- Email: [email protected]
Intracardiac Leiomyomatosis Complicated by Pulmonary Embolism: A Multimodality Imaging Case of a Rare Entity
Canadian Journal of Cardiology 29 (2013) 1743.e1e1743.e3 www.onlinecjc.ca
Case Report
Intracardiac Leiomyomatosis Complicated by Pulmonary Embolism: A Multimodality Imaging Case of a Rare Entity Vânia Ribeiro, MD,a Jorge Almeida, MD,b António J. Madureira, MD,c Elisa Lopez, MD,b Luís Machado, MD,d Roncon Albuquerque, MD, PhD,d and Paulo Pinho, MDb a
Cardiology Department, São João Hospital, Medicine Faculty of Porto University, Porto, Portugal b
c d
Cardiothoracic Surgery Department, São João Hospital, Porto, Portugal
Radiology Department, São João Hospital, Medicine Faculty of Porto University, Porto, Portugal
Vascular Surgery Department, São João Hospital, Porto, Portugal, Medicine Faculty of Porto University, Porto, Portugal
ABSTRACT
RESUM E
We present a case of intravenous leiomyomatosis with intracaval and right ventricle extension that was misdiagnosed as venous thrombus. Part of the mass had split and embolized the pulmonary artery, requiring urgent surgery. Although the mass fragments were removed from the inferior vena cava, right ventricle, and pulmonary artery successfully, this case clearly shows the importance of prompt surgery.
sentons un cas de le iomyomatose intraveineuse avec extenNous pre te diagnostique à tort sion intracave et au ventricule droit qui a e comme une thrombose veineuse. Une partie de la masse s’est tache e et a provoque une embolie de l’artère pulmonaire, qui de cessite une chirurgie urgente. Bien que les fragments de la masse a ne te retire s de la veine cave infe rieure, du ventricule droit et de aient e l’artère pulmonaire avec succès, ce cas montre clairement l’imporder rapidement à la chirurgie. tance de proce
We present the case of a 74-year-old woman using oral anticoagulation for inferior vena cava (IVC) thrombosis 3 years earlier. The patient was overweight (body mass index ¼ 29), and underwent total hysterectomy 18 years previously, because of uterine leiomyoma. On the work-up for exertional dyspnea, transthoracic echo showed an extensive mass that started at the IVC, extended into the right atrium and through the tricuspid valve into the right ventricle, ending at the level of the pulmonary valve (Fig. 1, A and B; Videos 1 and 2 , view video online). The diagnosis was venous thrombus in transit and a computed tomography scan was requested. The exam confirmed the echocardiographic findings but there were no signs of acute or chronic pulmonary embolism. It was noticed that the mass extended distally along the IVC and there were no abnormal masses in the liver or kidneys. The behaviour of the “thrombus” and its progression despite use of oral anticoagulation therapy led us to consider
intravenous leiomyomatosis (IVL) as the more probable etiology, in view of the patient’s gynecological history and the absence of liver or kidney involvement. A magnetic resonance imaging scan was performed, which depicted several pelvic masses that “invaded” and grew into the right common iliac vein and along the IVC, right atrium, right ventricle, and across the pulmonary valve into the pulmonary trunk (Fig. 1, C-E; Video 3 , view video online), strongly supporting the diagnosis of IVL. While waiting for elective surgery, an acute episode of dyspnea occurred and transthoracic echo showed that the mass had fractured at the IVC/right atrium junction level (Fig. 1F; Video 4 , view video online). Because a central right pulmonary artery embolism was observed in the computed tomography scan (Fig. 1G), we deduced that part of the intracardiac mass had split and a fragment had embolized the pulmonary artery. Urgent surgery was performed. During intraoperative transesophageal echo, 2 small fragments of the mass were observed at the right ventricle outflow tract. Tumoural masses were removed from the inferior vena cava, right ventricle, and pulmonary artery, successfully (Fig. 1H). The postoperative period was uneventful and the patient was discharged on the seventh day after surgery. Pathologic examination of the material removed from the different sites
Received for publication June 3, 2013. Accepted September 11, 2013. Corresponding author: Dr Vânia Ribeiro, Alameda Professor Hêrnani Monteiro, 4200-319 Porto, Portugal. Tel.: þ351-91-6256673. E-mail: [email protected] See page 1743.e3 for disclosure information.
