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CASE REPORT Open Access Malignantmitral stenosis Johann Auer 1,2* , Robert Berent 3 and Franz Gurtner 1 Abstract Symptomatic mitral stenosis caused by a left atrial mass as the first sign of metastasis of a malignant tumor is extremely rare and frequently associated with poor prognosis. We report a case of a 59-year-old man with a history of grade 3 malignant fibrous histiocytoma on his left tigh treated by limb-sparing surgery 17 months earlier, who was admitted with 10-days of worsening dyspnea. Imaging revealed a left atrial mass protruding through the mitral valve that resulted in severe mitral stenosis. Biopsy confirmed metastasis of malignant fibrous histiocytoma. Keywords: Metastasis, Heart failure, Dyspnea, Echocardiography, Computed tomography Background Symptomatic mitral stenosis caused by a left atrial mass as the first sign of metastasis of a malignant tumor is extremely rare and frequently associated with poor prognosis. Atrial tumours presenting as mitral stenosis are most commonly myxomata, occasionally peduncu- lated sarcoma, and very rarely metastases. Case Presentation A 59-year-old man with a history of grade 3 malignant fibrous histiocytoma on his left tigh Stage IIA (pT1bN0M0) treated by limb-sparing surgery 17 months earlier, was admitted with 10-days of worsening dys- pnea. The patient underwent postoperative chemother- apy after surgery and had follow up visits every six months. Blood pressure and heart rate were 150/85 mmHg and 136 beats/minute, respectively. Cardiac aus- cultation revealed a diastolic murmur. End-inspiratory crackles suggested pulmonary edema. Echocardiography revealed a left atrial mass protruding through the mitral valve (Figures 1 and 2). Continous wave spectral Dop- pler showed mitral stenosis with a mitral valve area less than 1.0 cm 2 (Figures 3 and 4). Additionally, a giant mass in the left pleural space penetrating the left pul- monary veins could be demonstrated by ultrasound (Fig- ures 5 and 6) and by computed tomography (Figures 7 and 8). Biopsy of the pleural tumor revealed metastasis of malignant fibrous histiocytoma (Figure 9). Pulmonary edema resolved with symptomatic treatment. Before * Correspondence: [email protected] 1 Department of Cardiology and Intensive Care, General Hospital Braunau, Braunau, Austria Full list of author information is available at the end of the article Figure 1 Echocardiography shows a giant left atrial mass. Figure 2 Echocardiogram with a left atrial mass protruding through the mitral valve. Auer et al. Journal of Cardiothoracic Surgery 2012, 7:19 http://www.cardiothoracicsurgery.org/content/7/1/19 © 2012 Auer et al; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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Page 1: CASE REPORT Open Access Malignant mitral stenosis

CASE REPORT Open Access

“Malignant” mitral stenosisJohann Auer1,2*, Robert Berent3 and Franz Gurtner1

Abstract

Symptomatic mitral stenosis caused by a left atrial mass as the first sign of metastasis of a malignant tumor isextremely rare and frequently associated with poor prognosis. We report a case of a 59-year-old man with a historyof grade 3 malignant fibrous histiocytoma on his left tigh treated by limb-sparing surgery 17 months earlier, whowas admitted with 10-days of worsening dyspnea. Imaging revealed a left atrial mass protruding through themitral valve that resulted in severe mitral stenosis. Biopsy confirmed metastasis of malignant fibrous histiocytoma.

Keywords: Metastasis, Heart failure, Dyspnea, Echocardiography, Computed tomography

BackgroundSymptomatic mitral stenosis caused by a left atrial massas the first sign of metastasis of a malignant tumor isextremely rare and frequently associated with poorprognosis. Atrial tumours presenting as mitral stenosisare most commonly myxomata, occasionally peduncu-lated sarcoma, and very rarely metastases.

Case PresentationA 59-year-old man with a history of grade 3 malignantfibrous histiocytoma on his left tigh Stage IIA(pT1bN0M0) treated by limb-sparing surgery 17 monthsearlier, was admitted with 10-days of worsening dys-pnea. The patient underwent postoperative chemother-apy after surgery and had follow up visits every sixmonths. Blood pressure and heart rate were 150/85mmHg and 136 beats/minute, respectively. Cardiac aus-cultation revealed a diastolic murmur. End-inspiratorycrackles suggested pulmonary edema. Echocardiographyrevealed a left atrial mass protruding through the mitralvalve (Figures 1 and 2). Continous wave spectral Dop-pler showed mitral stenosis with a mitral valve area lessthan 1.0 cm2 (Figures 3 and 4). Additionally, a giantmass in the left pleural space penetrating the left pul-monary veins could be demonstrated by ultrasound (Fig-ures 5 and 6) and by computed tomography (Figures 7and 8). Biopsy of the pleural tumor revealed metastasisof malignant fibrous histiocytoma (Figure 9). Pulmonaryedema resolved with symptomatic treatment. Before

* Correspondence: [email protected] of Cardiology and Intensive Care, General Hospital Braunau,Braunau, AustriaFull list of author information is available at the end of the article

Figure 1 Echocardiography shows a giant left atrial mass.

