Right-Side Dominant Cardiac Sarcoidosis Complicated With Multiple Coronary Artery-Right Ventricular Microfistulas

A 74-year-old man was referred to hospital with a complaint of chest discomfort. He had been histologically diagnosed with ocular and pulmonary sarcoidosis 6 years ago. Transthoracic echocardiography (TTE) at that time showed normal right (RV) and left ventricular (LV) function (Figure A), but 2 years before the index admission, coronary angiography (CAG) revealed multiple coronary artery-right ventricular microfistulas (CA-RVMF) from the right coronary artery (Figure B). On admission, RV and atrial dilation with severely reduced RV systolic function were observed on TTE (Figure C) and the left ventricular ejection fraction was mildly reduced (59%). Right heart catheterization revealed no pulmonary hypertension. Intriguingly, repeated CAG showed progression of CA-RVMF with a new fistula from the left coronary artery (Figure D). ¹⁸F-fluorodeoxyglucose positron emission tomography (FDG-PET) demonstrated FDG uptake in the RV free wall, right atrium, and left ventricle (Figure E). Together with positive findings on cardiac magnetic resonance imaging and gallium scintigraphy, and elevated levels of soluble interleukin-2 receptor and lysozyme, he was clinically diagnosed with cardiac sarcoidosis (CS). Repeat FDG-PET at 8 months after the initiation of corticosteroid therapy showed resolution of FDG activity. He has been free from symptoms.

Figure.

Normal findings on transthoracic echocardiography (TTE) 6 years ago (A). Multiple coronary artery-right ventricular microfistulas (CA-RVMF; arrows) from the RCA 2 years ago (B). Dilatation of the RV and RA with severely reduced RV systolic function (C) and progression of CA-RVMF with a new fistula from the LCA on admission (D). ¹⁸F-fluorodeoxyglucose uptake in the RV free wall, RA, and LV in the axial (Upper) and coronal (Lower) views (E). LA, left atrium; LCA, left coronary artery; LV, left ventricle; RA, right atrium; RCA, right coronary artery; RV, right ventricle.

This is the first report of right-side dominant CS complicated with CA-RVMF. Notably, the progression of RV dysfunction was accompanied with the development of CARVMF. The granuloma formation and subsequent neovascularization in the RV can cause CA-RVMF, which might be detectable by the combination of FDG-PET and CAG.¹ The impact of volume overload induced by CA-RVMF on RV function was considered to be small because the ratio of pulmonary-to-systemic-blood-flow was only 1.22.

Funging

None.

Reference

• 1.   Asakura K, Minami Y, Nagata T, Koitabashi T, Ako J. Cardiac sarcoidosis accompanied by a tumor-like blush on coronary angiography. J Cardiol Cases 2022; 26: 21–23.