Abstract
Intravascular leiomyomatosis (IVL) is a rare benign smooth muscle tumor that can extend from the uterine or pelvic veins into the inferior vena cava (IVC) and, in exceptional cases, the right cardiac chambers. Despite its benign histology, it may present with life-threatening cardiovascular complications and often mimics thromboembolic disease, delaying diagnosis. We report on a 47-year-old woman with a history of uterine fibroids who presented with recurrent syncope. Imaging revealed an intraluminal IVC mass extending through the tricuspid valve into the right ventricle, initially presumed to be thrombus. A prior percutaneous suction thrombectomy was nondiagnostic as no tissue could be aspirated from the mass. To establish tissue diagnosis and to potentially relieve obstruction, the mass was approached using a robotic minimally invasive approach from the right chest. Complete intracardiac resection down to the atriocaval junction was achieved, and pathology confirmed intravascular leiomyomatosis. Recovery was uneventful, and subsequent staged abdominal surgery achieved complete tumor removal. At 6 months, she underwent minimally invasive lobectomy for an additional pulmonary lesion, also consistent with IVL, and remains disease-free on follow-up. In conclusion, intravascular leiomyomatosis should be considered in women with intracardiac masses and a history of uterine leiomyomas. A robotically-assisted minimally invasive strategy offers a feasible and less morbid alternative to traditional open approaches for diagnosis and intracardiac tumor resection.
Graphical abstract
Intraoperative findings: a tumor mass with firm texture was found in the right atrium
Intravascular leiomyomatosis is a rare benign smooth muscle tumor originating from the uterine venous system or a uterine leiomyoma. Despite its benign histology, the tumor may extend intravascularly into the inferior vena cava (IVC) and, in rare cases, into right-sided cardiac chambers. , Symptoms are often nonspecific and may resemble thromboembolic disease, complicating diagnosis. We present a patient with intravascular leiomyomatosis extending from the IVC to the right atrium and ventricle, managed with a minimally invasive, robotically-assisted intracardiac resection for diagnosis and partial excision.
Case Presentation
A 47-year-old female with uterine fibroids presented after multiple unexplained syncopal episodes. Contrast-enhanced CT revealed an intraluminal IVC mass traversing the tricuspid valve into the right ventricle, initially suspected to be a thrombus ( Figure 1 ). She had no history of thrombosis or hypercoagulability, though she had recently traveled long distances and used oral contraceptives briefly. A prior percutaneous suction thrombectomy retrieved no tissue for analysis. Given unclear diagnosis and the extent of cardiac involvement, a minimally invasive robotic procedure was planned to obtain diagnostic tissue and to attempt resection.
Preoperative imaging.
Operative Technique
right mini-thoracotomy was created in the fourth intercostal space, robotic ports were positioned 2 interspaces laterally, plus a parasternal port in the fifth intercostal space for atrial retraction. In alignment with our standard right-sided robotic cannulation strategy, bicaval cannulation was performed using a percutaneous right internal jugular venous cannula for the superior vena cava and a femoral venous cannula for the inferior vena cava. Both cavae were subsequently snared to ensure unobstructed venous drainage and a blood-free operative field. Despite extensive tumor burden in the abdominal IVC, venous return remained adequate intraoperatively—likely because venous flow was able to pass around the firm, compact, and nonfriable tumor mass. Cardioplegia was administered through a dedicated ascending aortic cardioplegia cannula, secured with a pledgeted 3-0 Prolene suture and tourniquet; using a Y-connector, the line was connected to both the cardioplegia and vent systems.
After right atriotomy, a firm grey-white intraluminal mass was visualized extending from the IVC ( Figure 2 ). Robotic instruments enabled complete intracardiac resection down to the atrio-caval junction, relieving obstruction and yielding tissue for diagnosis. Several 3/0 Prolene sutures were placed into the tumor for mobilization. The atriotomy was closed, and separation from the bypass was uncomplicated. (Video)
