Cardiac cavernous haemangioma.

BACKGROUND: Cardiac haemangioma was first described in 1893. It is an infrequent and benign neoplasm which accounts for about 2.8% of all benign primary cardiac tumours, arising from the cardiac ventricles, valves, atria and rarely the epicardium. METHODS AND RESULTS: We report the case of a 24-year-old woman with a cardiac haemangioma detected by transthoracic echocardiography, computed tomography, and magnetic resonance imaging. The coronary CT showed a mass located in the pericardial cavity with close relationship to the myocardium. The mass surrounded completely the segments 2 and 3 of the left anterior descending coronary artery. At operation, the tumour was incompletely resected, leaving a remnant in the left anterior descending coronary artery contact, one diagonal branch has been sacrificed. Pathologic study diagnosed a cavernous haemangioma. Transthoracic echocardiography eight months later showed a remnant of haemangioma with a left ventricular ejection fraction estimated to 69%. The coronary CT 10 months after surgery showed the remnant of the tumour with normal cardiac cavities and absence of visualisation of the diagonal artery. The patient is currently asymptomatic and doing well 14 months after surgery. CONCLUSIONS: Cardiac haemangioma is a rare cardiac tumour with an unknown aetiology. The diagnosis is aided by imaging techniques. The coronary CT may be useful when more precise evaluation of the tumour extent is required. It allows specifying the relationship of the mass with the coronary vessels. Successful treatment usually requires timely surgery. However, periodic examinations and echocardiography are recommended.

[Hypothyroid dilated cardiomyopathy]. / Myocardiopathie dilatée d'origine myxoedémateuse.

Most patients with dilated cardiomyopathy have a poor prognosis due to progressive and irreversible myocardial dysfunction. Rarely, is a metabolic etiology responsive to specific therapy identified. Although many studies have confirmed that thyroid hormone deficiency is associated with a reversible decrease in myocardial contractility, it has remained controversial whether hypothyroidism alone can cause a dilated cardiomyopathy and clinical heart failure. In this study, we report the case of a patient with severe hypothyroidism and a dilated cardiomyopathy complicated by heart failure, which has receded after recovery to euthyroidism with L-thyroxin therapy. This case suggests that hypothyroidism should be evoked systematically when a dilated cardiomyopathy is diagnosed.

[Distribution of cardiovascular risk factors in a Tunisian cohort of 6901 coronary patients]. / Distribution des facteurs de risque cardiovasculaire chez une cohorte tunisienne de 6901 coronariens.

The Tunisian epidemiological data on cardiovascular disease in the hospital environment are scarce. The aim of this study was to evaluate the frequency of cardiovascular risk factors and their association in patients hospitalised for coronary disease in coronary care units at Rabta, Charles Nicolle, Habib Thameur and Military hospitals, Tunis, over the period 1994-1998. The clinical features of 6901 patients (75.7% men, 3760 myocardial infarction, 3141 unstable angina) on hospital admission were analysed. The prevalence of smoking, dyslipidemia, hypertension, diabetes and obesity was 86; 49.8; 33.9; 40.7 and 15.2% respectively in the men and 12.9; 52.4; 64.6; 53.4 and 29.8% respectively in women. With this risk factor profile Tunisia has to implement a national strategy of primary prevention and heart health promotion in addition to the efforts recently made in secondary prevention of some chronic disease such as hypertension, diabetes and smoking.

[The first Tunisian cardiovascular diseases register: processes and results]. / Le registre des maladies coronaires en Tunisie: organisation et premiers résultats.

BACKGROUND: In Tunisia, cardiovascular diseases are the leading causes of death (30%) and a few studies conducted in the population have demonstrated that the level of their risk factors is increasing. For policy makers, the health system impact of these diseases is currently a crucial issue. The National Public Health Institute has identified the implementation of a morbidity register as a priority. METHODS: A CVD morbidity register is implemented since 2001, in 3 different geographical populations having contrasted levels of health status (Tunis, Ariana and Ben Arous). The 3 regions are covering about 2 millions inhabitants which is the fifth of the overall Tunisian population. All coronary heart events occurring among adults 25 years old and above in the 3 populations are recorded. The diagnosis of events, case fatality and classification are defined according to MONICA criteria. The data are recorded from public and private hospitals, death certificates and autopsies. RESULTS: During the year 2001, the total number of myocardial infarction events was estimated at 942: in men, the age-standardized rates were 163.8/100000 in Tunis population vs. 161.9 in Ariana and 170.5 in Ben Arous. In women, the rates were respectively 43.4, 61.1 and 44.6. Medical causes of death registration was the most crucial problem in spite of the implementation of the death certificate designed according to WHO model. Specific surveys for clinical assessment and surveillance of risk factors were conducted in the register populations. CONCLUSION: It is the first time that data on coronary heart disease incidence and fatality are available in Tunisia through this experience which highlights the practical difficulties experienced in registering and coding coronary events in a developing country. The data source quality should be improved and the register should be integrated in the local health system.

[Atrial septum aneurysm. Report of 20 cases]. / L'anevrysme du septum interauriculaire. A propos de 20 cas.