0828-282X/$ - see front matter Ó 2013 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.cjca.2013.09.008
1743.e2
Canadian Journal of Cardiology Volume 29 2013
Figure 1. (A) Subcostal view of TTE showing a mass (arrow) extending from the inferior vena cava into the right ventricle. (B) Short axis view of TTE at the aortic level showing the mass (arrow) in the right ventricular outflow tract at the level of the pulmonary valve. (C) MIP from the 3-D angiography. The venous mass (arrow) and the enhancing pelvic masses (circle) are clearly identified. (D) Coronal T2 weighted magnetic resonance image depicts 3 slightly hyperintense pelvic masses with lobulated contours (circle). (E) Coronal steady-state free-precession image. The distal part of the mass extends from the inferior vena cava, through the right atrium and right ventricular outflow tract (arrows), into the main pulmonary artery
Ribeiro et al. Intracardiac Leiomyomatosis
was identical (Fig. 1I) and confirmed the diagnosis of IVL. No thrombus or clots were identified. IVL is a rare benign tumour of smooth muscle cells originating from the uterine venous wall or uterine leiomyoma.1-3 Despite its rarity it should be considered in the differential diagnosis of right cardiac mass in a female patient, particularly after hysterectomy or with known uterine fibroids.2,4 Although it is histologically benign, it might be clinically aggressive and cause fatal cardiovascular complications such as cardiac failure, valve obstruction, pulmonary embolism, or sudden death.3,5 Embolization into the pulmonary artery is a very rare complication constituting less than 5% of the reported cases,3 but the risk is real as evidenced by our case. Although this case is not unique, the risk of embolization needs to be considered to prompt urgent surgery when IVL is recognized. Disclosures The authors have no conflicts of interest to disclose. References 1. Lou Y, Shi X, Song Z. Intravenous leiomyomatosis of the uterus with extension to the right heart. Cardiovasc Ultrasound 2011;9:25.
1743.e3
2. Guo X, Zhang C, Fang L, et al. Echocardiographic characteristics of intravenous leiomyomatosis with intracardiac extension: a singleinstitution experience. Echocardiography 2011;28:934-40.
3. Lee S, Kim D, Narm KS, Cho S. Pulmonary artery embolization of intravenous leiomyomatosis extending into the right atrium. Korean J Thorac Cardiovasc Surg 2011;44:243-6.
4. Spanuchart I, Satitthummanid S, Cheanvechai C, et al. Intracardiac and intravenous leiomyomatosis. J Am Coll Cardiol 2012;60:e27.
5. Zhang C, Liu X, Ma G, et al. Pulmonary embolization as the primary clinical manifestation of intravenous leiomyomatosis with intracardiac extension. Ann Thorac Surg 2012;94:1012.
Supplementary Material To access the supplementary material accompanying this article, visit the online version of the Canadian Journal of Cardiology at www.onlinecjc.ca and at http://dx.doi.org/10. 1016/j.cjca.2013.09.008.
Figure 1. (continued) (not shown). (F) Subcostal view of TTE showing the mass fractured at level of the inferior vena cava, near the right atrium junction (arrow). (G) Thoracic computed tomography scan showing a central pulmonary embolism, blocking the lumen of the right pulmonary artery (arrow). (H) Gross findings of the masses removed from the right pulmonary artery (1), right ventricle (2) and inferior vena cava (3). (I) Hematoxylin and eosin staining showing smooth muscle cells with no abnormal mitotic activity. AV, aortic valve; LA, left atrium; MIP, maximum intensity projection; RA, right atrium; RV, right ventricle; TTE, transthoracic echo.
