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(Circulation. 2001;103:e99.)
© 2001 American Heart Association, Inc.

Images in Cardiovascular Medicine

Syncope in a Patient With Spindle Cell Sarcoma of the Main Pulmonary Artery

Bernardo Treistman, MD; Robert J. Card, MD; Tomas Klima, MD; Scott Flamm, MD

From the Department of Cardiology, St Luke’s Episcopal Hospital, Texas Heart Institute, Houston, Tex.

Correspondence to Bernardo Treistman, MD, St Luke’s Medical Towers, 6624 Fannin, Suite 2590, Houston, TX 77030.

A 44-year-old man was hospitalized after a syncopal episode. A thrill and loud systolic ejection murmur were present over the left border of the sternum. A 12-lead ECG and chest x-rays were normal. A 2D echocardiogram and Doppler studies documented normal cardiac chambers and a peak systolic pulmonary transvalvular gradient of 80 mm Hg. Biplane right ventriculography revealed a large mobile mass in the main pulmonary artery extending into the right ventricular outflow tract (Figure 1). Cardiac MRI showed the mass within the same area (Figure 2 and Figure I, which can be found at www.circulationaha.org). The patient underwent surgical resection of a large tumor in the main pulmonary artery (Figure 3). The tumor extended into the right pulmonary artery. The pulmonary valve was replaced with a homograft, and an endarterectomy of the main and right pulmonary arteries was accomplished. Histological examination revealed a spindle cell sarcoma (Figures 4 and 5). The patient remained asymptomatic and had no cardiac murmurs 8 months after surgery.¹

View larger version (140K): [in this window] [in a new window]   Figure 1. Biplane right ventriculography reveals a large mobile mass in the main pulmonary artery that extends into the right ventricular outflow tract (arrow).

View larger version (133K): [in this window] [in a new window]   Figure 2. MRI taken before (left) and after (right) administration of gadolinium. Images were obtained in the right ventricular outflow tract projection. A large, enhancing mass (arrows) is present in the main pulmonary artery and upper outflow tract.

View larger version (134K): [in this window] [in a new window]   Figure 3. Surgical resection of the large tumor in the main and right pulmonary arteries.

View larger version (165K): [in this window] [in a new window]   Figure 4. The tumor shows features of highly pleomorphic cell malignant neoplasm, with numerous . . . [Full Text of this Article]