Shortness of Breath, Malaise, and Pulmonary Artery Hypertension

The patient is a 40 year old man with a history of mitral valve prolapse and vague chest pain for the last several years. At the time MVP was diagnosed 5 years ago, he had a negative stress echocardiogram and completed 13 minutes of exercise without chest pain or inducible wall motion abnormalities. He is a non-smoker and has no other risk factors for coronary artery disease. His only medication was intermittent aspirin. There was no history of rheumatic fever or valvular heart disease.

Recently, he presented to the office with complaints of exertional breathlessness and occasional chest tightness. This was also associated with a 15 pound weight loss.

Transthoracic and Transesophageal (TEE) echocardiography showed a mass in the left atrium. His work-up ultimately showed a left atrial mass protruding from the left upper pulmonary vein into the left atrium, left atrial appendage and base of the posterior mitral valve leaflet. By echo Doppler the pulmonary artery pressure was severely elevated into the 60-70 range.

Cardiac catheterization shown above demonstrated normal coronary arteries. However, a 'tumor blush' is seen during injection of the left main coronary artery indicative of the extensive vascularity of the tumor. CT of the abdomen, brain and pelvis was within normal limits, showing no evidence of metastatic disease.

The patient underwent sternotomy with complete resection of the tumor, but the surgery was complicated by the development of severe mitral regurgitation requiring a bioprosthetic mitral valve replacement.

Pathology:

Surgical pathology showed poorly differentiated angiosarcoma of the heart.

This case demonstrates a very unusual finding. This patient presented with a very rare tumor which presented clinically as dyspnea on exertion. His work up showed severe pulmonary hypertension ultimately related to obstruction of left atrial emptying because of tumor involvement extending from the left pulmonary vein and involvement of the mitral valve apparatus. Functionally, the symptoms were consistent with mitral stenosis.