The authors report a case of malignant cardiac fibrohistiocytoma involving the right ventricular infundibulum and the pulmonary artery and responsible for a pseudo-thromboembolic right heart failure syndrome. Following discovery of the tumour mass on ultrasonography and catheterisation, wide excision of the pulmonary artery, pulmonary valve and the roof of the infundibulum as far as the middle part of the ventricle was performed under cardio-pulmonary by-pass. Reconstruction was performed with a valveless woven Dacron tube. The postoperative course was uncomplicated and the patient was still alive after 18 months. Histology revealed malignant fibrohistiocytoma. The authors also present a review of the literature of these rare tumours of the right-sided cavities (5 cases reported).

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