A 66-year-old woman with a history of mitral valve replacement with a
Starr-Edwards ball valve 25 years ago was treated for refractory heart
failure but died of right heart failure. At autopsy, primary pulmonar
y artery sarcoma was found in the right ventricular outflow tract, mai
n pulmonary trunk, and bilateral pulmonary artery, and had invaded the
aortic arch. The pathohistologic diagnosis was osteosarcoma. Echocard
iography, chest computed tomography and right ventriculography perform
ed 1 year before death did not reveal the presence of a tumor in the p
ulmonary artery. The history of this patient shows that primary pulmon
ary artery sarcoma grows rapidly, with, in this case, the patient dyin
g within 1 year of its appearance.