The authors report the case of a young 26 years old man admitted for abundant fluid pericarditis which was rapidly recurrent, requiring two perocardial punctures and a surgical drainage. The two dimensional echocardiogram demonstrated a large right atrio-ventricular tumour with infiltration of the right ventricular wall, suggesting its malignant nature. After confirmation by right angiography and in view of the rapid cardiovascular deterioration, it was decided to operate. The surgery was only exploratory; not even palliative surgery was possible for this tumour mass invading the right atrium, the right ventricle and the pericardium. Histology revealed it to be an angiosarcoma.

Adult↗

PubMedDate not supplied

[Pulmonary arterial hypertension and generalized scleroderma. A propos of 10 cases].

Whereas pulmonary fibrosis is a very frequent manifestation of scleroderma, pulmonary hypertension of the precapillary type is a much rarer occurrence in this affection. Two cases of scleroderma with subsequent development of a pulmonary hypertension are reported. Various hypotheses have been advanced to explain the origin of this disorder during the course of scleroderma. Rather than a direct consequence of the sclerodermic pulmonary fibrosis it probably arises secondary to the specific, diffuse, primary pulmonary vascular lesions.

Aged↗

PubMedDate not supplied

[Multiple coronary-left ventricular fistulae. Apropos of a case].

The authors report the case of a 57 years old man who was admitted for angina unresponsive to medical treatment. Coronary angiography revealed significant atherosclerotic lesions in the right coronary artery and also revealed multiple left coronaro-ventricular fistulae, hence the interest of this case. The fistulae were filiform and originated in the circumflex and posterior interventricular arteries. Coronaro-cardiac fistulae are rare, but this type of left-sided coronaro-ventricular fistula is exceptional. The patient was asymptomatic following surgical correction of the atheromatous lesions of the right coronary artery.

Coronary Angiography↗

PubMedDate not supplied

[Behçet's disease: a rare cause of recurrent thrombophlebitis].

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Adolescent↗

PubMedDate not supplied

[Value of cardiac scintigraphy with I123 meta-iodobenzylguanidine in congestive heart failure. A review].

Heart failure is accompanied by major disturbances of the functioning of the sympathetic nervous system: global overactivation and local modifications of the adrenergic system. 123I-MIBG cardiac scintigraphy is an isotope technique investigating presynaptic adrenergic function. The cardiac uptake of MIBG is decreased during heart failure, reflecting a reduction of norepinephrine reuptake by cardiac presynaptic nerve endings. Alteration of presynaptic function occurs early and plays an important role in the pathogenesis of the deterioration of heart failure. 123I-MIBG cardiac scintigraphy allows in vivo assessment of the myocardial adrenergic reserves of patients with congestive heart failure. It should be proposed in all patients with severe ventricular dysfunction to help define the indications for heart transplantation.

3-Iodobenzylguanidine↗

PubMedDate not supplied

[Leiomyosarcoma of the heart and great vessels].

Leiomyosarcomas (LMS) of the heart are exceptional primary malignant tumours with a catastrophic prognosis and a mean survival measured in months. Extensive radical surgical resection clearly remains the most appropriate treatment. We report three cases observed over a 3-year period, consisting of an LMS of the inferior vena cava, an LMS of the pulmonary artery trunk and an LMS of the left atrium. The first case was treated by radical resection and reconstruction by autologous vein graft of the cavorenal junction, the second case was treated by extensive resection and prosthetic reconstruction of the pulmonary artery bifurcation and the third case was treated by a first radical resection of the left atrium, requiring total cardiectomy and orthotopic heart transplantation for local recurrence at the sixth month. The survical was significantly improved compared to other treatment options (chemotherapy, radiotherapy). The first patient is still alive without recurrence at two years; the second died 12.5 months after the surgical procedure and the medium-term follow-up of the transplanted patient revealed cerebral and hepatic metastases nine months after transplantation. The authors review the literature concerning these extremely rare malignant tumours. Recent progress of diagnostic investigations, such as spiral CT with reconstruction, MRI, positron emission tomography (PET), are now able to establish the diagnosis more rapidly and therefore allow more radical surgical resection. This resection, possibly combined with venous reconstruction, must be associated with adjuvant therapies. Heart transplantation should be considered among the treatment options for leiomyosarcomas of the heart, in order to improve the poor prognosis of these lesions affections a young population.

Brain Neoplasms↗