Successful Transcatheter Diagnosis and Medical Treatment of Right Atrial Involvement in IgG4-related Disease

International Heart Journal
Toshiyuki YanoTetsuji Miura

Abstract

IgG4-related disease (IgG4-RD) is a fibro-inflammatory disorder characterized by lymphoplasmacytic infiltration of numerous IgG4-positive plasma cells, leading to fibrous thickening in the affected tissue. Typical cardiovascular manifestations of IgG4-RD are periaortitis, coronary arteritis, and pericarditis. Rare cases of myocardial involvement in IgG4-RD have been reported, but surgical resection or open biopsy was required for the diagnosis in those cases. Here, we report a case in which percutaneous transcatheter biopsy under the guidance of intracardiac echocardiography was useful for diagnosis of IgG4-RD manifested as an intracavitary right atrial mass, extending into the superior vena cava. Successful transcatheter diagnosis of myocardial involvement of IgG4-RD led to immediate favorable response to steroid therapy. Including the present case, previous IgG4-RD cases with myocardial involvement are reviewed to delineate its clinical characteristics.

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Citations

Sep 28, 2018·International Heart Journal·Nobukazu Ishizaka
Sep 9, 2020·Circulation. Heart Failure·Jamie E DiamondEdward Lin
Apr 21, 2021·Journal of Cardiothoracic and Vascular Anesthesia·Nami KondoSusumu Ide
Oct 8, 2021·Internal Medicine·Shun NomuraTatsuya Usui

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