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Ann Thorac Surg 2005;79:707-709
© 2005 The Society of Thoracic Surgeons

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Case report

Leiomyomatosis Metastatic to the Tricuspid Valve Complicated by Pelvic Hemorrhage

^a Division of Cardiothoracic Surgery, Oregon Health Sciences University, Portland, Oregon, USA
^b Department of Anesthesiology, Oregon Health Sciences University, Portland, Oregon, USA

Accepted for publication August 28, 2003.

^* Address reprint requests to Dr Slater, Division of Cardiothoracic Surgery, Oregon Health Sciences University, 3181 Sam Jackson Park Rd, Mail Code L253, Portland, OR 97201, USA.
slaterm{at}ohsu.edu

	Abstract

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Metastatic leiomyomatosis is a rare but potentially life-threatening tumor of smooth muscle cells. Leiomyomas originate predominantly in the uterus and can spread to the lung and, rarely, the heart. We present a case of a 36-year-old woman with known pelvic leiomyomatosis with metastasis to the tricuspid valve. Tricuspid valve replacement was complicated by retroperitoneal hemorrhage from residual pelvic tumor. This potentially catastrophic occurrence should be considered when undertaking resection of intracardiac leiomyomatosis.

	Introduction

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Metastatic leiomyomatosis is a rare but potentially life-threatening tumor of smooth muscle cells that has a propensity to invade the lumen of veins, most commonly the iliac and inferior vena cava. Rarely, it can extend to the cardiac chambers and result in heart failure or sudden death or extend to the lungs [1]. We present a case of metastatic leiomyomatosis requiring tricuspid valve replacement that was complicated by retroperitoneal hemorrhage.

A 36-year-old woman with known history of uterine intravenous leiomyomatosis was referred to the cardiothoracic service after an echocardiogram revealed a right heart mass and tricuspid regurgitation. The patient presented to her primary care provider with complaints of new-onset bilateral lower extremity swelling. She was found to have moderate jugular venous distention without shortness of breath and orthopnea. Lower extremity Doppler ultrasound did not reveal deep vein thrombosis. Ten years earlier, the patient had undergone a hysterectomy and left salpingo-oophorectomy for uterine masses. After surgery, she developed asymptomatic, stable metastasis to her left lung and was found to have residual tumor in her pelvis. She subsequently underwent re-resection of her pelvic mass and right salpingo-oophorectomy that was complicated by ureteral obstruction requiring a left nephrectomy. After her second surgery, she began long-term treatment with a gonadotropin-releasing hormone agonist. Persistent symptoms of pain and bowel obstruction related to her pelvic mass were treated with intravascular tumor embolization 2 weeks before the onset of her lower extremity edema.

At the time of referral to the cardiothoracic service, echocardiography was performed that showed severe right atrial and ventricular enlargement with mildly reduced systolic function, a linear echo dense mass in the right atrium traversing the tricuspid valve with severe tricuspid regurgitation and a 1.5-cm diameter mass in the right ventricle associated with the pulmonic valve. Preoperative laboratory analysis revealed a creatinine of 1.2 mg/dL, hematocrit of 37%, platelet count of 205,000, and an international normalized ratio of 1.05.

The patient was taken to the operating theater for tricuspid valve surgery and tumor resection. Cardiopulmonary bypass was instituted after systemic heparinization to an activated clotting time of greater than 400 seconds and using aortic and bicaval cannulation. The patient was cooled to 28°C, cardioplegia was administered, and the aorta cross-clamped. The tricuspid valve was found to be densely fibrosed with two of the three leaflets thickened and retracted. Multiple pedunculated masses were found attached to the leaflets and the annulus, one of which measured 2 x 6 cm and was prolapsing into the pulmonary outflow tract (Fig 1). The masses were released at their attachments and removed. The tricuspid valve was not reparable and was replaced with a pericardial tissue valve.

View larger version (88K): [in this window] [in a new window]   Fig 1. Gross specimen of the leiomyomatosis removed from the inferior vena cava, right atrium, and right ventricle.

