Single‐stage operation using hypothermic circulatory arrest to remove uterine intravenous leiomyomatosis extended to the vena cava and right atrium

Abstract

Intravenous leiomyomatosis (IVL) is an uncommon condition characterized by an intraluminal growth of benign smooth muscle cells in venous vessels. While IVL confined within the inferior vena cava usually causes few symptoms-including pelvic pain and edema of the lower extremities-the tumor may extend to the heart, causing intermittent obstruction of the tricuspid valve or right ventricular obstruction outflow, and subsequently right-sided heart failure or death. The present article reports a case of IVL extending to the heart that was treated in a single-stage operation involving cardiopulmonary bypass and hypothermic circulatory arrest.A 43-year-old patient was referred to Careggi University Hospital complaining of dyspnea and intermittent episodes of tachycardia; the patient had a previous history of uterine leiomyoma excision. Echocardiogram confirmed the presence of a right atrium intracardiac mass. MRI revealed a uterine mass measuring 8 cm in diameter with

Multiservice single-stage surgery for intracardiac leiomyomatosis

“…Although histologically benign, the extension of the tumor through the blood network and the possibility of causing right heart failure and cardiac arrest gives it a technical metastatic characteristic. [2,3,5] The presentation of a woman with intravascular leiomyomatosis includes an average age of 47.6 (range 20-81 years) that is multiparous (75%), has a history of previous hysterectomy or myomectomy (53.6%), and has a coexisting uterine leiomyoma on admission (29.9%). Most common presentations include dyspnea (65%), syncope (47%), and lower extremity edena (46%) however, 13% of patients are asymptomatic at presentation.…”

“…[16] The one-stage operation, as employed in this case, involves performing the sternotomy and laparotomy simultaneously. Both stages are performed under cardiopulmonary bypass and typically with hypothermic circulatory arrest, [3] similar to the procedure described in this case. However, there have been few cases that report the avoidance of cardiac arrest.…”

“…[2] Tumor extending beyond the tricuspid valve and obstructing the right ventricular outflow tract has the potential to cause right heart failure and death. [3] The most common presentation includes dyspnea, syncope, and lower extremity edema. However, it may also be found incidentally in an asymptomatic patient.…”

“…[6] This condition can be treated via two-stage procedure with a sternotomy first for intracardiac tumor removal followed by a laparotomy for complete tumor excision, or via a single-stage operation with a sternotomy and laparotomy approach under cardiopulmonary bypass. [2][3][4][5] The following case report employed a single-staged surgery with the involvement of Cardiothoracic, GynecologyOncology, Urological, and Vascular Surgery services.…”