Prognostic and Bioepidemiologic Implications of Papillary Fibroelastomas | Journal Scan
What are the frequency and clinical implications of papillary fibroelastoma (PFE), either surgically removed or echocardiographically detected?
This was a single-center study from the Mayo Clinic spanning a 16-year period from 1995-2010. Pathologic and echocardiographic databases were queried for diagnosis of PFE. A total of 511 patients were identified including 185 with surgically removed, pathologically confirmed PFE (group 1), and 326 (group 2) with echocardiographic evidence of PFE, but no pathological proof. Group 1 was divided into patients for whom PFEs were removed at primary surgery for PFE (group 1A, n = 94, 51%, and group 1B, n = 91, 49%) with PFE removed at the time of nonrelated cardiac surgery. Clinical data depositories were queried for prior and subsequent neurological events (NEs) including transient ischemic attack (TIA) or stroke.
Over the 16-year study period, 511 patients were identified as having PFE (0.089% of all echocardiograms), and 254 were identified as having typical cardiac myxoma. PFE was noted on the aortic valve in 304 (59%), mitral valve in 64 (13%), tricuspid valve in 23 (4%), and on the pulmonary valve in 9 (2%), with the remaining 111 (22%) on nonvavlular cardiac surfaces. For the total cohort, prior NEs occurred in 183 patients, including 58 (32%) in group 1 and 125 (38%) in group 2. In group 2, valvular PFE was associated with only mild valvular stenosis or regurgitation in 92%, and 14% had more than one PFE. No echocardiographic characteristic of PFE was significantly associated with cerebrovascular accident (CVA) including size, mobility, or valvular location. For the total patient cohort, PFE size ranged from 1-40 mm. In group 1, postoperative follow-up was available for 1.6 years (median). Recurrent PFE was documented in three patients (1.6%). For group 1A, the native valve was preserved in 98% of cases. Postoperatively for group 1, risk of CVA was 2% at 1 year and 8% at 5 years (p = 0.003). In group 1, 10 CVAs occurred during follow-up compared to 4.1 expected CVAs based on an age- and gender-based reference. For group 2, 1- and 5-year CVA rates were 6% and 13%. There were 29 CVAs during follow-up versus a predicted 8.4 (p < 0.001). There were 121 patients in group 2 with a prior history of NEs, 72% of whom were taking anticoagulation at the time of PFE diagnosis, including warfarin in 22%, aspirin in 47%, clopidogrel in 1%, and dual antiplatelet therapy in 2%. There was no difference in occurrence of subsequent stroke between patients treated with anticoagulation and those not taking medication.
The authors concluded that PFE may be more common than cardiac myxoma, and for patients not undergoing surgical removal, the risk of CVAs is increased compared to reference populations.
This study from a large echocardiographic experience nicely documents the prevalence of PFE and suggests it is more common than myxoma, which previously had been considered the most common benign cardiac tumor. Note should be made that this is a single-center study for which there may be substantial referral bias. The most important message from this study is the embolic potential of PFE. While this is not a prospective case-controlled study, the observed rates of NEs following diagnosis of PFE clearly exceeded calculated reference rates for a representative population. Furthermore, thromboembolic protection with either Coumadin or antiplatelet agents did not appear to provide a protective effect against embolic events. An additional important observation is that there is no relationship between PFE size and patients presenting with and without NEs. (The authors actually document a smaller PFE size in those with NEs compared to those without. Whether this observation is causal or random effect is unclear.) Overall, this publication should serve as the standard reference for clinical management of patients with PFE.
Clinical Topics: Anticoagulation Management, Cardiac Surgery, Invasive Cardiovascular Angiography and Intervention, Noninvasive Imaging, Aortic Surgery, Interventions and Imaging, Echocardiography/Ultrasound
Keywords: Anticoagulants, Aortic Valve, Aspirin, Cardiac Surgical Procedures, Constriction, Pathologic, Diagnostic Imaging, Echocardiography, Epidemiology, Heart Neoplasms, Ischemic Attack, Transient, Mitral Valve, Myxoma, Pathology, Platelet Aggregation Inhibitors, Pulmonary Valve, Tricuspid Valve, Warfarin
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