Three Cases of Intracardiac Leiomyomatosis with Very Long-term Follow-up

Uterine intravascular leiomyomatosis (IVL) with extension into the right heart is uncommon, with no more than 400 cases reported in the literature since 1907. The present study aims to report three patients with intracardiac IVL surgically treated in our institution, with long-term follow-up. Three female patients in their third to fifth decades of life, with a history of difficult hysterectomy due to extensive myomatosis, presented with symptoms of right-sided heart failure. Echocardiography and computed tomography were performed, where IVL extending from the pelvis into the right heart was observed. All three patients underwent a one-stage operation under extracorporeal circulation through a right auriculotomy and inferior vena cavotomy, accessed via a sterno-laparotomy. The tumors were extirpated without complications, with ligation of the vena cava or iliac vein. The patients at 10-, 13-, and 37-year follow-up were well and alive with mild lower extremities symptoms.