Inflammatory myofibroblastic tumor of the aortic valves causing sudden cardiac death: a case report and review of the literature

Pediatr Dev Pathol. 2014 May-Jun;17(3):231-9. doi: 10.2350/13-12-1414-CR.1. Epub 2014 Mar 20.


Cardiac inflammatory myofibroblastic tumor (IMT) is a rare entity affecting predominantly infants, children, and young adults. Although most tumors have a benign clinical course after complete surgical resection, some have significant clinical effects. We report the case of a 9-year-old girl who had sudden cardiac death as a result of occlusion of the left circumflex coronary artery. A review of 57 cases of cardiac IMTs reported in the literature in terms of epidemiology, clinical presentation, histologic and immunohistologic features, and outcome is presented. Recognition of this rare abnormality is important in order to initiate prompt surgical intervention.

Keywords: autopsy; cardiac inflammatory myofibroblastic tumor; pediatric; sudden cardiac death.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Aortic Valve / pathology
  • Bicuspid Aortic Valve Disease
  • Child
  • Death, Sudden, Cardiac / etiology*
  • Death, Sudden, Cardiac / pathology
  • Female
  • Granuloma, Plasma Cell / complications*
  • Granuloma, Plasma Cell / pathology
  • Heart Defects, Congenital / complications*
  • Heart Defects, Congenital / pathology
  • Heart Valve Diseases / complications*
  • Heart Valve Diseases / pathology
  • Humans