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Papillary fibroelastomas in 19 patients with hypertrophic cardiomyopathy undergoing septal myectomy

Gautam Kumar1, Ryan J Macdonald, Paul Sorajja

  • 1Division of Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota 55905, USA.

Journal of the American Society of Echocardiography : Official Publication of the American Society of Echocardiography
|May 26, 2010
PubMed

Insights

Papillary fibroelastomas (PFEs) in hypertrophic cardiomyopathy (HCM) patients frequently occur on the aortic valve or left ventricular outflow tract. These cardiac tumors may not be benign and warrant investigation in HCM patients with neurological events.

Area of Science:

  • Cardiology
  • Cardiac Pathology
  • Oncology

Background:

  • Papillary fibroelastomas (PFEs) are the third most common primary cardiac tumors.
  • PFEs can be associated with significant neurologic sequelae.
  • Endocardial trauma is a suspected factor in PFE pathogenesis.

Purpose of the Study:

  • To characterize papillary fibroelastomas (PFEs) in patients with hypertrophic cardiomyopathy (HCM).
  • To investigate the clinical sequelae associated with PFEs in HCM.
  • To analyze the occurrence and outcomes of PFEs in the context of HCM.

Main Methods:

  • Retrospective analysis of echocardiograms and medical records.
  • Study included 19 patients with HCM who underwent septal myectomy.
  • PFEs were identified preoperatively, perioperatively, and in patients with prior myectomy.

Main Results:

  • PFEs were detected in 5 patients preoperatively and 12 perioperatively.
  • The majority of patients (58%) had one PFE, with numbers ranging from 1 to over 40.
  • PFEs predominantly arose on the aortic valve (47%) and in the left ventricular outflow tract (42%).

Conclusions:

  • In HCM patients, 89% of PFEs were located on the aortic valve or left ventricular outflow tract, a higher incidence than previously reported.
  • Turbulent left ventricular outflow tract flow may predispose to PFE development in HCM.
  • PFEs in HCM patients may be associated with neurologic events and warrant investigation for unexplained neurological symptoms.
Abstract

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