Severe Aortic Valvular Incompetence From IgG4-Related Disease: An Unusual Entity

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

Research output: Contribution to journalArticlepeer-review

Abstract

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.)

Original languageEnglish
Article number102027
Pages (from-to)102027
JournalJACC: Case Reports
Volume24
DOIs
Publication statusPublished - 18 Oct 2023
Externally publishedYes

Keywords

  • cardiac mass
  • IgG4-RD
  • IgG4-related disease
  • multimodal imaging

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