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A Large Angiosarcoma of the Right Atrium

Huang, Jiapeng MD, PhD*†; Bouvette, Michael J. MD*†; Zhou, Jing MSNA, CRNA; Dwyer, George J. III MD*†; Bhopatkar, Shailesh MD*†; Bhatia, Aneeta MD*†

doi: 10.1213/ane.0b013e3181a1c631
Cardiovascular Anesthesiology: Echo Rounds
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Video 1
Video 2
Video 3

From the *Department of Anesthesia, Jewish Hospital and St. Mary’s Healthcare; and †Department of Anesthesiology and Perioperative Medicine, University of Louisville, Louisville, Kentucky.

Accepted for publication January 2, 2009.

Reprints will not be available from the author.

Address correspondence to Jiapeng Huang, MD, PhD, 200 Abraham Flexner Way, Louisville, KY 40202. Address e-mail to jiapenghuang@yahoo.com.

A 30-yr old man presented to the emergency room with chest pain and scant hemoptysis. His medical history was significant for hypertension, asthma, and pericardial effusions. A computed axial tomography scan of the chest showed a large mass involving the lateral, anterolateral, and inferior walls of the right artium. A pericardial effusion around the ascending aorta was also identified. It was unclear if the mass was intracardiac or extracardiac based on the computed axial tomography scan. The patient was scheduled for a right video-assisted thoracoscopy and biopsy.

After induction of general anesthesia, a multiplane transesophageal echocardiography (TEE) probe was placed into the upper esophageal position. A large pericardial effusion was seen around the ascending aorta and no significant fluid could be detected in other regions of the pericardial space (Video 1; please see video available at www.anesthesia-analgesia.org). As the TEE probe was positioned at the midesophageal (ME) level, a 5.8 × 8.6 cm mass was found to occupy most of the right atrium (Fig. 1 and Video 2; please see video available at www.anesthesia-analgesia.org). This mass was heterogeneous in nature with multiple irregular echo lucent areas. Several mobile segments were also present and moved in concert with each cardiac cycle; the largest one measured approximately 2 cm (Fig. 1, Video 2; please see video available at www.anesthesia-analgesia.org). The mobile segments shared similar echocardiographic features to the rest of this mass. Color flow Doppler demonstrated blood flow inside the echo lucent areas possibly indicating a vascular nature to the mass (Fig. 2 and Video 3; please see video available at www.anesthesia-analgesia.org).

Figure 1

Figure 1

Figure 2

Figure 2

The ME four-chamber view, ME right ventricular (RV) inflow-outflow view, and ME bicaval views with clockwise rotation of the probe revealed invasion of the tricuspid valve annulus without any clear involvement of the rest of the RV, superior or inferior vena cava. This large mass significantly decreased the contractility of the right atrium and RV. The tricuspid annular plane systolic excursion measured 12 mm, which is much less than the normal 20 to 25 mm ranges, possibly due to infiltration of the tricuspid annulus by the mass. The TEE probe was then advanced into the stomach and anteflexed to obtain a transgastric tricuspid short axis view and transgastric RV inflow view to fully assess encroachment of the mass into right heart structures. No mass was seen in other cardiac structures. In addition, no patent foramen ovale, atrial septal defect, or ventricular septal defect was detected on TEE examination.

During thoracoscopy, several areas of pulmonary infarction were found in the lungs. Biopsy of the mass was difficult because of the extensive bleeding upon puncture of the mass and a thoracotomy was required to get adequate exposure. We were unable to visualize the needle itself on TEE because the needle was only allowed to penetrate the very superficial areas due to concerns for bleeding. The surgeon identified and sampled the less vascular areas of the mass mainly guided by the indentation from the needle exertion. TEE showed no new pericardial effusion after the biopsy. Pathology was consistent with angiosarcoma. He underwent a tumor resection and right atrial remodeling procedure 3 mo later. Unfortunately, he then developed a large intracranial metastatic lesion with a grim prognosis.

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DISCUSSION

TEE is a useful imaging modality for assessment of cardiac masses. High resolution and the proximity between the transducer and heart provide superior evaluation of the tissue characteristics of cardiac masses compared with transthoracic echocardiography. Scanning the right heart structures through 0 to 150° in the ME position and standard views described above allows the echocardiographer to further appreciate three-dimensional properties of this mass. Three-dimensional echocardiography might provide additional information regarding the exact location and structure of right atrial masses. Normal right atrial variants, including right atrial appendage, Eustachian valve, and lipomatous hypertrophy of the interatrial septum, are distinguished from pathology by their trabeculations, location along the inferior vena cava and position in the interatrial septum. Pathologies of the right atrium include thrombi, benign primary tumors, malignant primary tumors, secondary tumors, and invading tumors.1,2 Anatomic location, size, shape, mobility, and clinical history may offer important clues to the type of mass (Table 1). The mass in our patient could have been a tumor or thrombus. One of the major echocardiographic features of malignant cardiac tumors is the enhancement of the tumor with ultrasound contrast or demonstration of blood flow inside the tumor by color flow Doppler due to their extensive vascularity as illustrated in this case.

Table 1

Table 1

Angiosarcoma is the most common malignant tumor of the heart and is characterized by rapid growth, local invasion, and distant metastasis. Treatment of cardiac angiosarcoma is very challenging and includes chemotherapy, radiation, surgical resections, and even transplantation.3

In summary, TEE provided important information about the morphology and possible pathophysiology of angiosarcoma in this patient. Localized periaortic effusions seen on TEE reflected significant inflammation and scarring of surrounding tissues preventing free communication of the pericardial fluid. Mobile segments of the tumor might have showered the lungs with emboli and caused chest pain and hemoptysis. TEE also showed blood flow inside the mass, which served to confirm the vascular nature of the tumor.

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REFERENCES

1. Van der Heusen FJ, Stratmann G, Russell IA. Right ventricular myxoma with partial right ventricular outflow tract obstruction. Anesth Analg 2006;103:305–6
2. Burch TM, Davidson MF, Pereira SJ. Use of transesophageal echocardiography in the evaluation and surgical treatment of a patient with an aneurysmal interatrial septum and an intracardiac thrombus traversing a patent foramen ovale. Anesth Analg 2008;106:769–70
3. Kurian KC, Weisshaar D, Parekh H, Berry GJ, Reitz B. Primary cardiac angiosarcoma: case report and review of the literature. Cardiovasc Pathol 2006;15:110–12
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