A. Short axis (SA), horizontal long axis (HLA), and vertical long axis (VLA) 99mTc-sestamibi myocardial perfusion SPECT and FDG PET imaging showing a medium-sized perfusion defect in the apical anterior, septal, and inferior walls, as well as the left ventricular apex. There is also a small-sized perfusion defect in the basal inferolateral wall (arrows). There is FDG uptake in the apical lateral wall and the mid and basal anterolateral walls (arrows). While this pattern of FDG uptake is generally considered a normal variant, its association with perfusion defects in the absence of obstructive CAD was deemed abnormal and categorized as CS probable.
B. Coronal PET (left) and PET/CT whole body FDG imaging showing anterolateral wall myocardial uptake, and the absence of abnormal extracardiac FDG uptake.
C. Gross photograph of four chamber view of the explanted heart showing fatty replacement of the right ventricular free wall characteristic of AC (arrow). An automatic implantable cardioverter-defibrillator lead is seen in the right heart along with evidence of an apically placed left ventricular assist device.
D. Photomicrograph of H&E stained section demonstrating transmural fibrofatty infiltration of the right ventricular free wall without other significant pathology. Occasional islands of viable myocardium remain. (40X original magnification)