Echocardiographic and Doppler Findings in Primary Sarcoma of the Pulmonary Artery

Primary sarcoma of the pulmonary artery is a rare heart tumor. In the reported case, the clinical findings were nonspecific, and were characterized by progressive dyspnea, fever, and a systolic murmur in the pulmonary area. Echocardiographic examination showed an echogenic mass partially obstructing the pulmonary artery trunk, dilation of the right cardiac chambers, and a pressure overload pattern. Doppler and color Doppler demonstrated a high-velocity systolic flow jet in the pulmonary artery due to obstruction of the vessel by the tumor, as well as severe high-velocity tricuspid regurgitation. The patient died suddenly soon thereafter. The autopsy confirmed almost total occlusion of the pulmonary artery by a neoplastic mass. Histopathological diagnosis was primary vascular leiomyosarcoma of the pulmonary artery. In addition, a large tumor embolus had occluded the right pulmonary artery. Doppler echocardiography proved useful in noninvasively recognizing the sarcoma of the pulmonary artery, and explaining the clinical picture and hemodynamic derangements produced by this tumor. This could, in other patients, allow an early diagnosis and timely surgical intervention.