Atrial septum aneurysm (ASA) is an uncommon congenital malformation, which is more frequently diagnosed since the introduction of transoesophageal echocardiography. ASA is often involved in the etiology of cerebral and systemic embolic events. The purpose of our study is to determine from our experience the frequency, the diagnostic aspect and therapeutic modalities of ASA. We report in this study 20 cases of ASA collected from the echocardiographic register. The prevalence of this malformation is 0.08%, with a high male predominance. The mean age was 51 years. ASA was detected after brain embolism in 55% of cases. The diagnosis was established in all cases by transesophageal echocardiography, however only 5 cases were diagnosed by Transthoracic echocardiography. The patients showed favorable evolutions after a of follow up of three years.

A novel mutation in the SLC19A2 gene in a Tunisian family with thiamine-responsive megaloblastic anaemia, diabetes and deafness syndrome.

Thiamine-responsive megaloblastic anaemia (TRMA) syndrome with diabetes and deafness was found in two patients from a Tunisian kindred. The proband was homozygous for a novel mutation, 287delG, in the high-affinity thiamine transporter gene, SLC19A2. We demonstrated that fibroblasts from this patient exhibited defective thiamine transport. These data confirm that the SLC19A2 gene is the high-affinity thiamine carrier and that this novel mutation is responsible for TRMA syndrome.

[Cardiac sarcoidosis revealed by ventricular tachycardia]. / Sarcoïdose cardiaque révélée par une tachycardie ventriculaire: a propos d'une observation.

Cardiac sarcoidosis is a multisystem granulomatous disease of unknown etiology, that is more seen in younger adults. The prognosis of myocardial sarcoidosis is poor and depends on arrhythmia, conduction disorders. We report the case of 35 years old woman with cardiac sarcoidosis and pulmonary involvement who presented acutely with sustained ventricular tachycardia well tolerated. She made a good recovery after steroid therapy and administration of antiarrhythmic agent.

[Congenital coronary aneurysm. Report of two cases]. / Anevrysmes coronaires congenitaux. A propos de deux observations.

Congenital coronary aneurysms are an unusual anatomical entity. Their prognosis appears to be particularly dependent on the presence or absence of aneurysm thrombosis. We report two cases of congenital coronary aneurysms, diagnosed in one case after myocardial infarction. The two patients were treated successfully by an exclusion of the aneurysm and coronary bypass. The aim of this study is to discuss the clinical features, prognosis and management of this disease.

[Spontaneous contrast of the left atrium in mitral valve stenosis]. / Le contraste spontané de l'oreillette gauche dans le retrecissement mitral.

The left atrial spontaneous contrast is uncommon finding during transthoracic échocardiography. Transesophageal echocardiography provides superior imaging of the left atrium and left spontaneous contrast has been detected more frequently by this technique in rheumatic mitral stenosis. In order to analyze the significance of spontaneous contrast, we have studied 100 patients with mitral stenosis. Trans thoracic and transesophageal echocardiography were performed in all patients. Left atrial spontaneous echo contrast was detected in 60 patients (group A) and was absent in 40 patients (group B) The mean of mitral valvular area was 0.9 10.2 mm in group A and 48 9.5 mm in group B (p < 0.001). Ther was a high incidence of atrial fibrillation in group A; 63% VS 12% in group B (p < 0.001). A systemic embolization was noted in 20% of patients in group A; No one of group B has presented this complication (p < 0.001).

[Level of cardiovascular risk factors in the urban and rural populations of Cap-Bon: Tunisia]. / Niveau des facteurs de risque cardio-vasculaire dans la population urbaine et rurale du Cap-Bon: Tunisie.

Cardiovascular risk factors were studied in a random sample from the urban and rural population of the Cap-Bon, including 692 men and women, aged 35-50 years. In this sample, including 56 % urban dwellers, the mean total cholesterol was higher in urban areas (4.64 mmol/l) than in rural areas (4.16 mmol/l). In urban areas 10.2 % of the subjects had values above 6.2 mmol/l compared with 6.6 % in rural areas. Obesity was much more frequent among women (35.2 % versus 26.3 %) but less frequent among men (16.0 % versus 5.4 %) in urban and rural areas. Diabetes was found in 8.8 % of men and 10.4 % of women in urban areas, and only 0.8 % and 3.9 % in rural areas. Hypertension was found in 13.8 % of women and 11.6 % of men in urban areas, 13.3 % and 4.0 % in rural areas (13.3 %). Cigarette smoking was found in 60.7 % of men in urban areas and 75.8 % in rural areas (p < 0.05).

[Malignant non-Hodgkin lymphoma of cardiac localization. Apropos of a case]. / Lymphome malin non hodgkinien à localisation cardiaque. A propos d'une observation.

Malignant non-Hodgkin's lymphomas are rare in the absence of human immunodeficiency virus infection and it is exceptional for a cardiac site to be the prominent feature. In our case, the malignant lymphoma was revealed by pericardial effusion in a context of alteration of the general state. Echocardiography revealed a heterogeneous mass in the right atrium and an abundant circumferential pericardial effusion. Thoracic computed tomography allowed local staging and magnetic resonance imaging (MRI) allowed a better definition, than CT scan, of the extension of the tumour into the various cardiac structures. The histological diagnosis was established on biopsy of a mediastinal lymph node. The patient died 7 months after the diagnosis, despite chemotherapy. The authors emphasize the contribution of echocardiography in the diagnosis of cardiac tumours, computed tomography in local staging, and MRI in the analysis of the various cardiac structures.