Abstract
IgG4-related disease is an immune-mediated fibro-inflammatory disorder with multisystemic
involvement. Aortitis and peri-aortitis are the most common cardiovascular manifestations
of the disease. We present the case of a 65-year-old man with symptomatic severe aortic
stenosis and concomitant IgG4 aortitis. The diagnosis was confirmed by IgG4 serum
levels, positive positron emission computed tomography (PET-CT) scans, and pathology
from mediastinal dissection. Surgical aortic valve replacement (SAVR) was unfeasible
owing to extensive mediastinal fibrosis, and transcatheter aortic valve replacement
(TAVR) was successfully performed. As ascending aorta access for SAVR in IgG4 aortitis
with long-run fibrosis entails a high risk of mortality, TAVR could be considered
in certain suitable patients.
Résumé
La maladie liée aux IgG4 est un trouble fibro-inflammatoire à médiation immunitaire
qui touche plusieurs organes. L’aortite et la périaortite sont les manifestations
cardiovasculaires les plus courantes de la maladie. Nous présentons ici le cas d’un
homme de 65 ans atteint d’une sténose aortique grave symptomatique et d’une aortite
associée aux IgG4 concomitante. Le diagnostic a été confirmé par les taux sériques
d’IgG4, un résultat positif à l’examen de tomographie à émission de positrons assistée
par ordinateur et l’observation de la maladie lors de la dissection médiastinale.
La chi- rurgie de remplacement valvulaire aortique (CRVA) n’était pas possible en
raison de la fibrose médiastinale étendue, et une implantation valvulaire aortique
par cathéter (IVAC) a été effectuée avec succès. Comme l’accès à l’aorte ascendante
pour la CRVA dans les cas d’aortite associée aux IgG4 comportant une fibrose de longue
date entraîne un risque élevé de mortalité, l’IVAC peut être envisagée chez certains
patients.
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References
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- The enigmatic immunoglobulin G4-related disease and its varied cardiovascular manifestations.Heart. 2021; 107: 790-798
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Article info
Publication history
Published online: July 29, 2021
Accepted:
July 23,
2021
Received:
May 11,
2021
Footnotes
See page 1876 for disclosure information.
Identification
Copyright
© 2021 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.
