Symptomatic Tricuspid Valve Obstruction Due to Intravenous Leiomyomatosis.

Regarding her history of prior RA mass, the patient presented to another institution 2 years prior with dizziness and syncope. She was found to have a RA mass (measuring 1.3 × 0.5 × 1.7 cm) on TTE, which was surgically resected. Computed tomography (CT) of the chest did not reveal other acute pathology. Immunohistochemical stains were compatible with a morphologic diagnosis of hyalinized thrombus, thought to be due to intravascular endothelial proliferation or hyperplasia, consistent with the diagnosis of Masson’s tumor/hemangioma. She had no known gynecologic history of fibroids, cysts, endometriosis, or abnormal pap smears. No previous abdominal or pelvic imaging was available in the medical record.

A 48-year-old female patient with a history of right atrial (RA) Masson’s tumor/hemangioma (resected 2 years prior) presented to our hospital for definitive management of a new RA mass (measuring 2.4 cm × 3.1 cm with a stalk attached to the interatrial septum) crossing the tricuspid annulus causing mild-to-moderate tricuspid regurgitation (TR). Take-Home Messages • In patients with intracardiac mass, maintaining preload to minimize valvular obstruction when bridging them to surgical resection is imperative. • In patients presenting with heart failure symptoms and an intracardiac mass, chest and abdominal imaging may be necessary to identify the source of the mass and ensure appropriate surgical planning. • In patients with intracardiac mass, maintaining preload to minimize valvular obstruction when bridging them to surgical resection is imperative. • In patients presenting with heart failure symptoms and an intracardiac mass, chest and abdominal imaging may be necessary to identify the source of the mass and ensure appropriate surgical planning. In patients with intracardiac mass, maintaining preload to minimize valvular obstruction when bridging them to surgical resection is imperative. In patients presenting with heart failure symptoms and an intracardiac mass, chest and abdominal imaging may be necessary to identify the source of the mass and ensure appropriate surgical planning. Approximately 6 months after her prior RA mass resection, the patient gradually developed chest discomfort, orthopnea, and dyspnea on exertion. Her symptoms worsened over the following year-and-a-half, and she was eventually seen by a cardiologist, who ordered a transthoracic echocardiogram (TTE). The TTE revealed the presence of a RA mass (measuring 2.4 cm × 3.1 cm with a stalk attached to the interatrial septum) crossing the tricuspid annulus causing mild-to-moderate TR. Subsequent magnetic resonance imaging of the chest and abdomen revealed an intracardiac mass extending from the interatrial septum into the inferior vena cava (IVC) and the right common iliac vein. Given these findings, the patient was referred to our center for management of heart failure symptoms, evaluation of the contiguous mass, and multidisciplinary surgical resection.

The patient had a relatively unremarkable postoperative course. Other than 1 week of self-resolving hematuria requiring a total of 3 blood transfusions and a nonocclusive deep vein thrombosis in the left internal jugular vein discovered on postoperative day 7 that was treated with anticoagulation, the patient recovered well. A postoperative TTE revealed resolution of TR. The patient was discharged on postoperative day 11 on apixaban. The patient has continued to follow up at our institution. The patient reports a complete resolution of symptoms 2 months after resection.

