Intravenous leiomyomatosis involved cor dextrum: A review of diagnosis and management with an illustrative case

Abstract

Intravenous leiomyomatosis (IVL) is a benign disease that originates in the uterus and is characterized by aggressive intravenous growth that can extend to the inferior vena cava and even involve the intracardiac cavity. This extension of the IVL into the right heart is known as intracardiac leiomyomatosis (ICL). The clinical incidence of this disease is extremely low, and the characteristics of atypical aggressive growth lead to diagnostic dilemmas. Thus, studies on this disease have profound clinical significance. Here, we report a case of intracardiac leiomyomatosis and review the relevant literature. A 46-year-old female patient presented with exertive chest tightness and shortness of breath with an onset of approximately half a month. The patient underwent uterine myomectomy in 2018 and recovered well after surgery. On preoperative echocardiography, a hypoechoic mass was detected in the right atrium, which was diagnosed as a leiomyoma. Cardiac magnetic resonance imaging (MRI) revealed a T2-weighted mixed high-signal mass with partial cystic changes that extended from the inferior vena cava into the right atrioventricular cavity, occupying most of the atrial cavity and was misdiagnosed as an atrial mucinous tumor. The patient underwent cardiac tumor resection, tricuspid valvuloplasty, and lower-extremity vascular exploration under general anesthesia with extracorporeal circulation. The final pathological diagnosis was ICL. This is an illustrative analysis of various aspects of the disease, including pathology, etiology, clinical symptoms, imaging diagnosis, and treatment modalities.

Keywords

• Case report
• Intracardiac leiomyomatosis (LCL)
• Intravenous leiomyomatosis (LVL)
• Uterine leiomyoma