Sorour, Ahmed A.

Background: Papillary fibroelastoma (PFE) is the most common benign cardiac tumor. Because PFEs have the potential to embolize, they typically are surgically excised. Prior studies have suggested that postoperative recurrence of PFE is rare or does not occur. Objectives: To determine the rate at which PFEs recurred after surgical removal and to identify any risk factors associated with recurrence. Methods: Patients with pathologically proven PFE were retrospectively identified from a single center. Patients were included in the study if they had an echocardiographic examination at least 1 year after surgery. Echocardiographic images obtained intraoperatively (after excision) and at dismissal were compared with those of the most recent examination to assess the possibility of PFE recurrence. Results: Ninety eight patients were identified. The mean (SD) duration of follow-up was 5.4 (3.7) years (range, 1-17 years). Twelve patients (12.2%) had echocardiographically proven PFE recurrence. Three patients had the recurrent lesion surgically re-excised, and pathologic analysis showed that 2 were recurrent PFEs and 1 was a Lambl excrescence. Clinical presentation of stroke or TIA was more common for the recurrence patients (for the first PFE) than for the nonrecurrence patients (83% vs 26%; P <.001). Conclusions: Contrary to published data, PFEs do recur after surgical excision. This emphasizes the importance of postoperative follow-up with transesophageal echocardiography for identifying recurrent masses. Table. Characteristics of Patients With Recurrent PFE Abbreviations: AV, aortic valve; LA, left atrium; LE, Lambl excrescence; LVOT, left ventricular outflow tract; MV, mitral valve; PV, pulmonary valve; RA, right atrium; RC, right cusp; TV, tricuspid valve. a Corresponds to the same location. b Corresponds to a different location .

Abstract Funding Acknowledgements Type of funding sources: None. Background Papillary fibroelastoma (PFE) is now regarded as the most common primary tumor of the heart. Although benign, they are clinically significant for their high risk of embolization. They are most commonly found on cardiac valves but can also be present on non-valvular endocardial surfaces. The aim of this study was to better characterize patients with left-sided non-valvular PFE and its clinical sequelae. Methods We retrospectively identified patients with pathology-proven PFEs at a single center between January 1995 and December 2018 (n = 279). Patients with left-sided non-valvular PFE were analyzed. Medical records were retrospectively reviewed for clinical characteristics and outcomes. In addition, intra-operative transesophageal echocardiograms were manually reviewed to estimate overall size and location. Results During the study period, we identified 37 patients with left-sided non-valvular PFE (mean age 61 ± 14 years; 62% females) (Table). PFEs were located on the left ventricle in 41%, left atrium in 35%, and left ventricular outflow tract in 24% of patients. Around a quarter of patients (27%) had a diagnosis of hypertrophic cardiomyopathy, 19% had prior cardiac surgery, and 27% had cancer diagnosed prior to PFE diagnosis. Transient ischemic attack or stroke was the presenting symptom in 22% of patients, myocardial infarction in 6% and peripheral embolization in 6%. Median maximal length for PFE on the left ventricle was 11.1 mm [3;18], on the left atrium 9 mm [2;25], and left ventricular outflow tract 8 mm [6;13]. A minority of patients (9/37 [24%]) had associated valvular PFE on the mitral valve and/or aortic valve (1 patient had both mitral valve and aortic valve PFE, 7 had aortic valve PFE and 1 had Mitral valve PFE). Only 13 patients had follow up transthoracic/transesophageal echocardiogram 1 year after PFE removal; 4/13 (31%) had documented PFE recurrence (3 PFE recurred in the same location as the original; 1 in a different location). Conclusion Left-sided non-valvular PFE is associated with thromboembolic events and at least in those that had follow-up echocardiograms, had a high recurrence rate. More studies are needed to evaluate the management of patients with asymptomatic PFE. Abstract Figure. Baseline Characteristics

Abstract Background Papillary fibroelastoma (PFE) is a rare benign cardiac tumor with embolic potential. It is most commonly found on cardiac valves but can also be present on non-valvular endocardial surfaces. We sought to better understand the average PFE growth and recurrence rates, and characteristics that might be associated with embolization. Purpose To examine growth and recurrence rates and factors associated with embolization in patients with pathology-proven PFE. Methods Pathology-proven PFEs from 279 patients were identified at a single center between January 1995 and December 2018,and those with at least two transesophageal echocardiograms (TEE) more than 30 days apart were analyzed (n=62). Medical records were retrospectively reviewed for clinical characteristics and outcomes. In addition, intra-operative TEEs were manually reviewed and compared to previous TEEs at our institution to estimate overall size, location, and average PFE growth rates. Results The TEEs from 62 patients with pathology-proven PFE (mean age 65±12 years, female 63%) were reviewed. PFE was discovered incidentally during cardiac surgery in 18% of patients. Most PFEs were located on the aortic valve (AV) (73%) followed by the mitral valve (MV) (16%). The majority of PFEs (71%) presented with a stalk versus a sessile configuration. Average maximal length for PFE (including stalk if present) on the AV was 8.5±3.3 mm, and on the MV 6.8±2.0 mm. The number of PFEs located in other locations was too small to be analyzed for growth. PFE growth varied depending on location and configuration. The PFE growth on the AV was an average increase of 0.47 mm/year, those on the MV was 0.115 mm/year. Valvular PFE was associated with significant functional valve abnormality in 15% of patients, and the abnormality was attributed to the PFE in 9% of patients. There were 25 patients (40%) who had a documented stroke/transient ischemic attack prior to PFE removal (46% located on AV, 44% on MV, 10% other locations). In addition, some patients had other documented embolic complications, such as myocardial infarction, amaurosis fugax, and peripheral embolization to the spleen and kidney. PFE size and location (up-/ downstream of the valve) were not predictive of cardiovascular thromboembolic events. At a median follow up of 2 years, PFEs recurred in the same location in 7.5% of the patients. Conclusion PFE is highly associated with thromboembolic events and seem to grow slowly, with an average growth rate of 0.47mm/year on AV and 0.115mm/year on MV. Growth rates for PFE have not been previously described. PFE can be safely excised with preservation of the native valve and recurs in 7.5% of patients, suggesting that close follow-up maybe warranted.