[Tuberous sclerosis and cardiac tumor (case report)]. / Sclérose tubéreuse de Bourneville et tumeur cardiaque (à propos d'un cas).

We report the case of a cardiac tumor, which is found in a systematic cardiac examination of an 11 year old girl who had a tuberous sclerosis. The interest of this observation is to show the importance of a general examination, especially cardiac, within this pathology. Tuberous sclerosis is an hereditary disease, associated with rhabdomyomas in 50% of cases or more. Cardiac rhabdomyomas are frequently multiple and detected in utero in some cases. Conversely, 60% of these tumors are seen in a context of tuberous sclerosis. Rhabdomyomas are the most common cardiac tumors of infants and children, the large majority occurring in patients younger than one year and are diagnosed more and more in foetal echocardiography.

[Primary cardiac lymphoma. Report of a case]. / Lymphome cardiaque primitif. A propos d'un cas.

Malignant non-Hodgkins lymphomas have a secondary cardiac localisation in 20% of cases. However, a cardiac primary site is rare (44 cases described up to now). A positive diagnosis is rarely made before death. There is great interest in echocardiography, a non-invasive method, to identify these tumours early. The prognosis remains nevertheless gloomy. We report the case of a child aged 8 years, admitted with a scenario of low output right cardiac insufficiency. Chest radiography identified cardiomegaly with a prominent right border, and the electrocardiograph showed right auricular hypertrophy. A tumour mass infiltrating the right atrium, the right ventricle and the lateral face of the left ventricle was discovered on trans-thoracic echocardiography. Investigation for tumour spread was negative. The patient died before operation in a state of extreme low output. The histology favoured a highly malignant non-Hodgkins lymphoma type B.

[Heart involvement in carcinoid syndrome: report of a case]. / Atteinte cardiaque au cours du syndrome carcinoïde: à propos d'un cas.

The authors report the case of tricuspid and pulmonary disease, revealing a carcinoid syndrome in a 32 years-old young man who was admitted for asthenia and an effort hepatology. The carcinoid syndrome was confirmed by hormonal proportioning, and by an anatomopathologic and immunohistochimic study of a hepatic metastatic biopsy. The check-up to search the primitive tumor was negative. The clinicals, paraclinicals and therapeutics aspects of the carcinoid heart were reported in this work.

[Giant aneurysm of the inter-atrial septum]. / Anévrisme géant du septum inter-auriculaire.

We report the case of a large atrial septal aneurysm and a review of the literature. Atrial septal aneurysm is found in 1-8% of normal subjects. Its prevalence is higher among patients with ischemic stroke. Transesophageal echocardiography is an optimal tool for the diagnosis of atrial septal aneurysm. The clinical course may be complicated by arterial embolism, but mechanical complications may also occur, as in this case. Due to the lack of general agreement, treatment options should be discussed on an individual basis for patients with atrial septal aneurysm.

[Pericardial mesothelioma. A case report]. / Mésothéliome péricardique. A propos d'un cas.

Primary pericardial mesothelioma is a rare malignancy, with an estimated incidence of 0.0022% in a large autopsy study. We report a case of primary pericardial mesothelioma revealed by a large and recurrent pericardial effusion. Through a literature review, we analyse the clinical findings of this tumor. With or without therapy, prognosis is poor.

[Right atrial hemangioma presenting as pericardial tamponade. A case report]. / Tamponnade péricardique révélant un hémangiome de l'oreillette droite. A propos d'un cas.

Intracardiac haemangioma is a very rare benign primary cardiac tumour. A 40-year-old patient, with no notable history, presented with chest pain, accentuated by deep inspiration, associated with NYHA stage III dyspnoea. Physical examination revealed signs of right heart failure and electrocardiogram showed low voltage with diffuse repolarization disorders. Chez x-ray showed a cardiothoracic index CTI > 0.70, and enlarged right margins. A large pericardial effusion with a tumour mass filling the right atrial cavity was discovered on transthoracic echocardiography. Chest computed tomography demonstrated the tissue origin of the tumour and suggested extension to the inferior vena cava and hepatic veins. The patient was referred to a surgeon for tumour resection. Histological examination revealed intracardiac capillary haemangioma. Five month postoperative follow-up did not reveal any abnormalities.

