Transesophageal Echocardiographic Detection of Papillary Fibroelastoma in the Pulmonary Artery

Abstract

A 57-yr-old man presented for excision of a pulmonary artery (PA) mass after a 5-mo history of productive cough and wheezing. Computerized tomography scan revealed a large, stable, eccentric, filling defect within the main and left PA. Positron emission tomography demonstrated a single, intensely hypermetabolic focus in the PA region. Preoperative transthoracic echocardiography showed no abnormalities. After the induction of anesthesia, transesophageal echocardiography (TEE) examination was performed, revealing normal left ventricular systolic function and mild enlargement of the right ventricle. The main PA was dilated, with a mass extending from the main PA into the left main PA (Fig. 1). Close inspection of the pulmonary valve (PV) revealed a previously undetected small mobile lesion, independent of the PA mass, and not associated with PV restriction (Fig. 2 and video loop (available at www.anesthesia-analgesia.org). After cardiopulmonary bypass, a longitudinal incision along the PA exposed both the PA mass and the PV lesion. The PA mass nearly occluded the main PA and extended into the left main PA. Partial resection of the mass was performed, and a freestyle graft was used to repair the PA. Pathology examination indicated a spindle cell sarcoma. The PV lesion was excised and the valve repaired; microscopic examination identified a papillary fibroelastoma. Intensive care and hospital stay were uncomplicated.Figure 1.: Upper esophageal short axis view demonstrating the tumor (T) in the main pulmonary artery (PA). Right ventricle (RV) and aortic valve (AV) are also seen in this view.Figure 2.: Upper esophageal short axis view demonstrating the pulmonary valve fibroelastoma (PVFE). Right atrium (RA) and left atrium (LA), right ventricle (RV), and aortic valve (AV) are also seen in this view.In this case, the finding of the fibroelastoma of the PV during TEE examination was unexpected based on preoperative diagnostic evaluation, including transthoracic echocardiography. Before the advent of TEE, these rare, benign cardiac tumors were largely found incidentally during cardiac surgery or on necropsy. Transthoracic echocardiography is not usually effective for identification of such lesions (1). Fibroelastomas typically originate from valvular tissue, rather than myocardium. The differential diagnosis of a PV mass includes vegetation, thrombus, myxoma, and fibroelastoma. Definitive diagnosis is made only by histologic examination (2). Although fibroelastomas are typically benign, their presence increases the risk of embolic events, including myocardial infarction, pulmonary emboli, stroke, and even sudden death. The independent predictor of death or nonfatal embolization is tumor mobility (3). Treatment of choice is excision via surgical shaving and repair or replacement of the affected valve (3). In this case, the TEE allowed for the identification of the previously undiscovered fibroelastoma on the PV and assessment of the spindle cell sarcoma in the PA.