Acute Cardiac Care, September 2014; 16(3): 112–113 Copyright © 2014 Informa UK, Ltd ISSN 1748-2941 print/ISSN 1748-295X online DOI: 10.3109/17482941.2014.911916

VIEWPOINT

Sinister syncope Kaveh Sadigh1, Sandeep Gupta2, Muzammil H. Musani3 & Kathleen Stergiopoulos1 1Department

of Medicine, Division of Cardiology, Stony Brook University Medical Center, Stony Brook, NY, USA, of Surgery (Cardiothoracic), Stony Brook University Medical Center, Stony Brook, NY, USA, 3Department of Radiology, Stony Brook University Medical Center, Stony Brook, NY, USA 2Department

A 55-year-old man with no significant past medical history presented to an outpatient office with a history of new-onset exertional syncope. He was otherwise well and did not seek immediate medical attention. An electrocardiogram was unremarkable. At the time of his initial visit, he had new abdominal complaints of nausea and vomiting, and peritoneal signs. His evaluation included a transthoracic echocardiogram which demonstrated normal left ventricular systolic function with no regional wall motion abnormalities, and a large left ventricular mass (6 cm in length) obstructing the left ventricular cavity, originating from the left ventricular apex, with a large base and highly mobile components (Figure 1). No obvious stalk was identified and the mass did not fill with echocardiographic contrast (Figure 2). He was referred to the emergency room, where a computed tomography of the abdomen with angiography revealed occlusion of the distal superior mesenteric artery with

ischemic necrosis of multiple loops of small bowel with evidence of pan-jejunal and ileal pneumatosis. He was urgently referred for exploratory laparotomy, superior mesenteric embolectomy, and extensive small bowel resection. Pathologic specimen revealed malignant tumor cells present within thrombus material, consistent with tumor emboli. Cardiac magnetic resonance imaging of the intracardiac mass revealed intramuscular invasion of the tumor without extension beyond the cardiac borders (Figure 3). Intraoperative transesophageal echocardiography (Figures 4 and 5, Supplementary Video 1 to be found online at http://informahealthcare.com/doi/abs/ 10.3109/17482941.2014.911916) revealed a large mass with highly mobile components, with no other cardiac tumor noted. Eventual wide resection of the intracardiac tumor, which required partial myomectomy and mitral valve replacement due to its extensive nature, revealed a poorly differentiated malignant neoplasm with an extensive

Figure 1. Apical three-chamber view, two-dimensional. A large left ventricular mass (6 cm in length) obstructing the left ventricular cavity, originating from the left ventricular apex.

Figure 2. Apical three-chamber view, two-dimensional with echocardiographic contrast. The mass does not fill with echocardiographic contrast suggestive of thrombus or necrosis rather than a vascular structure.

Correspondence: Kathleen Stergiopoulos, Stony Brook University Medical Center, Department of Medicine, Division of Cardiology, Health Sciences Center T 16–080, Stony Brook, NY 11794–8167, USA. Fax: ⫹ 1 631 444 1054. E-mail: [email protected] (Received 27 February 2014; accepted 11 March 2014)

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Figure 5. Intraoperative transesophageal echocardiography (TEE). Three-dimensional TEE mid-esophageal view at 0 degrees, demonstrating a large intracardiac mass with highly mobile surface components (white arrow). Figure 3. Cardiac magnetic resonance imaging of the intracardiac mass revealed intramuscular invasion of the tumor without extension beyond the cardiac borders (Panel C).

Figure 4. Intraoperative transesophageal echocardiography (TEE). Mid esophageal view at 0 degrees, demonstrating a large intracardiac mass with highly mobile surface components.

centrally necrotic tumor with high-grade, pleomorphic morphology with marked mitotic activity, consistent with a sarcoma. Primary cardiac tumors are rare, most of which are

Supplementary material available online Supplementary Video 1. This video shows intraoperative three-dimensional TEE at 0 degrees mid-esophageal view. Supplementary Video 1 to be found online at http:// informahealthcare.com/doi/abs/10.3109/17482941.2014. 911916.

© 2014 Informa UK, Ltd.

benign, and 25% are classified as malignant (1). Primary cardiac sarcomas often affect young patients and have a poor prognosis. Left-sided cardiac sarcomas often present with heart failure, obstruction to blood flow, interference with valve function, arrhythmias due to local invasion, pericardial effusion and tamponade, and/or embolic phenomena from tumor fragments or thrombus (1). Aggressive surgical excision offers the best long-term prognosis but recurrence may occur. The anatomic location of a left ventricular cardiac sarcoma makes complete excision a challenge for the surgeon. Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.

Reference 1. Shanmugam G. Primary cardiac sarcoma. Eur J Cardio-thorac Surg. 2006:29;925–32.

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