Pulmonary hypertension and von Recklinghausen's disease: association and therapeutic difficulties.

The neurofibromatosis type 1 (NF1) or Von Recklinghausen's disease is a genetic disorder. The café-au-lait spots and neurofibromas are the most common manifestations. Respiratory symptoms are rare in this disease, described as neurofibromas, infiltrative lesions, cysts, bubbles or emphysema. Pulmonary hypertension is rarely reported. It is due to the plexiform lesions in pulmonary arterioles or to parenchymal lung lesions reducing the vascular bed. We report a case of idiopathic precapillary pulmonary hypertension in a young patient with Von Recklinghausen's disease.

Epidemiological and evolutionary characteristics of heart failure in patients with left bundle branch block - A Moroccan center-based study.

BACKGROUND: In patients with heart failure, left bundle branch block (LBBB) seems to be associated with an increased risk of cardiovascular mortality. PURPOSE: The purpose of this study is to determine the in-hospital outcome of congestive heart failure patients with LBBB versus those without. METHODS: We conducted a prospective observational study at the Department of Intensive Care and Rhythmology at the Mohammed V Military Hospital of Rabat, where 330 patients were admitted for heart failure between January 2008 and September 2012. Screening out patients with missing data yielded a cohort of 274 patients. Among the 274 patients, only 110 had LBBB and a left ventricular ejection fraction lower than 50%. We randomly selected a subset of 110 patients diagnosed as non-LBBB to ensure a significant statistical comparison between LBBB and non-LBBB patients. We therefore considered two groups in our analysis: 110 heart failure (HF) patients with LBBB and 110 HF patients without LBBB. Patients with incomplete records were excluded. RESULTS: Male gender was dominant in both groups (82.7% vs. 66.7%, p = 0.005). Patients with LBBB had a higher prevalence of idiopathic dilated cardiomyopathy (39.1% vs. 4.8%, p < 0.001); and a higher prevalence of previous hospitalization for heart failure (64.5% vs. 23.3%, p < 0.001). The left ventricular ejection fraction was significantly lower in the group with LBBB (25.49% vs. 39.53%, p < 0.001). Age, cardiovascular risk factors, rhythmic and thromboembolic complications did not significantly differ. In patients with LBBB, 61.8% received cardiac resynchronization therapy performed both during the index hospital stay (50.9%) and previously (10.9%). Hospital outcome was marked by 20 in-hospital deaths in the group with LBBB and eight deaths in the group without LBBB (p = 0.008). CONCLUSION: Our analysis emphasizes increased in-hospital mortality and higher disease severity, over a short period of stay, in heart failure patients with left bundle branch block.

[Myocardial infarction as complication of acute pancreatitis]. / Pancréatite aiguë compliquée d'infarctus du myocarde à coronaires normales.

We report a case of an acute pancreatitis complicated by myocardial infarction with normal coronary arteries on angiography. This observation presents a double interest. First, it illustrates unusual forms of coronary disease represented by the non-atherosclerotic infarction. On the other hand, it shows that the multiple visceral damage associated with acute pancreatitis can include myocardial infarction which complications aggravate prognosis.

[Early surgery in infective endocarditis. Retrospective study apropos of 30 cases]. / Chirurgie précoce dans l'endocardite infectieuse. Etude rétrospective à propos de 30 cas.

AIM: The aim of this study is to stress the interest of the early surgery in infective endocarditis (IE), its indications and prognostic implications. METHODS: It is a retrospective descriptive study of 30 cases (29 men and 1 woman with an average age of 35+/-12 years) with IE underwent surgery management in the acute phase between September 1993 and June 2005. RESULTS: They were 25 rheumatic lesions, 2 aortic bicuspids and 3 mechanical valves prosthesis. Four twenty-six percent of the patients were operated for hemodynamic deterioration and 10% for embolic complication. We report 3 cases (that is to say 10%) of IE late form on prosthesis. Three patients died in the first post operative month by respectively total desinsertion of mitral prosthesis on peroperative, 1 septic shock at the 13th post operative day and 1 tamponade at the 14th postoperative day. On 72 months an average follow-up, 26 were controlled regularly: 25 evolved favourably and 1 died in third postoperative year (severe heart failure). CONCLUSION: A high early surgery rate is related to good long term results and does not increase in hospital mortality. The reduced mortality was particularly evident among patients with moderate to severe congestive heart failure.

[Silent myocardial infarction and antiphospholipid antibody syndrome]. / Infarctus du myocarde silencieux chez une jeune femme de 18 ans présentant un syndrome des anticorps antiphospholipides.

We describe a case of 18-year-old woman followed for 3 years for systemic lupus erythematosis (SLE) complicated of a nephrectomy. Having like only factor of cardiovascular risk a balanced arterial hypertension. The patient was hospitalized because of choreo-athetosic's movement. We discovered fortuitously during this hospitalization an inferior myocardial necrosis as well as a mitral regurgitation. Coronary angiography was normal and the ventriculography showed an akinesy in the inferior territory. Biology made it possible to pose the diagnosis of antiphospholipid antibody syndrome (APS) on (SLE). We suppose that surgery started myocardial necrosis and underline through this case interest of early identification and appropriate treatment of APS as well as a narrow monitoring particularly in young patients candidates to surgery.

[Cardiac localization of non Hodgkin's lymphoma: a study on four cases]. / Localisations cardiaques du lymphome non hodgkinien: à propos de quatre cas.

