Papillary fibroelastoma of the aortic valve--a case report and literature review

J Cardiothorac Surg. 2010 Oct 17:5:84. doi: 10.1186/1749-8090-5-84.

Abstract

The prevalence of primary cardiac tumour ranges from 0.0017-0.28% and papillary fibroelastoma is rare but not uncommon benign cardiac neoplasm. Currently, with the advent of higher-resolution imaging technology especially transoesophageal echocardiography such cases being recognized frequently. The clinical presentation of these tumours varies from asymptomatic to severe ischaemic or embolic complications. We herein, present a 50-year-old female patient with a papillary fibroelastoma of the aortic valve arising from the endocardium of the right coronary cusp very close to the commissure between the right and non-coronary cusps. The patient presented with angina-like chest pain and was investigated using echocardiography and CT angiographic modalities in addition to the usual investigations. The differential diagnosis considered was a thrombus, myxoma, Lambl's excrescence and infective vegetation. The surgical management included a prompt resection of the tumour on cardiopulmonary bypass avoiding injury to the aortic valve. The patient recovered well. A review of the literature suggests that the cardiac papillary fibroelastoma is a rare but potentially treatable cause of embolic stroke and other fatal complications, therefore, a strong suspicion; appropriate use of imaging modality, preoperative anticoagulation and urgent surgical resection is warranted. Also, possibility of this diagnosis should be kept in mind while managing cardiac or valvular tumours.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Aortic Valve*
  • Diagnosis, Differential
  • Female
  • Fibroma / diagnosis*
  • Fibroma / pathology
  • Fibroma / surgery
  • Heart Neoplasms / diagnosis*
  • Heart Neoplasms / pathology
  • Heart Neoplasms / surgery
  • Heart Valve Diseases / diagnosis*
  • Heart Valve Diseases / pathology
  • Heart Valve Diseases / surgery
  • Humans
  • Middle Aged