P75 MULTIDISCIPLINARY MANAGEMENT OF INTRACARDIAC LEIOMYOMATOSIS: A CASE REPORT

Abstract

Abstract A 60–year–old woman with no cardiological history came to our attention for exertional dyspnea since few months. The electrocardiogram and chest radiograph were normal. Trans–thoracic echocardiogram revealed the presence of a floating parenchymatous formation measuring 12 x 2 cm starting from the inferior vena cava (IVC) and jutting into the right chambers, without obstruction to inflow. Chest CT and cardiac MRI described a nonvascularized inhomogeneous formation suggestive of thrombotic apposition in VCI, so the patient was treated with unfractionated heparin. Total body CT/PET scan excluded hypercaptant lesions. After two weeks of anticoagulant therapy, on echocardiogram the mass appeared unchanged in morphology and size. On reevaluation of the CT images, a probable origin of the formation at the level of the right ovarian vein was identified. In light of the positive history of bilateral hysteroannessiectomy for uterine myomas and the lack of response to anticoagulant treatment, the suspicion of intravascular and intracardiac leiomyomatosis was raised. After collegial discussion, given the high risk of an eventual thoraco–abdominal surgery, it was decided to remove only the portion of the mass adherent to the VCI jutting into the right heart chambers. The patient underwent cardiac surgery: a polypoid, capsular, hard–elastic mass, 12 x 2 cm in size, jutting into the right chambers and with the base of implantation located in the anterior wall of the VCI, was removed. The final histologic diagnosis was sclero–jalin leiomyoma. The surgery and postoperative course proceeded without complications. At follow–up at 20 months, the patient is asymptomatic and no mass is detectable on echocardiogram. Intracardiac leiomyomatosis is a rare condition characterized by benign smooth muscle tumors of extracardiac origin reaching the right heart by direct extension into the venous canal, described predominantly in women with a history of uterine myomas. Although histologically benign, intracardiac leiomyomatosis can result in sudden death if there is total obstruction to the outflow tract. In our case, the multimodality imaging approach and serial monitoring by echocardiography were essential to guide treatment choices and for the follow up.