JACC: Case Reports (Oct 2023)

Severe Aortic Valvular Incompetence From IgG4-Related Disease

  • Bing Wei Thaddeus Soh, MB,
  • Sophie Catherine Prendergast, MB,
  • Louise Burke, MD,
  • Cormac T. O’Connor, MB,
  • Ihsan Ullah, MBBS,
  • Julie M. O’Brien, MB,
  • John A. Hinchion, MB,
  • Samer Arnous, MD,
  • Thomas J. Kiernan, MD

Journal volume & issue
Vol. 24
p. 102027

Abstract

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IgG4-related disease (IgG4-RD) is a new clinical entity characterized by lymphoplasmacytic lesions rich in IgG4-positive plasma cells. Myocardial involvement is extremely rare and not a typical cardiovascular manifestation of IgG4-RD. We report a rare case of IgG4-RD–associated myocardial mass causing severe aortic incompetence, successfully treated with surgery and corticosteroids. (Level of Difficulty: Intermediate.)

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