Papillary fibroelastomas are the second most common primary cardiac tumors. They can cause symptoms due to embolic phenomena or obstruction. We present a 60-year-old woman with new-onset atrial fibrillation along with complaints of shortness of breath and chest pressure. Her transesophageal echocardiogram and computed tomography angiogram revealed a mass (10 x 8 mm) involving the left coronary sinus and causing intermittent obstruction of the left main coronary artery. We proceeded with surgical removal of the mass due to its peculiar location, size, and hypermobility. Histopathology diagnosed the mass as papillary fibroelastoma. This case is unique in its location, invaginating into the left main coronary artery and causing obstruction. We also discuss the current treatment options and need of randomized studies for further recommendations.
A 60-year-old Caucasian woman was referred for evaluation of new-onset atrial fibrillation. Her complaints included shortness of breath on exertion and chest pressure. On examination, her heart rate was 90 beats per minute, irregular and without any murmurs. She was started on warfarin and underwent nuclear perfusion scan, which revealed reversible ischemia in the anterolateral myocardial walls. She was admitted to the hospital for transesophageal echocardiogram-guided cardioversion followed by elective cardiac catheterization. Her transesophageal echocardiogram showed an echogenic, pedunculated mobile mass located at the junction of the left coronary sinus and the root of the aorta. This mass appeared to be obstructing the left main coronary artery intermittently (see Figure 1). Cardiac computed tomography confirmed the findings of the transesophageal echocardiogram (see Figure 2). The cardiothoracic team was consulted for excision of this mass. Intra-operatively, a pedunculated mass was noted, attached to the free surface of the aorta in close proximity to the commissure between the left and non-coronary cusps. Gross examination revealed a gray-tan, polypoid mass that measured 14 x 10 x 8 mm with numerous fine papillations covering the tissue. Histology showed papillary fronds with a myxoid stroma, lined by benign endothelial cells (see Figure 3). These features led to a diagnosis of papillary fibroelastoma. Her post-operative recovery was uneventful and she was discharged home on the seventh day of hospitalization.
- Reynen K, Frequency of primary tumors of the heart, Am J Cardiol, 1996;77:107.
- Loire R, Donsbeck AV, Nighoghossian N, et al., Papillary fibroelastoma of the heart. A review of 20 cases, Arch Anat Cytol Pathol, 1999;47:19–25.
- Shahian DM, Labib SB, Chang G, Cardiac papillary fibroelastoma, Ann Thorac Surg, 1995;59(2):538–41.
- Ryan PE Jr, Obeid AI, Parker FB Jr, Primary cardiac valve tumors, J Heart Valve Dis, 1995;4(3):222–6.
- Lembcke A, Meyer R, Kivelitz D, et al., Images in cardiovascular medicine. Papillary fibroelastoma of the aortic valve: appearance in 64-slice spiral computed tomography, magnetic resonance imaging, and echocardiography, Circulation, 2007;115:e3–e6.
- Gowda RM, Khan IA, Nair CK, et al., Cardiac papillary fibroelastoma: a comprehensive analysis of 725 cases, Am Heart J, 2003;146(3):404–10.
- Sun JP, Asher CR, Yang XS, et al., Clinical and echocardiographic characteristics of papillary fibroelastomas: a retrospective and prospective study in 162 patients, Circulation, 2001;103(22):2687–93.