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Pulmonary artery embolization of intravenous leiomyomatosis extending into the right atrium.
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PMID:  22263160     Owner:  NLM     Status:  PubMed-not-MEDLINE    
A 43-year-old woman was diagnosed with an intravenous leiomyomatosis at a previous hospital and transferred to our hospital to undergo surgical treatment. Emergency one-stage operation for coincidental removal of intra-abdominal, right atrial, and intravenous masses were planned. Upon arriving at the operating room, she suffered a sudden onset of severe dyspnea and showed hemodynamic instability. Intraoperative TEE showed pulmonary embolization of a right atrial mass. Removal of the pulmonary artery mass and the intra-abdominal mass, and the cardiopulmonary bypass were performed without any complications.
Sak Lee; Do-Kyun Kim; Kyoung Shik Narm; Sang-Ho Cho
Publication Detail:
Type:  Journal Article     Date:  2011-06-11
Journal Detail:
Title:  The Korean journal of thoracic and cardiovascular surgery     Volume:  44     ISSN:  2093-6516     ISO Abbreviation:  Korean J Thorac Cardiovasc Surg     Publication Date:  2011 Jun 
Date Detail:
Created Date:  2012-01-20     Completed Date:  2012-08-23     Revised Date:  2013-05-29    
Medline Journal Info:
Nlm Unique ID:  101563922     Medline TA:  Korean J Thorac Cardiovasc Surg     Country:  Korea (South)    
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Languages:  eng     Pagination:  243-6     Citation Subset:  -    
Department of Thoracic and Cardiovascular Surgery, Severance Hospital, Yonsei University Health System, Korea.
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Journal Information
Journal ID (nlm-ta): Korean J Thorac Cardiovasc Surg
Journal ID (publisher-id): KJTCS
ISSN: 2233-601X
ISSN: 2093-6516
Publisher: Korean Society for Thoracic and Cardiovascular Surgery
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© The Korean Society for Thoracic and Cardiovascular Surgery. 2011. All right reserved.
Received Day: 03 Month: 10 Year: 2010
Revision Received Day: 27 Month: 10 Year: 2010
Accepted Day: 10 Month: 5 Year: 2011
Print publication date: Month: 6 Year: 2011
Electronic publication date: Day: 11 Month: 6 Year: 2011
Volume: 44 Issue: 3
First Page: 243 Last Page: 246
ID: 3249311
PubMed Id: 22263160
DOI: 10.5090/kjtcs.2011.44.3.243

Pulmonary Artery Embolization of Intravenous Leiomyomatosis Extending into the Right Atrium
Sak Lee, M.D., Ph.D.*
Do-Kyun Kim, M.D., Ph.D.**
Kyoung Shik Narm, M.D.*
Sang-Ho Cho, M.D.*
*Department of Thoracic and Cardiovascular Surgery, Severance Hospital, Yonsei University Health System, Korea.
**Department of Thoracic and Cardiovascular Surgery, Ilsan Hospital, Korea.
Correspondence: Corresponding author: Sak Lee, Department of Thoracic and Cardiovascular Surgery, Severance Hospital, Yonsei University College of Medicine, 134, Sinchon-dong, Seodaemun-gu, Seoul 120-749, Korea. (Tel) 82-2-2228-8488, (Fax) 82-2-313-2992,


A 43-year-old woman diagnosed with intravenous leiomyomatosis at another hospital was transferred to our hospital. She had suffered from palpitation, dizziness, dyspnea, and chest pain for two weeks prior to admission into another hospital, and had experienced syncope three times on the day of admission to our hospital. No cardiac murmur was noted, and laboratory findings were unremarkable with the exception of anemia (Hemoglobin: 9.7 g/L). Cardiac and pelvic magnetic resonance imaging (MRI) taken at the previous hospital revealed multiple low attenuation masses in the uterus, involving both ovaries, the left gonadal vein, the left internal iliac vein, and the left renal vein, which extended into the inferior vena cava (IVC), and to the right atrium (RA) (Fig. 1). A trans-thoracic echocardiogram revealed a 4.8×1.8-cm hypermobile and echogenic mass in the RA connected to the mass in the IVC. Emergency one-stage operation was planned with a gynecologist, and upon being transferred to the operation room, the patient had a sudden onset of dyspnea and unstable blood pressure. Rapid endotracheal intubation was performed to maintain blood oxygen saturation. In transesophageal echocardiography (TEE), the echogenic mass in the RA and IVC was not seen. We suggested that the mass detached from the RA and migrated to the right ventricle or the pulmonary artery. We discovered an echogenic linear mass in the pulmonary artery between the arterial bifurcation and both main pulmonary arterial branches (Fig. 2). Trans-abdominal hysterectomy and left salphingo-oophorectomy were performed through median laparotomy, and the mass in the left gonadal and internal iliac veins was completely removed (Fig. 3A). Another mass in the pulmonary artery was also removed under conventional cardiopulmonary bypass without circulatory arrest (Fig. 3B). The RA was opened to confirm that there was no remnant mass in the RA and the IVC. The histologic study confirmed intravenous leiomyomatosis. The patient's postoperative course was uneventful, and she has received follow-up care for two years without any evidence of tumor recurrence.


