Abstract
OBJECTIVE: Intravascular leiomyoma (IVL) is a rare benign tumor extending from the uterus into the surrounding venous structures and may extend into the heart, leading to occlusion of the tricuspid or pulmonary valve resulting in sudden cardiac death. KEY STEPS: We present a 56-year-old woman with an IVL extending into the right atrium and ventricle. We excised in a staged procedure, first removing the intracardiac and suprahepatic tumor component, followed by laparotomy, excision of the pelvic mass, bilateral oophorectomy, and caval thrombectomy 6 weeks later. POTENTIAL PITFALLS: IVL may extend into the heart necessitating total cardiopulmonary bypass for excision either in a single or multistage procedure. We recommend a multistage approach because the tumor is slowly growing and encapsulated. TAKE-HOME MESSAGE: Management requires careful planning with multiple surgical disciplines, cardiology, and radiology to determine the extent and plan for surgical excision.