Abstract
This study presents an unusual manifestation of Takayasu arteritis in a 16-year-old girl with significant left main and right coronary artery vasculitis. The distinct clue on the diagnosis was cardiac magnetic resonance findings of increased periaortic tissue enhancement in late gadolinium enhancement sequences. Cardiac magnetic resonance has high accuracy in the diagnosis of patients with Takayasu arteritis.
Key Words: aorta, cardiac magnetic resonance, computed tomography, magnetic resonance sequences, myocardial infarction, Takayasu arteritis
Graphical abstract
A 16-year-old girl presented to our emergency department with heavy chest pain and shortness of breath. She had no previous medical history, and the physical examination was unremarkable except for mild sinus tachycardia. Electrocardiogram revealed ST-segment elevation in the anterior leads and an elevated cardiac troponin level (8.37 μg/L). Echocardiography showed a left ventricular ejection fraction of 45% to 50% with hypokinesia in the left ventricular anterior wall. Because of the patient’s age, an emergency cardiac computed tomography (CT) angiography was acquired, which revealed a significant ostial lesion of the left main and right coronary artery (Figures 1A and 1B, Video 1).
Figure 1.
Multimodality Imaging Cardiac Imaging in an Adolescence With Significant Left Main Lesion
Cardiac computed tomography angiography shows severe left main (A) and right coronary artery ostial (B) narrowing (arrows). Angiography shows significant left main narrowing (C) and then stenting (D) (arrows). (E) Cardiac magnetic resonance late gadolinium enhancement depicts periaortic enhancement (arrow). (F) Cardiac computed tomography angiography re-evaluation shows increased periaortic tissue thickness (arrow).
The patient was delivered to the catheterization laboratory and underwent left main stenting (Ultimaster 4 × 15 mm, Terumo) via radial access (Figures 1C and 1D, Video 2). A rheumatologic consult was requested because of the patient’s age and coronary involvement. On further evaluation, she had a history of loss of appetite during the past year, which resulted in weight loss. However, there was no evidence of joint or musculoskeletal pain, fever, skin rash, photosensitivity, mouth or genital sores, eye problems, bloody sputum, sinusitis, or Raynaud’s phenomenon.
The patient’s medical history did not show evidence of previous Kawasaki disease. Peripheral pulses were symmetrical, without swelling or tenderness on the carotid arteries. Further laboratory evaluation revealed anemia (hemoglobin 10 g/dL) with a high erythrocyte sedimentation rate (50 mm/h). Results of blood cultures and a complete rheumatology panel, including anti-nuclear antibody, double-stranded DNA, antineutrophilic cytoplasmic antibody, lupus anticoagulant, anticardiolipin, HLA-B27, HLA-B51, rheumatoid factor, complement level, and COVID-19, were negative. There was no evidence of endocarditis, brucellosis, or tuberculosis. Pulmonary high-resolution CT and abdominal CT imaging without contrast showed no pathologic findings. Cardiac magnetic resonance indicated active aortic root and periaortic tissue inflammation with significant enhancement in the late gadolinium enhancement sequences (Figure 1E, Video 3). Aortic branches show no evidence of stenosis.
Re-evaluation of the CT angiography images revealed increased periaortic tissue thickness (Figure 1F, Video 4). Overall, the findings were in favor of Takayasu arteritis. The patient was treated with a 3-day pulse of methylprednisolone followed by oral steroids and the biological drug adalimumab. We visited the patient after 1 month, and she declared no chest pain or dyspnea. We scheduled close follow-up clinical and imaging visits for the patient.
This report signifies the vital role of cardiac imaging in patients with suspicious vasculitis.
Takayasu arteritis is a type of vasculitis involving large and medium-sized vessels, cardiac valves, myocardium, and coronary arteries, and it seems that immune-mediated inflammation has a central role.1 Early manifestations of acute Takayasu arteritis vary from the chronic phase, comprising only nonspecific constitutional symptoms, which can be misleading.2 Magnetic resonance imaging, especially late gadolinium enhancement images, are valuable at this early stage and may show enhancement of periaortic tissue or aortic branches.3 In some studies, magnetic resonance imaging showed a high accuracy in depicting aortic involvement compared with gold standard angiography.1
Funding Support and Author Disclosures
The authors have reported that they have no relationships relevant to the contents of this paper to disclose.
Footnotes
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Appendix
For supplemental videos, please see the online version of this paper.
Appendix
Angiography depicts significant left main narrowing.
Angiography shows left main stenting.
Late gadolinium enhancement series show significant aortic root enhancement.
Axial computed tomography angiography shows significant left main and right coronary artery narrowing and periaortic thickening.
References
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Associated Data
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Supplementary Materials
Angiography depicts significant left main narrowing.
Angiography shows left main stenting.
Late gadolinium enhancement series show significant aortic root enhancement.
Axial computed tomography angiography shows significant left main and right coronary artery narrowing and periaortic thickening.