IVL is a rare benign neoplasm that is typically associated with premenopausal women with a history of uterine fibroids. 1 In a case analysis of 194 patients found to have IVL, dyspnea, syncope, and lower extremity edema were the most common presenting symptoms. 1 Of these, approximately 40% of the patients had involvement in the RA. 1 In the case of our patient, she presented with chest pain and dyspnea on exertion. Although she did not have a known history of fibroids, she was found to have multiple fibroids on imaging. Given the size of the uterine mass with extensive intravenous extension, the IVL likely originated from the uterine smooth muscle 2 and less likely from the smooth muscle of the vascular wall. 3 If IVL with cardiac extension is left untreated, syncope, dyspnea, right-sided heart failure, pulmonary embolism, and sudden death can occur. 1 Although definitive management of IVL is surgical resection, 4 the preoperative management while bridging these patients to surgery is less clear. Although management of symptomatic left ventricular outflow obstruction in cases of hypertrophic cardiomyopathy is well established, 5 the management of heart failure symptoms due to intracardiac obstruction from tumors or masses is less defined. In the case of our patient, her symptoms of chest pain and dyspnea on exertion were likely due to TV obstruction compromising stroke volume. Given the concern for TV obstruction, preload was maintained to minimize the risk of obstruction and subsequent hemodynamic collapse. In the case of hypertrophic cardiomyopathy, beta-blockers and calcium channel blockers can be effective in increasing preload to relieve left ventricular outflow tract obstruction. However, in the case of IVL, the risks of initiating such medications before surgical resection may not outweigh the benefits of increasing preload, especially in a patient who is otherwise hemodynamically stable. In the case of this patient, fluid boluses were administered as needed to ensure daily net positive fluid status to maintain preload. Given the patient’s past medical history, there was some consideration that the patient’s RA mass was due to recurrent Masson’s tumor/hemangioma. Masson’s tumor/hemangioma is exceedingly rare and can affect any part of the body, including the cervix. 6 However, pathology demonstrated a proliferation of smooth muscle cells and closely packed muscular veins embedded in a myxoid to fibrous stroma, a significant contrast to the almost entirely hyalinized lesion seen 2 years prior. This suggests that the 2 tumors originated from distinct disease processes. There is no known association between Masson’s tumor/hemangioma and IVL. Importantly, this is an exceedingly rare case of a patient with a history of 2 distinct benign cardiac tumors requiring surgical resection. As IVLs typically involve the right side of the heart, heart failure symptoms are frequently the presenting symptom of these masses. Obtaining cross-sectional imaging, including of the abdomen and pelvis, to identify the source of the mass, and surgical intervention are imperative in preventing hemodynamic collapse and possible mortality in these cases. Furthermore, follow-up is of utmost importance because IVL has a high rate of recurrence after the operation, with the involvement of the iliac veins and IVC as risk factors for recurrence. 7 Given the rarity of IVL, there are no established guidelines regarding monitoring for recurrence. In our patient with risk factors for recurrence (eg, iliac vein involvement), pelvic ultrasonography every 3-6 months within 2 years after surgery and once a year thereafter for 8 years, with CT and TTE as needed based on physical examination and abnormal ultrasound findings, would be reasonable. 7

The cardiology team was consulted by the primary medical team to assist with preoperative management. Given the concern for TV obstruction, preload was carefully maintained to minimize the risk of obstruction. Fluid boluses were administered as needed to ensure daily net positive fluid status. The patient was maintained on telemetry to observe for arrhythmia. Multiple surgical teams were consulted, and, ultimately, the patient underwent multidisciplinary (cardiothoracic, gynecology, urology, liver, and vascular) resection of the RA mass ( Figure 4 ), including removal of the extending tumor, total hysterectomy, and bilateral salpingo-oophorectomy. There were no operative complications, and circulatory arrest during the operation was not required. Pathology revealed proliferation of smooth muscle cells and closely packed muscular veins embedded in a myxoid to fibrous stroma. Immunohistochemical staining revealed diffusely positive estrogen receptor, progesterone receptor, smooth muscle actin, and desmin, and negative HMB45, WT1, CD10, and CD34. Overall, these findings were consistent with the diagnosis of IVL. Figure 4 Excised Pedunculated Mass Excised pedunculated mass affixed to the interatrial septum (red arrow) that extended into the IVC (yellow arrow). Abbreviations as in Figure 2 . Excised Pedunculated Mass Excised pedunculated mass affixed to the interatrial septum (red arrow) that extended into the IVC (yellow arrow). Abbreviations as in Figure 2 .