Case Report
Intracardiac Leiomyomatosis Complicated by Pulmonary Embolism: A Multimodality Imaging Case of a Rare Entity Vânia Ribeiro, MD,a Jorge Almeida, MD,b António J. Madureira, MD,c Elisa Lopez, MD,b Luís Machado, MD,d Roncon Albuquerque, MD, PhD,d and Paulo Pinho, MDb a
Cardiology Department, São João Hospital, Medicine Faculty of Porto University, Porto, Portugal b
c d
Cardiothoracic Surgery Department, São João Hospital, Porto, Portugal
Radiology Department, São João Hospital, Medicine Faculty of Porto University, Porto, Portugal
Vascular Surgery Department, São João Hospital, Porto, Portugal, Medicine Faculty of Porto University, Porto, Portugal
ABSTRACT
RESUM E
We present a case of intravenous leiomyomatosis with intracaval and right ventricle extension that was misdiagnosed as venous thrombus. Part of the mass had split and embolized the pulmonary artery, requiring urgent surgery. Although the mass fragments were removed from the inferior vena cava, right ventricle, and pulmonary artery successfully, this case clearly shows the importance of prompt surgery.
sentons un cas de le iomyomatose intraveineuse avec extenNous pre te diagnostique à tort sion intracave et au ventricule droit qui a e comme une thrombose veineuse. Une partie de la masse s’est tache e et a provoque une embolie de l’artère pulmonaire, qui de cessite une chirurgie urgente. Bien que les fragments de la masse a ne te retire s de la veine cave infe rieure, du ventricule droit et de aient e l’artère pulmonaire avec succès, ce cas montre clairement l’imporder rapidement à la chirurgie. tance de proce
We present the case of a 74-year-old woman using oral anticoagulation for inferior vena cava (IVC) thrombosis 3 years earlier. The patient was overweight (body mass index ¼ 29), and underwent total hysterectomy 18 years previously, because of uterine leiomyoma. On the work-up for exertional dyspnea, transthoracic echo showed an extensive mass that started at the IVC, extended into the right atrium and through the tricuspid valve into the right ventricle, ending at the level of the pulmonary valve (Fig. 1, A and B; Videos 1 and 2 , view video online). The diagnosis was venous thrombus in transit and a computed tomography scan was requested. The exam confirmed the echocardiographic findings but there were no signs of acute or chronic pulmonary embolism. It was noticed that the mass extended distally along the IVC and there were no abnormal masses in the liver or kidneys. The behaviour of the “thrombus” and its progression despite use of oral anticoagulation therapy led us to consider
intravenous leiomyomatosis (IVL) as the more probable etiology, in view of the patient’s gynecological history and the absence of liver or kidney involvement. A magnetic resonance imaging scan was performed, which depicted several pelvic masses that “invaded” and grew into the right common iliac vein and along the IVC, right atrium, right ventricle, and across the pulmonary valve into the pulmonary trunk (Fig. 1, C-E; Video 3 , view video online), strongly supporting the diagnosis of IVL. While waiting for elective surgery, an acute episode of dyspnea occurred and transthoracic echo showed that the mass had fractured at the IVC/right atrium junction level (Fig. 1F; Video 4 , view video online). Because a central right pulmonary artery embolism was observed in the computed tomography scan (Fig. 1G), we deduced that part of the intracardiac mass had split and a fragment had embolized the pulmonary artery. Urgent surgery was performed. During intraoperative transesophageal echo, 2 small fragments of the mass were observed at the right ventricle outflow tract. Tumoural masses were removed from the inferior vena cava, right ventricle, and pulmonary artery, successfully (Fig. 1H). The postoperative period was uneventful and the patient was discharged on the seventh day after surgery. Pathologic examination of the material removed from the different sites
Received for publication June 3, 2013. Accepted September 11, 2013. Corresponding author: Dr Vânia Ribeiro, Alameda Professor Hêrnani Monteiro, 4200-319 Porto, Portugal. Tel.: þ351-91-6256673. E-mail: [email protected] See page 1743.e3 for disclosure information.
