Revista Española de Cardiología (English Edition) Revista Española de Cardiología (English Edition)
Rev Esp Cardiol. 2018;71:112 - Vol. 71 Num.02 DOI: 10.1016/j.rec.2017.05.016

Multimodality Imaging for Pulmonary Valve Papillary Fibroelastoma

Albert Teis a,, Xavier Saenz-Sardà b, Xavier Ruyra c

a Servicio de Cardiología, Hospital Universitari Germans Trias i Pujol, Badalona, Barcelona, Spain
b Servicio de Anatomía Patológica, Hospital Universitari Germans Trias i Pujol, Badalona, Barcelona, Spain
c Servicio de Cirugía Cardiaca, Hospital Universitari Germans Trias i Pujol, Badalona, Barcelona, Spain

Article

A 45-year-old man with Crohn disease was referred for colectomy. Preoperative chest X-ray showed a right pulmonary hilum nodular image that prompted a thoracic computed tomography scan. Incidentally, a round mass was detected in the main pulmonary artery (Figure, asterisk) and was further confirmed on computed tomography-angiography (Figure; arrows; PA, pulmonary artery; RV, right ventricle). The patient denied prior chest pain, palpitations, dyspnea, or fever. Transthoracic and transesophageal echocardiography identified a round mass attached to the arterial side of the pulmonary valve, with no valve regurgitation. A magnetic resonance scan confirmed the presence of a 12-mm diameter mobile mass attached to the pulmonary valve, which was isointense on T1-weighted images, highly hyperintense on T2, and was not perfused but was enhanced 10 minutes after gadolinium administration (Figure, arrows; Video 1 of the supplementary material). Thus, the differential diagnosis between pulmonary valve papillary fibroelastoma and myxoma was established. Cardiac surgery was performed 1 month after colectomy. A well-defined round mucinous mass attached to the tip of the pulmonary valve was easily excised, with valve sparing (Figure; Video 2 of the supplementary material). It consisted of an elongated and branching papillary proliferation covered by a single layer of cells (Figure) on hematoxylin-eosin stain, which expressed endothelial cell markers (Figure) on immunohistochemistry for erythroblast transformation-related gene, thus allowing diagnosis of papillary fibroelastoma.

Figure.

Papillary fibroelastomas are common, but not in a pulmonary position. The differential diagnosis with myxoma can be difficult, mainly if the masses are round-shaped. Magnetic resonance findings facilitate the diagnosis of a wide range of masses.

SUPPLEMENTARY MATERIAL

Video 1

Video 2

Corresponding author: a_teis@yahoo.es

1885-5857/© 2018 Sociedad Española de Cardiología. Published by Elsevier España, S.L.U. All rights reserved

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