Abstract 3261: Papillary Fibroelastomas in Hypertrophic Cardiomyopathy
Introduction: Papillary fibroelastomas (PFEs) are the 3rd most common primary cardiac tumors with a prevalence of around 0.01% in patients with echocardiograms. The pathogenesis is controversial but endocardial trauma has been implicated. We hypothesized that increased turbulence in hypertrophic cardiomyopathy (HCM) would be a risk factor for PFE development and characterized PFEs in HCM. Around 5% of HCM patients had neurologic events. We postulated that patients with both PFE and HCM had a higher risk of neurologic events.
Methods: We reviewed pathology records from 1990 –2006 and identified 129 subjects with PFEs and 2479 patients with HCM. We then identified the subjects who had both HCM and PFE (n = 19). All subjects signed release for use of their medical records for research.
Results: 19 subjects (68.4% female) had both HCM and PFE. Mean age at diagnosis of HCM was 49 (range 21– 81) years. All had outflow tract obstruction with resting gradient >/=30 mm Hg except 2 who had resting gradients <30 mm Hg but elevated gradients on provocation. PFE diagnosis was after a mean of 9.7 years (SD 8.3) following HCM diagnosis. 5 subjects were diagnosed pre-operatively by transthoracic echocardiography, 2 subjects by transesophageal echocardiography and 12 subjects at surgery only. Most subjects had only 1 PFE (57.9%) but 1 subject had as many as 40 tumors. Most PFEs were on the aortic valve (47.4%) and the left ventricular side of the septum and left ventricular outflow tract (42.1%). Only 2 patients had right heart lesions that were presumably unrelated to HCM. 3 patients had previous septal myectomies and all 3 had PFEs near the previous resection. 5 of 19 subjects (26.3%) with PFE and HCM had neurologic events (either strokes or transient ischemic attacks) compared to 199 of 2261 subjects (8.8%) with HCM alone (OR = 4.1; 95% CI 1.4 –11.4, p < 0.05).
Conclusion: Nearly 90% of PFEs in HCM are located in the septum, left ventricular outflow tract and aortic valve. This may be supportive of endocardial trauma as a risk factor for PFE development in HCM. About a quarter of patients with PFEs and HCM had neurologic events. This may indicate that these lesions are not innocuous and should perhaps be actively searched for in patients with HCM and unexplained neurologic events.