A giant left ventricular pseudoaneurysm in Behçet's disease: a case report.

Behçet's disease is a chronic autoimmune disease with vascular complications that are most frequently manifested as thromboembolism in veins and pseudoaneurysm in arteries. We report the case of a 13-year-old boy admitted for clinical and biological signs of rheumatic fever associated with chest pain. The clinical examination found heart sounds with a discrete systolic murmur of mitral regurgitation. The electrocardiogram showed a microvoltage with diffuse repolarisation disorder. Biologically, he had inflammatory syndrome. Transthoracic echocardiography showed circumferential pericardial effusion with anterosepto-apical hypokinesia of the left ventricle with systolic dysfunction, and a minimal mitral regurgitation. The patient was treated by corticotherapy and antibiotherapy. The outcome was marked by orogenital aphthous ulceration and decreased visual acuity related to intermediate uveitis. The retinal angiography showed a vasculitis. The late appearance of this symptom led to the right diagnosis of Behçet's syndrome. Transthoracic echocardiography showed a hypokinetic dilated cardiomyopathy left ventricular with septo apical and anterior akinesia and severe systolic dysfunction, with a defect of the inferior septal with a collar communicating the left ventricle with a giant pseudo aneurysm. Magnetic resonance imaging showed a giant pseudoaneurysm communicating with the left ventricle. The coronary computed tomography was normal. The patient had undergone surgical treatment for the pseudoaneurysm with good outcomes.

[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.

Complete heart block in a young adult with non-isolated congenitally corrected transposition of the great arteries: Case report.

Introduction and importance: Congenitally corrected transposition of the great arteries (ccTGA) or L-looped transposition of the great arteries (L-TGA) is a very rare and complex form of congenital heart disease. The majority of patients with ccTGA have at least one or more associated congenital heart disorders, essentially ventricular septal defects. Patients with ccTGA can remain asymptomatic for a long time and the diagnosis can sometimes be made late in life at the stage of complications. Case presentation: Here, we report a rare case of a 19-year-old patient, with no medical or surgical history, presenting a complete heart block as initial presentation of a ''non-isolated'' ccTGA. The diagnosis is made essentially by echocardiography.This case aims to show diagnostic difficulties of this rare congenital heart disease and be aware of the risk of its relative complications.

Acute ST-elevation myocardial infarction (STEMI) in a young woman with unknown mitral stenosis and atrial fibrillation: Case report.

Introduction and importance: Chronic rheumatic heart disease is the most common cause of mitral stenosis. It remains a major public health problem. In almost half of the cases, paroxysmal or chronic atrial fibrillation occurs during the evolution of mitral stenosis, thereby exposing to an increased risk of thrombo-embolic events.Whereas the most frequent site for embolism is the cerebral circulation, any organ may be involved, especially the coronary circulation, resulting in a myocardial infarction (MI). Case presentation: Here, we report a rare case of a 50-year-old patient, with no risk factors for cardiovascular disease, presenting an acute ST-elevation myocardial infarction (STEMI) as initial presentation of unknown mitral stenosis with atrial fibrillation and strongly suggesting an embolic origin. The diagnosis was made based on the national cerebral and cardiovascular center (NCVC) criteria for the clinical diagnosis of coronary artery embolism (CE). Coronary angiography showed a distal thrombus in the right coronary artery that has been medically treated. The outcome was favorable and the patient was referred after that for mitral valve replacement.

Abnormal Origin of the Left Coronary Artery From the Pulmonary Artery Discovered at Age 67: What to Do?

Anomalous left coronary artery from the pulmonary artery (ALCAPA) was described by Abbott in 1908. In most cases, it is an isolated lesion, being the most common cause of myocardial ischemia in children. The associated mortality rate without intervention reaches 90% during childhood. We report the case of a 67-year-old woman, who underwent coronary angiography for investigation of atypical chest pain and was found to have ALCAPA. The patient refused surgery and has remained asymptomatic on a medical regimen.

Unusual findings in secondary hypertension: double orifice mitral associated to aortic coarctation, bicuspid aortic valve, and ventricular septal defect.

Double orifice mitral valve is a rare congenital anomaly presenting as the division of the mitral orifice into two anatomically distinct orifices, it is most often associated with other congenital heart defects such as left-sided obstructive lesions, ventricular septal defects or aortic coarctation. We report the case of a 15 year's old boy, admitted for arterial hypertension, auscultation revealed a rude aortic systolic murmur. Femoral pulses were weak. Owing to the suspicion of aortic coarctation, transthoracic echocardiography was performed, the aortic coarctation with dilation of the aorta proximal to the stenosis was confirmed and bicuspid aortic valve was found with good function. The mitral valve was dysmorphic, having two orifices; it was divided into 2 separate valve orifices by a fibrous bridge. No mitral or aortic regurgitation was documented by color Doppler flow imaging. The left ventricular ejection fraction was normal. There was a small peri membranous ventricular septal defect with left to right shunt. Owing to the severity of the aortic coarctation and taking into account the anatomy and characteristics of the patient, he was made a surgical correction of aortic coarctation with good outcome.

Simple D-transposition of great arteries operated at the age of 11 years.

The simple transposition of the great arteries is a lethal congenital heart disease. The life expectancy of unoperated patients is about 9 months. We report the original observation of a girl with unoperated simple transposition of the great arteries, who survived until the age of 11 years. An atrial switch was successfully performed according to the technique of Senning-Mustard.

Transposition of the great arteries in situs inversus totalis.

A rare case of a newborn with situs inversus totalis associated with simple transposition of the great arteries is reported. A successful anatomical surgical repair was accomplished on day 10 of life, consisting of an arterial switch operation with reimplantation of the coronary arteries.

Use of magnetic resonance imaging in assessment of constrictive pericarditis: a Moroccan center experience.

BACKGROUND: The diagnosis of constrictive pericarditis continues to be a clinical challenge. Magnetic resonance imaging provides excellent visualization of the pericardium. The aim of our study is to clarify the contribution of this non invasive exploration in the diagnosis of constrictive pericarditis in our center. METHODS: we conducted a prospective study over a period of two years, since 2008, covering a series of patients (n = 11), mean age 44 ± 15 years, in whom constrictive pericarditis was suspected clinically and on transthoracic echocardiography. We studied its characteristics on magnetic resonance imaging. RESULTS: Magnetic resonance imaging confirmed the diagnosis showing pericardial thickening in all cases, measuring 8.2 +/- 2.6 mm on average, circumferential in 64%, and localized in 36%. The imaging data, particularly pericardial thickening and its topography, were confirmed by surgical exploration, and results were concordant in all cases. CONCLUSION: Magnetic resonance imaging is a powerful tool to establish constrictive pericarditis diagnosis.