Artunduaga Maddy,  Jadhav Siddharth,  Ketwaroo Pamela,  Masand Prakash

Final Pr. ID: Poster #: CR-006

Papillary fibroelastomas are benign primary cardiac tumors that usually arise from the valve apparatus and are rare in the pediatric population. Involvement of the tricuspid valve is even rarer, with only a few cases reported in the literature. We present the case of an 11-month-old female referred by her pediatrician after a non-cardiovascular acute visit to investigate a murmur noted since birth. The patient was asymptomatic. Family history was negative for congenital heart disease, seizures or genetic disorders. Her physical exam revealed a soft holosystolic murmur at the left lower sternal border but was otherwise normal. Initial work up with ECG and echocardiogram revealed limb lead reversal and a large mass in the right ventricle. Subsequent MRI showed a mobile 15mm oval mass along the septal surface of the right ventricle, in close proximity to the tricuspid valve, without valve inflow or right ventricular outflow tract obstruction. The mass was faintly visualized on TFE, isointense on T1, centrally-hyperintense and peripherally-hypointense on T2, and peripherally enhancing on post-contrast images. After a multidisciplinary review of the case, the decision was made to proceed with surgical resection. A preoperative CT of the chest did not reveal pulmonary embolism. During surgery, the mass was attached to the septal leaflet and the chordae of the tricuspid valve but not to the septum. Visually, the mass was compatible with a fibroelastoma. Off-bypass intraoperative transesophageal echocardiogram showed moderate tricuspid regurgitation that required on-bypass annuloplasty. Pathology confirmed the diagnosis of papillary fibroelastoma. There were no surgical complications. She was discharged on postoperative day 6 and had been doing well until her most recent follow-up four months after surgery. Papillary fibroelastomas are typically asymptomatic, although they carry potential risks for complications such as pulmonary or systemic emboli, depending on their location. MRI is a valuable examination that can characterize intracardiac tumors and thrombi. To our knowledge, this is the first report of a pathology-proven papillary fibroelastoma of the tricuspid valve characterized by MRI in such a young child Read More

Authors:  Artunduaga Maddy , Jadhav Siddharth , Ketwaroo Pamela , Masand Prakash

Keywords:  Papillary fibroelastoma, MRI, intracardiac mass