Cardiac Unknown. Case Presentation 68 year male with history of liver transplant 13 years back Presented with fatigue Underwent contrast enhanced CT (computed.

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Presentation transcript:

Cardiac Unknown

Case Presentation 68 year male with history of liver transplant 13 years back Presented with fatigue Underwent contrast enhanced CT (computed tomography) scan

Contrast enhanced axial CTSagittal reformatted image Findings: Extensive enhancing soft tissue masses surrounding the left anterior descending (LAD) (yellow arrow) and circumflex coronary (green arrow) arteries

2 Chamber long axis SSFP (steady state free precision) cine 4 Chamber SSFP cine

2 Chamber long axis SSFP (steady state free precision) cine Findings: MRI (magnetic resonance imaging) confirms CT findings and shows underlying aneurysmal dilatation of LAD (yellow arrow) 4 Chamber SSFP cine

3 chamber SSFP cine showing incidental significant aortic valve stenosis

Dynamic gradient perfusion images showing relatively increased perfusion as compared to normal myocardium

Pre and postcontrast non fat suppressed sagittal T1 weighted turbo spin echo images reveal heterogeneous enhancement of the soft tissue mass lesion

Imaging Findings Extensive soft tissue surrounding the left anterior descending and circumflex coronary arteries seen on CT and MRI images This soft tissue is enhancing on post contrast images Incidental aortic stenosis

Discussion/Teaching Points Enhancing soft tissue masses around the coronary artery is nonspecific in appearance Differentials include inflammatory disorder or neoplastic etiology like lymphoma Open biopsy was performed which revealed IgG4 related disease

Discussion/Teaching Points IgG4 is a chronic inflammatory disease characterized by IgG4 positive plasma cell infiltrate This type of inflammatory changes were first described histopathologically in autoimmune pancreatitis in 2001 Though IgG4 related disease occurs commonly in glandular and ductal tissues this entity is also discussed in association with vascular system

Discussion/Teaching Points IgG4 related inflammatory pseudotumor have been reported involving coronary artery Coronary artery aneurysm formation is also described with IgG4 related disease Recent reports suggested that there may be a role of IgG4 related inflammatory reactions in coronary atherosclerosis development leading to coronary stenosis

References Hamano H, Kawa S, Horiuchi A, et al. High serum IgG4 concentrations in patients with sclerosing pancreatitis. N Engl J Med 2001;344: Sugimoto T, Morita Y, Isshiki K et al. Constrictive pericarditis as an emerging manifestation of hyper-IgG4 disease. Int J Cardiol 2008; 130:e100. Matsumoto Y, Kasashima S, Kawashima A, Sasaki H, Endo M, Kawakami K, Zen Y, Nakanuma Y. A case of multiple immunoglobulin G4-related periarteritis: a tumorous lesion of the coronary artery and abdominal aortic aneurysm. Hum Pathol Jun;39(6): Nobukazu Ishizaka. IgG4-related disease underlying the pathogenesis of coronary artery disease. 2013, Clinica Chimica Acta 415, 220–225.

Coronary wrap IgG4 disease – inflammatory pseudotumor Dhiraj Baruah, MD Jason Rubenstein, MD Kaushik Shahir, MD Department of Radiology Medical College of Wisconsin