0828-282X/$ - see front matter Ó 2013 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.cjca.2013.09.008
1743.e2
Canadian Journal of Cardiology Volume 29 2013
Figure 1. (A) Subcostal view of TTE showing a mass (arrow) extending from the inferior vena cava into the right ventricle. (B) Short axis view of TTE at the aortic level showing the mass (arrow) in the right ventricular outflow tract at the level of the pulmonary valve. (C) MIP from the 3-D angiography. The venous mass (arrow) and the enhancing pelvic masses (circle) are clearly identified. (D) Coronal T2 weighted magnetic resonance image depicts 3 slightly hyperintense pelvic masses with lobulated contours (circle). (E) Coronal steady-state free-precession image. The distal part of the mass extends from the inferior vena cava, through the right atrium and right ventricular outflow tract (arrows), into the main pulmonary artery
Ribeiro et al. Intracardiac Leiomyomatosis
was identical (Fig. 1I) and confirmed the diagnosis of IVL. No thrombus or clots were identified. IVL is a rare benign tumour of smooth muscle cells originating from the uterine venous wall or uterine leiomyoma.1-3 Despite its rarity it should be considered in the differential diagnosis of right cardiac mass in a female patient, particularly after hysterectomy or with known uterine fibroids.2,4 Although it is histologically benign, it might be clinically aggressive and cause fatal cardiovascular complications such as cardiac failure, valve obstruction, pulmonary embolism, or sudden death.3,5 Embolization into the pulmonary artery is a very rare complication constituting less than 5% of the reported cases,3 but the risk is real as evidenced by our case. Although this case is not unique, the risk of embolization needs to be considered to prompt urgent surgery when IVL is recognized. Disclosures The authors have no conflicts of interest to disclose. References 1. Lou Y, Shi X, Song Z. Intravenous leiomyomatosis of the uterus with extension to the right heart. Cardiovasc Ultrasound 2011;9:25.
1743.e3
2. Guo X, Zhang C, Fang L, et al. Echocardiographic characteristics of intravenous leiomyomatosis with intracardiac extension: a singleinstitution experience. Echocardiography 2011;28:934-40.
3. Lee S, Kim D, Narm KS, Cho S. Pulmonary artery embolization of intravenous leiomyomatosis extending into the right atrium. Korean J Thorac Cardiovasc Surg 2011;44:243-6.
4. Spanuchart I, Satitthummanid S, Cheanvechai C, et al. Intracardiac and intravenous leiomyomatosis. J Am Coll Cardiol 2012;60:e27.
5. Zhang C, Liu X, Ma G, et al. Pulmonary embolization as the primary clinical manifestation of intravenous leiomyomatosis with intracardiac extension. Ann Thorac Surg 2012;94:1012.
Supplementary Material To access the supplementary material accompanying this article, visit the online version of the Canadian Journal of Cardiology at www.onlinecjc.ca and at http://dx.doi.org/10. 1016/j.cjca.2013.09.008.
Figure 1. (continued) (not shown). (F) Subcostal view of TTE showing the mass fractured at level of the inferior vena cava, near the right atrium junction (arrow). (G) Thoracic computed tomography scan showing a central pulmonary embolism, blocking the lumen of the right pulmonary artery (arrow). (H) Gross findings of the masses removed from the right pulmonary artery (1), right ventricle (2) and inferior vena cava (3). (I) Hematoxylin and eosin staining showing smooth muscle cells with no abnormal mitotic activity. AV, aortic valve; LA, left atrium; MIP, maximum intensity projection; RA, right atrium; RV, right ventricle; TTE, transthoracic echo.