IVL is a benign neoplasm that can extend into cardiac chambers, causing TV obstruction, TR, and heart failure symptoms. IVL should be on the differential for premenopausal women who present with heart failure symptoms and are found to have an intracardiac mass. Cross-sectional imaging (ie, chest, abdomen, and pelvis) is necessary for evaluation. Maintaining preload is imperative in bridging these patients to surgical resection. Visual Summary Timeline of the Case Timeline Events Day 1 Presented to our institution for dyspnea on exertion. Approximately 2 weeks prior, presented to an outside institution where TTE revealed a RA mass and magnetic resonance imaging of the chest and abdomen revealed an intracardiac mass extending from the interatrial septum into the IVC and the right common iliac vein. Day 2 TTE revealed RA mass, preserved left ventricular ejection fraction, and moderate TR. The mass was measured as 5.4 × 2.9 cm. The mean gradient across the tricuspid valve was 16.6 mm Hg. The pulmonary artery systolic pressure was estimated to be 34 mm Hg plus the RA pressure. CT venography with intravenous contrast of the chest and abdomen demonstrated multiple intramural uterine fibroids and a large exophytic mass arising from the right uterine body (measuring 15.2 × 6.6 × 17.5 cm). The mass was endophytic into the right internal iliac vein and extended into the right common iliac vein, IVC, and RA. Days 2-10 Fluid boluses were administered as needed to ensure daily net positive fluid status to minimize the risk of obstruction. Day 11 Underwent multidisciplinary (cardiothoracic, gynecology, urology, liver, and vascular) resection of the RA mass, including removal of the extending tumor, total hysterectomy, and bilateral salpingo-oophorectomy. Did not require circulatory arrest. POD 1 Extubated without complication. POD 2 Transferred from surgical ICU to the gynecology oncology stepdown unit. POD 2-3 Had ongoing hematuria. Received a total of 3 U of red blood cells, last on POD 3. POD 6 TTE demonstrated resolution of TR. Chest tube was removed. POD 7 Found to have nonocclusive deep vein thrombosis in left internal jugular vein on upper extremity doppler ultrasound. Started on therapeutic enoxaparin. POD 11 Discharged from hospital. 30-day follow-up Complete resolution of symptoms. CT = computed tomography; ICU = intensive care unit; IVC = inferior vena cava; POD = postoperative day; RA = right atrial; TR = tricuspid regurgitation; TTE = transthoracic echocardiogram. Timeline of the Case CT = computed tomography; ICU = intensive care unit; IVC = inferior vena cava; POD = postoperative day; RA = right atrial; TR = tricuspid regurgitation; TTE = transthoracic echocardiogram.

The patient’s RA mass was notable given its size (measuring 2.4 × 3.1 cm) and structure (with a stalk attached to the interatrial septum). Although the images were not reviewable at our center, radiographical reports described extension of the mass from the tricuspid valve (TV) into the RA and IVC into the right common iliac vein. Given her previous history of RA mass, we considered the diagnosis of recurrent Masson’s tumor/hemangioma. Other nonmalignant tumors, such as myxoma, papillary fibroelastoma, and lipoma, were considered, although the extension of the mass into the venous system was less consistent with these diagnoses. Given the findings of intravenous extension from the RA to the iliac vein, the diagnosis of intravenous leiomyomatosis (IVL) was high on the differential.

The authors have reported that they have no relationships relevant to the contents of this paper to disclose.

At our center, TTE redemonstrated her known RA mass and revealed preserved left ventricular ejection fraction and moderate TR ( Figure 1 ). The mass was measured as 5.4 × 2.9 cm. The mean gradient across the tricuspid valve was 16.6 mm Hg. The pulmonary artery systolic pressure was estimated to be 34 mm Hg plus the RA pressure. CT venography with intravenous contrast of the chest and abdomen demonstrated multiple intramural uterine fibroids and a large exophytic mass arising from the right uterine body (measuring 15.2 × 6.6 × 17.5 cm). The mass was endophytic into the right internal iliac vein and extended into the right common iliac vein, IVC, and RA ( Figure 2 ). Magnetic resonance imaging of the abdomen and pelvis revealed heterogeneous enhancement of a uterine mass with moderately high signal intensity on T2-weighted sequences but no evidence of internal fat signal, hemorrhage, or restricted diffusion ( Figure 3 ). Figure 1 RA Mass RA mass (red arrow) that originates from the uterus that transiently crosses into the RV (left) and color doppler demonstrating moderate TR secondary to the mass (right), seen on TTE. RA = right atrium; RV = right ventricle; TR = tricuspid regurgitation; TTE = transthoracic echocardiography. Figure 2 RA Mass With Extension RA mass with extension through the IVC (red arrows) that originates from the uterus seen on contrasted chest and abdomen computed tomography coronal view. IVC = inferior vena cava; UM = uterine mass; other abbreviations as in Figure 1 . Figure 3 Uterine Mass Uterine mass seen on contrasted abdomen and pelvis magnetic resonance imaging coronal (left) and axial (right) views. RA Mass RA mass (red arrow) that originates from the uterus that transiently crosses into the RV (left) and color doppler demonstrating moderate TR secondary to the mass (right), seen on TTE. RA = right atrium; RV = right ventricle; TR = tricuspid regurgitation; TTE = transthoracic echocardiography. RA Mass With Extension RA mass with extension through the IVC (red arrows) that originates from the uterus seen on contrasted chest and abdomen computed tomography coronal view. IVC = inferior vena cava; UM = uterine mass; other abbreviations as in Figure 1 . Uterine Mass Uterine mass seen on contrasted abdomen and pelvis magnetic resonance imaging coronal (left) and axial (right) views.