After exclusion of a secondary cause of hypereosinophilia, diagnosis of endomyocardial fibrosis in the context of a hypereosinophilic syndrome (HES) is therefore retained. The patient’s clinical presentation with cardiac involvement leads us to start a treatment with corticosteroids. The patient is then regularly followed every 6 months with an initially stable course without complications. Two years later, he develops progressive signs of heart failure. Transthoracic echocardiography shows a left ventricular (LV) dilatation with a normal ejection fraction, but decreased volume due to a large echodense mass in the apex, and moderate aortic regurgitation caused by myocardial infiltration. In view of this rapid evolution, resection of the LV mass with concomitant aortic valve replacement is performed. Pathology confirms eosinophilic infiltration. The clinical course is very good with a patient who remains stable with dyspnoea NYHA Classes 1–2, and echocardiography at 1 year shows a normalization of LV filling pressure. Discussion HES represents a heterogeneous group of disorders characterized by overproduction of eosinophils. One of the major causes of mortality is associated cardiac involvement. Endocardial fibrosis and mural thrombosis are frequent cardiac findings. Echocardiography plays a crucial role in initial diagnosis of endomyocardial fibrosis, and for regular follow-up in order to adapt medical treatment and monitor haemodynamic evolution of the restrictive physiology and of valvular damage caused by the disease’s evolution. This case also shows that surgery can normalize filling pressure and allow a clear improvement on the clinical condition even at the terminal fibrotic state.