Looking for the Right Side in Large Vessel Vasculitis

Introduction

Information about a real patient is presented in stages (boldface type) to an expert clinician (Dr Silva Marques), who responds to the information, sharing his reasoning with the reader (regular type). A discussion by the authors follows.

Patient presentation: A 76-year-old woman presents to her regular cardiology appointment reporting worsening fatigue during the previous 4 months. Her symptoms are progressive such that she can no longer take a bath or dress up without becoming symptomatic. She had previously had a slight limitation of physical activity (New York Heart Association functional class II). The patient’s most recent medical history is notable for the diagnosis of Takayasu arteritis (TA) 3 years before, involving the aorta, its main branches, and proximal pulmonary artery (PA). Supracoronary replacement of the ascending aorta and aortic valve replacement were performed for secondary symptomatic severe aortic regurgitation. At that time, enlargement of the pulmonary trunk and proximal right PA artery with a bovine pericardial patch was also performed because of supravalvular pulmonary stenosis. Histopathologic findings supported TA diagnosis. In addition, there was a 70% stenosis in the distal right brachiocephalic trunk and a 50% stenosis of the proximal left subclavian artery. Her medical history also includes hypertension, hyperlipidemia, hypothyroidism, glaucoma, and reactive depression. After surgery, the patient remained stable under immunosuppressive therapy that included azathioprine and prednisolone. Six months ago, she weaned off glucocorticoid therapy because of increased iatrogenic intraocular pressure. One month later, she was hospitalized for fever and raised levels of inflammatory markers. Endocarditis was suspected, but the transthoracic echocardiogram was nondiagnostic and the transesophageal probe could not progress because of anatomic constraints. Therefore, a fluorodeoxyglucose-positron emission tomography (FDG-PET)/computed tomography (CT) was performed that showed focal abnormal activity near the prosthetic valve, leading to the diagnosis of aortic prosthesis endocarditis. Although blood cultures were negative, antibiotics …