Immunoglobulin G4-related disease of the thickened aortic valve extending to the left ventricular outflow tract causing severe aortic regurgitation and complete atrioventricular block: a case report

Background Immunoglobulin G4-related disease (IgG4-RD) is a systemic disease characterized by the tumefactive lesions and infiltration of IgG4-positive plasma cells. IgG4-RD has been described in various organs, but rarely the aortic valve. There are only a few reports of aortic stenosis, and none on significant aortic regurgitation. In addition, previous case reports relating to aortic valve lesions led to surgery as a first-line treatment. The effect of steroid treatment has not yet been determined. Case Summary A 62-year-old man, receiving steroid therapy, who presented with general malaise, shortness of breath, and bradycardia. He had suspected IgG4-RD because of pancreatitis, lacrimal gland enlargement, and retroperitoneal fibrosis. An examination revealed a thickened aortic valve extending to the left ventricular outflow tract with severe aortic regurgitation and complete atrioventricular block. He received intensive steroid therapy for a suspected IgG4-related aortic valve lesion. The complete atrioventricular block improved, but worsening aortic regurgitation caused congestive heart failure. He required replacement of the aortic valve. A histopathological examination of the excised aortic valve leaflets revealed IgG4-positive lymphoplasmacytic infiltration with fibrotic tissue. The prosthetic valve was functioning well without leakage around the valve at the 1-year follow-up. Discussion This case highlights the rare possibility that IgG4-RD of the aortic valve also causes significant aortic regurgitation. Conservative treatment with steroids may induce regression of the lesion and contribute to the stability of the prosthetic valve after surgery, but it may also exacerbate heart failure due to the progression of aortic regurgitation in patients with aortic valve lesions.