Echocardiographic Characteristics of Intravenous Leiomyomatosis with Intracardiac Extension: A Single-Institution Experience

Background: Intravenous leiomyomatosis (IVL) is a rare smooth-muscle proliferation arising from a uterine myoma and occasionally extending into cardiac chambers. Methods and Results: A series of 10 consecutive patients with histologically and surgically proven intracardiac IVL between 2000 and 2010 in our hospital were reviewed. The echocardiographic features of 10 cases with IVL and extensive spread into the right-sided cardiac chambers were described for the first time. All patients were female and the mean age was 42 +/- 7 years old. The first symptoms of six patients (60%) were exertional dyspnea and palpitation of cardiac origin. Echocardiography showed that all the tumors originated from the inferior vena cava (IVC) and located in cardiac right chambers (70% in right atrium alone, 30% in right ventricle and atrium). Eight masses (80%) were oval, whereas the others (20%) were serpentine, all with well-demarcated borders and most (70%) with heteroechogenic texture. Five tumors (50%) intermittently prolapsed into right ventricle through the tricuspid valve. Two patients with nodules adhering to the top of the tumors had pulmonary tumorous thromboembolism. Conclusion: Echocardiography is a simple and important technique to diagnose IVL with intracardiac extension. This disease should be considered in a female patient presenting with an extensive mass from IVC with well-demarcated border in the right-sided cardiac chambers. (Echocardiography 2011;28:934-940)