CASE REPORT article
Front. Cardiovasc. Med.
Sec. Cardiovascular Surgery
Volume 12 - 2025 | doi: 10.3389/fcvm.2025.1544902
This article is part of the Research TopicExploring New Frontiers in Heart Surgery: Case Studies 2025View all 7 articles
Complete atrioventricular block and left ventricular outflow tract obstruction as primary manifestations of immunoglobulin G4-related disease: A case report
Provisionally accepted
Yihua LIU*
Lucie SchnedeckerFlorian EggenspielerDamien MandryShirine MohamedDaniel GrandmouginJuan-Pablo Pablo Maureira- Centre Hospitalier Universitaire de Nancy, Nancy, France
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Background: Immunoglobulin G4 related disease (IgG4-RD) is a systemic immunologic fibroinflammatory condition that can affect a wide range of organ systems. We present a rare case in which the primary manifestations were complete heart block and left ventricular outflow tract (LVOT) obstruction due to involvement of the cardiac skeleton.Case presentation: A 66-year-old male presented with syncope. Evaluation revealed paroxysmal complete atrioventricular block (AVB) and LVOT obstruction caused by diffuse fibrous thickening of the aortic valve extending to the anterior mitral leaflet, with a maximal gradient of 70mmHg. A semi-urgent operation was performed, including aortic and mitral valve replacement with bioprostheses and implantation of a definitive epicardial pacemaker.Histopathologic examination suggested IgG4-RD; however, glucocorticoid therapy was initially withheld. Two months later, the patient developed recurrent AVB and pacemaker dysfunction. Salvage glucocorticoid therapy led to normalization of pacemaker thresholds.Conclusions: IgG4-RD may underlie valvulopathy and conduction disorders via lymphoplasmacytic infiltration and fibrosis of the cardiac skeleton. Surgical intervention and timely glucocorticoid therapy are associated with favorable outcomes.
Keywords: Immunoglobulin G4, Cardiac skeleton, Atrioventricular Block, Valvular prosthesis, Glucocorticoids
Received: 13 Dec 2024; Accepted: 02 Jun 2025.
Copyright: © 2025 LIU, Schnedecker, Eggenspieler, Mandry, Mohamed, Grandmougin and Maureira. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
* Correspondence: Yihua LIU, Centre Hospitalier Universitaire de Nancy, Nancy, France
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