INTRODUCTION: Cardiac localization of non-Hodgkin's lymphoma is rare and usually seen in the latest phase of the disease. However, its frequency is certainly under-estimated because 20% of patients who die of lymphoma have a heart involvement at autopsy. EXEGESIS: We report four cases of cardiac involvement of non-Hodgkin's lymphoma with various revelation modalities. Features of cardiac lymphoma are multiple and not specific. Echocardiography can be helpful in finding cardiac localizations, which can involve every cardiac structure. Histological proof of such an involvement is sometimes difficult to obtain. CONCLUSION: Prognosis of cardiac lymphoma remains poor despite the real progress in the treatment of lymphomas.

[Heart and sports: modifications of electrocardiogram, late potentials and echocardiography. Study of 75 sportsmen and 46 witnesses]. / Coeur du sportif: modifications électriques et échocardiographiques au repos. Etude de 75 sportifs et de 45 témoins.

The authors report the results of prospective study, which compared 75 sports subjects and 45 witnesses. All subjects were male, the differentiating parameter being the type of sport practiced. This work analysed surface Electrocardiogramme, thransthoracic echocardiography and high amplification ECG. The statistical study used the student test t compare means and the Chi2 test for the percentages, the signification limit was fixed to 5%. Clinically, our two series didn't show a significant statistical difference, concerning: age, weight, height or arterial pressure. On the electrocardiographical level, the sport's men have a lower cardiac frequency (p = 0.005), a larger PR space (p = 0.05), an important Sokolow parameter (p < 0.005), and repolarisation disorders represented, essentially by negative T waves (p = 0.02) and an upper movement of ST segment in V2-V3 (p < 0.005). Echocardiography showed a dilatation of the right cavities: right auricular (p = 0.0125) and right ventricular (p = 0.025). Move over, it has been showed that the sport's men left ventricular walls were tabor (septal wall, p = 0.0125), (posterior wall, p = 0.025), despite a difference in the values of the left ventricular telediastolic diameter (4 mm average in the two series). The signification limit was not reached and it was also showed that the left auricular was also dilated (p = 0.025). The study of the delayed Potentials, tried to bring an explication to certain sudden deaths of sport's men which are to date unexplained and which could have a rhythmical origin? It is also to be noted that sport's men present more delayed ventricular Potentials. However, the statistical signification was not reached (p = 0.07).

[Endomyocardial fibrosis in Behcet's disease: a case report of a pseudo-tumoral form]. / Fibrose endomyocardique au cours de la maladie de Behçet: un cas à forme pseudo-tumorale.

Endomyocardial fibrosis is very rare in Behcet's disease. The authors report the case of a 28 year old patient with Behcet's disease complicated by a pseudo-tumoral right ventricular formation on echocardiography. This misleading appearance suggested the diagnosis of cardiac thrombus or tumour and led to a surgical approach which revealed a fibrous moderator band suggesting endomyocardial fibrosis, confirmed by antomopathological analysis. Besides the originality of this case and the unusual pseudo-tumoral presentation, the authors underline the difficulties of establishing the diagnosis, despite the advances of medical imaging. The pseudo-tumoral intra-cardiac lesion in a suggestive clinical context (Behcet's disease) should raise suspicion of the diagnosis of endomyocardial fibrosis.

[Papillary fibroelastoma of the large mitral valve disclosed by ischemic cerebrovascular accident: a case report]. / Fibroélastome papillaire de la grande valve mitrale révélé par un accident vasculaire cérébral ischémique: à propos d'un cas.

Thirty-four observations of papillary fibroelastoma (PFE) of the mitral valve have to date been reported in the literature. Our findings are the tenth so far documented on PFE revealed by a cerebral ischaemic vascular accident. The echocardiographic investigation detected a pedunculated tumour of the mitral valve 9 mm in diameter, and associated with moderate mitral leakage. The tumour was surgically excised tumour confirmed the original PFE diagnosis. The evolution seven years after surgery is favourable, with no signs of tumour recurrence and good functioning of the mitral valve system.

[Traumatic left ventricle-right atrial communication. A case report]. / Communication ventricule gaucheoreillette droite traumatique. A propos d'un cas.

The authors report the case of an acquired left ventricle-right atrial communication after open chest trauma. The initial clinical presentation was a haemothorax and haemopericardium responding well to emergency surgical drainage. Secondarily, a systolic murmur suggesting a ventricular septal defect and signs of right heart failure were observed. Colour Doppler echocardiography led to the diagnosis of a left ventricle-right atrial communication associated with tricuspid regurgitation with dilatation of the right heart chambers and pulmonary hypertension. At surgery, a defect in the membranous interventricular septum was confirmed with rupture of the septal tricuspid leaflet causing tricuspid regurgitation. The surgeon closed the defect with a patch and performed a De Vega tricuspid valvuloplasty. The postoperative outcome was uneventful.

[Angiosarcoma of the right atrium. Presentation of a surgically treated case and comparison with data of the literature]. / Angiosarcome de l'oreillette droite. Présentation d'un cas opéré et comparaison avec les données de la littérature.

The authors report the case of a 28-year-old woman referred to their department by a respiratory medicine department with an inferior mediastinal tumour arising from the right atrium, presenting in the form of dysponea, dry cough and chest pain associated with a general syndrome composed of fever, weight loss and physical asthenia. Physical examination revealed a superior vena cava syndrome, the electrocardiogram showed diffuse repolarization disorders and the chest x-ray showed an opacity of the anterior and inferior mediastinum. The diagnosis of tumour of the right atrium was based on echocardiography and thoracic CT scan. Subtotal surgical resection under cardiopulmonary bypass allowed examination of the histological type of the tumour. After routine chemotherapy, despite negative secondary staging and a favourable immediate course, the patient died 11 months after the operation in a context of local recurrence and hepatic and bone metastases.