Papillary fibroelastomas in 19 patients with hypertrophic cardiomyopathy undergoing septal myectomy

Abstract

Background: The aim of this study was to characterize papillary fibroelastomas (PFEs) and their clinical sequelae in patients with hypertrophic cardiomyopathy (HCM). PFE is the third most common primary cardiac tumor and can be associated with neurologic events. Because endocardial trauma has been implicated in its pathogenesis, the occurrence and outcomes of PFEs in patients with HCM were retrospectively analyzed in this study.

Methods: Echocardiograms and medical records were reviewed for characteristics of PFEs in 19 patients with HCM who underwent septal myectomy.

Results: PFEs were detected preoperatively by transthoracic echocardiography in 5 patients, by transesophageal echocardiography in 2 patients, and perioperatively in 12 others. Three patients had previously undergone septal myectomy. Although the majority of patients (n = 11 [58%]) had 1 PFE, the number ranged from 1 to >40 (median, 1.0). PFEs arose on the aortic valve (47%), in the left ventricular outflow tract (42%), and in the right heart (11%).

Conclusions: In this study of patients with HCM, 89% of PFEs were located on the aortic valve or in the left ventricular outflow tract compared with about 40% to 50% in prior studies of PFEs in general. This suggests that endocardial trauma due to turbulent left ventricular outflow tract flow may predispose to PFE development. Because of the high incidence of neurologic events among patients with HCM, PFEs may not be innocuous and should be searched for in patients with HCM and unexplained neurologic events.