Intracardiac Thrombosis: An Uncommon Manifestation of Behçet's Disease.

Behçet's disease is a chronic multisystemic vasculitis of unknown etiology that evolves in relapses and remissions with a predominance of cutaneous and ocular lesions. It is more frequent in young adults, with a predominantly male population from the Mediterranean and Middle East. Cardiac involvement is rare (1 to 6%) but represents one of the most serious complications that can affect the three tunics of the heart. Cardiac thrombus is uncommon, its diagnosis relies mainly on echocardiography, and the development of the empirical treatment is based on immunosuppressive therapy and anticoagulation depending on the case. We report the case of a 21-year-old man admitted for a 2-month history of fever, loss of weight, and body condition in association with an intracardiac thrombus in the context of Behçet's disease. Intracardiac thrombus is a rare manifestation of Behçet's disease; the diagnosis must be made quickly in order to allow early management.

[Tetralogy of Fallot with absent pulmonary valve syndrome diagnosed in adulthood by infective endocarditis : Case of report]. / Agénésie de la valve pulmonaire avec communication interventriculaire découverte à l'âge adulte suite à une endocardite infectieuse : à propos d'un cas.

Absent pulmonary valve syndrome is a rare congenital heart disease. Associated with ventricular septal defect, it is considered a rare variant of Tetralogy of Fallot "Tetralogy of Fallot with absent pulmonary valve syndrome". It is characterized by its association with aneurysmal pulmonary arteries responsible for airways compression. Survival to adulthood of this unrepaired congenital heart disease is very rare, and the case of the patient we report in this article is added to the rare cases reported in the literature. Clinical tolerance depends on the degree of severity of the malformation and in particular on the importance of the aneurysmal dilation of the pulmonary arteries, thus determining the age of the diagnosis, the severity of symptoms, and the mode of evolution. Diagnosis of Tetralogy of Fallot with absent pulmonary valve syndrome must be established by transthoracic echography. Other investigations can be of capital contribution, such as thoracic computed tomography angiography and cardiac catheterization. The treatment is surgical and includes closure of the ventricular septal defect, relieve right ventricular outflow tract obstruction, and surgical reduction of the aneurysmal pulmonary arteries.

Biventricular arrhythmogenic cardiomyopathy diagnosed in a young patient: A case report with literature review.

Arrhythmogenic cardiomyopathy is a genetic heart muscle disease that typically affects the right ventricle. However, 2 other phenotypes affecting the left ventricle were recently discovered. Here, we report the case of an 18-year-old patient with biventricular arrhythmogenic cardiomyopathy, highlighting the challenges encountered in establishing this diagnosis. Diagnostic criteria for the left-sided phenotypic variants of arrhythmogenic cardiomyopathy were only introduced in 2020 by an international expert consensus document, known as the "Padua criteria," they are divided in 6 categories with an emphasis on morpho-functional ventricular abnormalities and structural myocardial tissue alterations to diagnose biventricular arrhythmogenic cardiomyopathy.

Accelerated junctional rhythm (AJR) revealing light-chain cardiac amyloidosis: A case report with literature review.

Introduction and importance: Cardiac amyloidosis (CA) is a rare condition, characterized by fibrillary proteins infiltration in the extracellular space of the heart. Even though many types of cardiac amyloidosis exist, light chain amyloidosis (AL) and transthyretin amyloidosis (ATTR) remain the most described forms. The diagnosis of amyloidosis represents a real challenge for clinicians, requiring both invasive and non-invasive investigations. Conduction defects and atrial arrhythmias are well known complications of cardiac amyloidosis. However, only a few studies have reported junctional rhythm a primary presentation of light chain cardiac amyloidosis (AL). An early diagnosis and proper management are crucial to improve the prognosis of this disease. Case presentation: Here, we report a rare case of a 48 year-old patient, in acutely decompensated heart failure, presenting an accelerated junctional rhythm (AJR) as initial presentation of light-chain cardiac amyloidosis. The diagnosis was made based on clinical, biological, radiological and histological findings. This case shows diagnostic difficulties and management of this rare disease.

Postural instability revealing infective endocarditis secondary to severe mitral stenosis: A case report with literature review.

INTRODUCTION AND IMPORTANCE: Infective endocarditis is a severe infection of the endocardial surface of the heart. One or more heart valves can be infected. However infective endocarditis complicating mitral stenosis is rare. It can be revealed by several and various symptoms such as fever and cardiac murmurs but also by complications such as focal neurological complaints. CASE PRESENTATION: We report a case of a febrile postural instability as the primary presentation of an infective endocarditis secondary to a severe mitral stenosis in a young patient with a history of mitral stenosis for which he benefited from percutaneous mitral dilation. The diagnosis was based on the modified Duke criteria. In this case, the treatment was based mainly on antibiotic therapy. The outcome was favorable; with a clinical, biological and radiological improvement. The patient was subsequently referred to the cardiovascular department for surgical treatment of his valve disease. CLINICAL DISCUSSION: Rheumatic heart disease is the main cause of mitral stenosis and its prevalence is higher in developing nations than in the rest of the world, yet only few articles have reported infective endocarditis as a complication of mitral stenosis. CONCLUSION: Mitral stenosis is rarely complicated by infective endocarditis, but this diagnosis should not be excluded in developing countries, particularly because of its high prevalence. To that end, clinicians should recognize its symptoms and complications and act accordingly to allow early treatment.