Intravenous leiomyomatosis is a benign intravascular proliferation of smooth muscle cells originating from the intrauterine venules and reaching the right heart [1]. The uncommon benign tumor usually arises from either the uterine venous wall or uterine leiomyoma [2], and although it is histologically benign, it can cause fatal cardiovascular complications such as cardiac failure, pulmonary embolization, or sudden death [3]. In previously reported cases describing intracardiac involvement, the tumor extended to the right atrium in 30% of cases, and to the right ventricle in 70% of cases [3,4]. Extension or embolization into the pulmonary artery or lung metastasis is a very rare complication constituting less than 5% of the reported cases [5]. However, when it occurs, the course of the patient can be fatal, and urgent surgical correction is imperative.

Ariza and colleagues described the first successful two-stage removal of an intracaval mass, with delayed laparotomy after resection of the intracardiac portion of a tumor in 1982 [6]. Since then, a staged operation has been performed in many cases (radical resections of intracavocardiac tumors and intrapelvic components in separate operations), but more recently, a one-stage approach by median sternotomy with cardiopulmonary bypass with or without hypothermic circulatory arrest, and a separate laparotomy has been performed successfully [1,3,4]. The advantages of the one-stage resection include avoiding risks of tumor embolism, tumor progression, or hemodynamic complications in the interval between the two stages of two-staged operations. In addition, because both abdominal and thoracic cavities are opened, the tumor can be removed completely, and vascular reconstruction and bleeding control may be more easily performed [7]. However, in hemodynamically unstable patients, the longer operative time may not be appropriate, and a two-stage operation may be beneficial.

Since incomplete resection of the tumor may causes recurrence, gathering detailed preoperative information regarding tumor localization, size, and extent by using abdominal ultrasound, echocardiogram, computed tomography, magnetic resonance imaging, and venography is required [8]. Transesophageal echocardiography should also be performed to detect possible changes in the location and size of the tumor, as occurred in this case.

In conclusion, intracardiac leiomyomatosis should be considered in a female patient presenting with an extensive mass in the right side of the heart, and urgent surgical removal should be performed to avoid possible fatal complications, even if the patient is asymptomatic. As the tumor may move or detach from the central venous system even during operation, intraoperative TEE is considered a very important diagnostic tool.

1. Rispoli P,Santovito D,Tallia C,Varetto G,Conforti M,Rinaldi M. A one-stage approach to the treatment of intravenous leiomyomatosis extending to the right heartJ Vasc SurgYear: 20105221221520471777
2. Nam MS,Jeon MJ,Kim YT,Kim JW,Park KH,Hong YS. Pelvic leiomyomatosis with intracaval and intracardiac extension: a case report and review of the literatureGynecol OncolYear: 20038917518012694674
3. Uchida H,Hattori Y,Nakada K,Iida T. Successful one-stage radical removal of intravenous leiomyomatosis extending to the right ventricleObstet GynecolYear: 20041031068107015121610
4. Harris LM,Karakousis CP. Intravenous leiomyomatosis with cardiac extension: tumor thrombectomy through an abdominal approachJ Vasc SurgYear: 2000311046105110805899
5. Lo KW,Lau TK. Intracardiac leiomyomatosis. Case report and literature reviewArch Gynecol ObstetYear: 200126420921011205711
6. Ariza A,Cerra C,Hahn IS,Shaw RK,Rigney B. Intravascular leiomyomatosis of the uterus. A case reportConn MedYear: 1982467007037151431
7. Zhang C,Miao Q,Liu X,et al. Intravenous leiomyomatosis with intracardiac extensionAnn Thorac SurgYear: 2010891641164320417800
8. Kocica M,Vranes MR,Kostic D,et al. Intravenous leiomyomatosis with extension to the heart: rare or underestimated?J Thorac Cardiovasc SurgYear: 20051301724172616308028

Article Categories:
  • Case Report

Keywords: Pulmonary artery, Embolization, Leiomyomatosis.

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