Postoperative echocardiography revealed no residual cardiac mass and good function of the prosthetic valve. On postoperative day 1, the patient underwent intravascular embolization of the left internal iliac artery to further control the ongoing pelvic hemorrhage. The patient returned to the operating room on postoperative day 3, at which time the packs were removed and her abdomen was closed. She was discharged on hospital day 11. Pathologic examination of both the cardiac and retroperitoneal specimens was consistent with leiomyomatosis.

One year after surgery, the patient continues to have a diastolic murmur on examination. Echocardiography demonstrates severe pulmonary insufficiency, but the patient is asymptomatic.

	Comment

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Metastatic leiomyomatosis is a rare benign neoplasm of smooth muscle that has only been described in women. Two theories regarding the etiology of this tumor prevail. The tumor is thought to originate from either the uterine smooth muscle or the smooth muscle of the vessel walls themselves [2]. The intravenous tumor is typically confined to the pelvic vessels, but it can extend to the iliac veins or inferior vena cava. Rarely, it will extend to the cardiac chambers. Although the tumor is histologically benign, it has clinically malignant potential. Leiomyomatosis has been known to occlude the inferior vena cava or metastasize to the lung, as in our patient [3].

With respect to cardiac complications, tumor extension can result in heart failure and sudden death. Patients may remain asymptomatic until the tumor is relatively advanced within the cardiac chambers. Patients commonly present with symptoms of right heart failure secondary to atrioventricular obstruction or valvular insufficiency [4]. Most women with cardiac complications are in the fifth decade of life with a history of hysterectomy [5].

Multiple strategies have been used for resection of intravascular leiomyomas. Reported hemorrhage in one-stage removal of the tumor due to distal anchoring within the inferior vena cava has been documented [6]. With freely mobile tumors, however, thrombectomy is possible without trauma to distal vessels [6].

We present a case of cardiac tumor resection complicated by hemorrhage in a focus of residual pelvic tumor remote from the operative field. This eventuality, although rare, should be considered when undertaking resection of intracardiac leiomyomatosis. In this case, preoperative embolization was not effective in avoiding perioperative hemorrhage and in fact may have been a contributing factor.

	References

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1. Wakiyama H, Sugimoto T, Ataka K, et al. Intravenous leiomyomatosis extending into the right ventricular cavity: one stage radical operation using cardiopulmonary bypass. Angiology. 2000;51:505–509
2. Kullo I, Oh J, Keeney G, Khandheria B, Steward J. Intracardiac leiomyomatosis: echocardiographic features. Chest. 1999;115:587–591[Medline]
3. Takahiro K, Shinfield A, Reni H. Pelvic leiomyoma in the right atrium. Ann Thorac Surg. 1998;99:2095–2096
4. Steinmetz O, Bedard P, Prefontaine M, Bourke M, Bardber G. Uterine tumor in the heart: intravenous leiomyomatosis. Surgery. 1996;119:226–229[Medline]
5. Harris L, Karakouisis C. Intravenous leiomyomatosis with cardiac extension: tumor thrombectomy through an abdominal approach. J Vasc Surg. 2000;31:1046–1051[Medline]
6. Tsuji Y, Yamashita C, Wakiyama H, et al. Surgical treatment for transvenous tumor extension into the heart: four cases. J Vasc Surg. 1998;27:740–744[Medline]

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				N. Luciani, A. Anselmi, F. Glieca, L. Martinelli, and G. Possati Diagnostic and Surgical Issues in Emergency Presentation of a Pelvic Leiomyoma in the Right Heart Ann. Thorac. Surg., May 1, 2009; 87(5): 1589 - 1592. [Abstract] [Full Text] [PDF]

This Article Abstract Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Matthew S. Slater Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Thukkani, N. Articles by Slater, M. S. Search for Related Content PubMed PubMed Citation Articles by Thukkani, N. Articles by Slater, M. S. Related Collections Cardiac - other