[Capillary hemangioma of the left ventricle. A case report and review of the literature]. / Hémangiome capillaire du ventricule gauche. A propos d'un cas et revue de la littérature.

The authors report a case of isolated left ventricular capillary haemangioma, diagnosed after chest X-ray during a routine medical check-up showed a deformation of the left heart border. Echocardiography showed a solid well circumscribed tumour. Its vascular origin was confirmed at coronary angiography. Myocardial biopsy performed after left thoracotomy confirmed the histological diagnosis. The authors review the medical literature of this condition in the light of this rare finding.

[Spontaneous cardiac rhythm in patients wearing cardiac stimulators]. / Evolution du rythme cardiaque spontané chez des malades porteurs de stimulateurs cardiaques.

Cutaneous electrical stimulation allows inhibition of permanent on-demand cardiac pacemakers. The evolution of the spontaneous cardiac rhythm was studied, using this technique of inhibition in 164 patients paced for 4 to 16 years. At implantation, 117 patients (70 p. 100) had atrioventricular block, 45 patients (27 p. 100) had sinoatrial dysfunction and 2 patients had a carotid sinus syndrome. Of these 164 patients, 153 were symptomatic. Three groups were distinguished: - 41 patients (25 p. 100) with a spontaneous ventricular rhythm at each check-up; - 75 patients (46 p. 100) with an underlying ventricular rhythm recorded when the pacemaker was inhibited by cutaneous electrical stimulation; - 48 patients (29 p. 100) with no underlying ventricular rhythm during 3 successive inhibitions of their pacemakers of 3 to 4 seconds duration on each occasion. All these patients had atrioventricular block at implantation; none had sinoatrial disease. In conclusion, disease of the atrioventricular conduction pathway, contrary to sinoatrial disease, leads to a prolonged absence of the spontaneous ventricular activity. This seems to be related to a longer survival of patients without associated pathology, and also suggests a degenerative cause of the atrioventricular block.

Mitral valve replacement in children. Comparative study of pre- and postoperative haemodynamics and left ventricular function.

Haemodynamic variables and left ventricular function were studied before and after mitral valve replacement in 44 children age 3 to 17 years (mean 11.9 years). Thirty-nine Starr-Edwards prostheses and five Hancock prostheses were used; postoperative study took place two to six months (mean 3.9 months) after operation. Pulmonary hypertension was present preoperatively in most patients, with mean pulmonary artery pressures of 18 to 75 (mean 46.5 mmHg). Postoperatively there was a pronounced drop in pressure to a mean value of 25.6 mmHg, partially explained by a decrease in pulmonary capillary wedge pressure. Pulmonary arteriolar resistance, however, also decreased conspicuously from an average of 590 dynes s cm-5 m-2 preoperatively to 282 dynes s cm-5 m-2 postoperatively. A return to normal resistance was seen in every case when preoperative resistance did not exceed 650 dynes s cm-5 m-2; above this threshold some degree of pulmonary hypertension often persisted. The residual gradient across the prosthetic valve was slightly higher for the Hancock than for the Starr-Edwards prosthesis (mean 8.7 mmHg, vs mean 6.9 mmHg). The left ventricular end-diastolic volume was much increased before surgery, with a mean value of 190 ml/m2; it decreased conspicuously after operation to 103 ml/m2. The left ventricular ejection fraction ranged from 40% to 76% (mean 57%) before operation; there was no significant change after operation, with values ranging from 40% to 73%. This left ventricular dysfunction is probably the result of myocardial injury caused by a chronic volume overload and the sequelae of rheumatic carditis.