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The authors wish to describe a combined open and endovascular approach to repair a complex thoracic aortic aneurysm. A 72-year-old man with chronic obstructive pulmonary disease, aortic valvular insufficiency and diffuse thoracic aortic aneurysm underwent aortic valve and ascending aorta replacement by a Bentall-procedure and replacement of arch aneurysm using the elephant trunk technique, performed in a first procedure. During the second procedure, endovascular stenting of the descending thoracic aorta was done. Only a few similar case reports have been presented. Endovascular repair after an elephant trunk procedure for complex thoracic aortic aneurysms is an elegant approach to deal with such mega aortas. Further research is necessary to compare open and endovascular repair and to determine long-term follow-up with regard to endoleaks and mortality.

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SUMMARYPrimary leiomyosarcomas of the heart, particularly those affecting the right ventricle, are uncommon. We report the case of a 70-year-old Belgian woman presenting with the symptoms of progressive exertional dyspnea and left-sided pleuritic pain. A leiomyosarcoma which originated from the right lateral ventricle wall, causing pulmonary outflow obstruction. was diagnosed. Pathology revealed a neoplasm with a myxoid stroma, high mitotic activity and nuclei expressing atypia. Immunohistochemical staining was positive for vimentine and desmin. Seven months after complete surgical resection the tumor relapsed.This case demonstrates the poor outcome, the high relapse rate and inefficiency of treatment associated with primary cardiac leiomyosarcomas. The current literature regarding the incidence, diagnostic techniques, treatment strategies and survival rates of this rare but terminal disease is reviewed. (Jpn Heart J 2001; 42: 377-386)