Introduction: Intravenous leiomyomatosis (IVL) is a rare endovascular disease, which is histologically benign but can exhibit quasi-malignant behaviour. Intracardiac IVL is even rarer, with approximately 200 cases reported worldwide. It can present asymptomatically or with cardiovascular instability and even death. Traditionally, a two-stage surgery has been performed, where the cardiac component of the IVL would be resected first to prevent cardiac complications prior to the pelvic and abdominal component. However, tumour embolisation risk remains and a second operation is required. Hence, a one-stage approach has been proposed. There is little published data on the ideal surgical approach with surgical steps rarely detailed.
Methods: We present the step-by-step surgical management of two cases of intracardiac IVL, which were managed with a one-stage multidisciplinary surgical approach.
Results: Case A is a 54-year-old female whose intracardiac IVL extended from the uterus via the uterine veins and inferior vena cava (IVC) into the right atrium.
Case B is a 51-year-old female whose intracardiac IVL extended from the uterus via the ovarian and uterine veins into the right atrium and right ventricle with adherence to the tricuspid valve.
Both patients were treated with a multi-disciplinary Gynaecological Oncology, Cardiac and Vascular surgical team. A combined sternotomy-laparotomy, cardiopulmonary bypass without cardioplegia, intraoperative cooling, total abdominal hysterectomy, bilateral salpingo-oophorectomy and resection of tumour from the IVC and heart were performed. We detail the surgical decision-making and steps taken in each case.
Conclusions: Intracardiac IVL can be successfully treated in a one-stage procedure with a multidisciplinary team.