[Behcet disease: uncommon cause of myocardial infarction]. / Maladie de Behçet: cause rare d'inarctus du myocarde.

A 25-year-old man who was known to have Behçet's syndrome and who has no coronary risk factors suffered an acute anterior wall myocardial infarction which was complicated by a ventricular fibrillation. The diagnosis of Behçet's syndrome was based on recurrent thrombophlebitis, genital and oral aphtoses, posterior uveitis, positive pathergy test and HLAB51. About 20 cases of myocardial infarction were reported in the literature but the etiopathogeny, the causal relationship and the treatment are yet unknown.

[Mitral stenosis secondary to Hurler's syndrome]. / Rétrécissement mitral secondaire à un syndrome de Hurler.

Valvular disease in mucopolysaccharidosis type I-Hurler (MPS/1H) is relatively common, but mitral stenosis is very rare in this genetic abnormality. The authors describe the case of a 16-year old girl with Hurler's syndrome diagnosed at 4 years of age. The morphological features were characteristic: bridged nose, thickened lips, macroglassia, short neck (gargoylism, short, thick fingers and limitation of brachial and fore-arm flexion. She presented with stage II dyspnoea and paroxysmal nocturnal dyspnoea. Radiological and echocardiographic studies revealed severe mitral stenosis with haemodynamic complications requiring mitral valve replacement. Anatomopathological analysis of the mitral valve confirmed mucopolysaccharide deposits as the cause of this particular case of mitral stenosis.

[Place of definitive cardiac stimulation in cardiomyopathies and atrial arrhythmia]. / Place de la stimulation cardiaque definitive au cours des cardiomyopathies et des arythmies atriales.

Definitive cardiac stimulation ralatually used for brady cardia are going to be in the third millennium the specific therapy of arrhythmia and cardiomyopathy. In the last 10 years, may progress occur in the use of cardiac stimulation as hemodynamic therapy of obstructions hypertrophic and dilated cardiomyopathies resistant to conventional medical treatment. Moreover auricular stimulation present antiarrhythmic effects in some atrial arrhythmias as auricular fibrillations auricular diseases and atypical flutters. It confirmed by prospective multicentric randomized trials cardiac stimulation may represent in the future a good physiopathologic and efficient treatment free from the side effects of drugs that remain partially active. These perspectives needs to be modulated by the known and acceptable risks of a definitive cardiac stimulation.

[Infectious enterococcal endocarditis associated with Laubry and Pezzi syndrome]. / Endocardite infectieuse a enterocoque sur syndrome de Laubry et Pezzi.

Laubry and Pezzi syndrome is a rare but serious complication of ventricular septal defect that increase the infectious endocarditis risk. Authors report a case of an 18 years old young girl presenting an enterococcus infectious endocarditis associated to Laubry and Pezzi syndrome. Initial course is not favourable requiring a surgical treatment in the acute stage. Bacterial endocarditis combined with Laubry and Pezzi syndrome have a poor prognosis needing observation and strict preventive precautions when a favoring factor is present.

[Right mid-ventricular stenosis with intact interventricular septum]. / Sténose médio-ventriculaire droite avec septum interventriculaire intact.

Pure infundibular stenosis of the right ventricle is a rare congenital anomaly. Right midventricular stenosis with intact interventricular septum is even rarer. It consists of obstruction of the trabecular zone of the right ventricle by a hypertrophied moderator band with or without abnormal muscle bundles. This anomaly can now be diagnosed by transthoracic echocardiography, while haemodynamic and angiographic diagnosis is reserved for doubtful cases. Two cases of midventricular stenosis are reported. In both patients, the diagnosis was suspected bu ultrasonography which showed a right intraventricular gradient of about 80 mmHg without identifying the exact site (infundibular or midventricular). The diagnosis was corrected by the operative findings. A good postoperative course was observed for both patients.