Figure 2 Echocardiogram with a left atrial mass protrudingthrough the mitral valve.

Auer et al. Journal of Cardiothoracic Surgery 2012, 7:19http://www.cardiothoracicsurgery.org/content/7/1/19

© 2012 Auer et al; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative CommonsAttribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction inany medium, provided the original work is properly cited.

Page 2: CASE REPORT Open Access Malignant mitral stenosis

discussing further treatment options, the patient diedsuddenly four days after admission.

ConclusionAtrial tumours presenting as mitral stenosis are mostcommonly myxomata, occasionally pedunculated sar-coma, and very rarely metastases [1,2].Symptomatic mitral stenosis caused by a left atrial

mass as the first sign of metastasis of a malignant tumoris extremely rare and frequently associated with poor

Figure 3 Continous wave (CW) - spectral Doppler tracingindicating mitral stenosis with a mitral valve area less than 1.0cm2.

Figure 4 CW - spectral Doppler tracing indicating severe mitralstenosis.

Figure 5 Sonography demonstrating a giant mass in the leftpleural space penetrating the left pulmonary veins.

Figure 6 Sonography with a large mass in the left pleuralspace.

Figure 7 Computed tomography shows a large mass in the leftpleural space.

Auer et al. Journal of Cardiothoracic Surgery 2012, 7:19http://www.cardiothoracicsurgery.org/content/7/1/19

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Page 3: CASE REPORT Open Access Malignant mitral stenosis

prognosis [1-3]. However, there are some reports aboutsuccessful favourable response with combined treatmentparticularly in patients with high tumor mitotic rate[4,5].

ConsentWritten informed consent was obtained from the patientfor publication of this report and any accompanyingimages.

AcknowledgementsWe would like to acknowledge and thank Dr. Günter Schatzl and Dr. CarinaPrimus for their support and critical reviews.

Author details1Department of Cardiology and Intensive Care, General Hospital Braunau,Braunau, Austria. 2Department of Cardiology and Intensive Care, GeneralHospital Simbach, Simbach, Germany. 3Center of Cardiac Rehabilitation, BadSchallerbach, Austria.

Authors’ contributionsJA wrote the manuscript and formatted the images. FG providedcardiovascular images and reports. RB supervised and revised the draftmanuscript. All authors read and approved the final manuscript.

Competing interestsThe authors declare that they have no competing interests.

Received: 3 November 2011 Accepted: 8 March 2012Published: 8 March 2012

References1. Reynen K, Köckeritz U, Strasser RH: Metastases to the heart. Ann Oncol

2004, 15:375-381.2. Stems LP, Eliot RS, Varco RL, Edwards JE: Intracavitary cardiac neoplasms.

A review of fifteen cases. Br Heart J 1966, 28:7543-7546.3. Hepp A, Larbig D, Bader H: Left atrial metastasis of chorion carcinoma,

presenting as mitral stenosis. Br Heart J 1977, 39:1154-1156.4. Recchia F, Saggio G, Amiconi G, Di Blasio A, Cesta A, Candeloro G, Rea S,

Nappi G: Cardiac metastases in malignant fibrous histiocytoma. A casereport. Tumori 2006, 92:76-78.

5. Schena S, Caniglia A, Agnino A, Caruso G, Ferlan G: Survival followingtreatment of a cardiac malignant fibrous histiocytoma. Chest 2000,118:271-273.

doi:10.1186/1749-8090-7-19Cite this article as: Auer et al.: “Malignant” mitral stenosis. Journal ofCardiothoracic Surgery 2012 7:19.

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Figure 8 Computed tomography shows a large mass in the leftpleural space penetrating the left pulmonary veins andprotruding to the left atrium and through the mitral valve.

Figure 9 Biopsy of the pleural tumor revealed metastasis ofmalignant fibrous histiocytoma.

Auer et al. Journal of Cardiothoracic Surgery 2012, 7:19http://www.cardiothoracicsurgery.org/content/7/1/19

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