ABSTRACT
In generalized amyloidosis, involvement of heart is not rare, but development fo severe conduction distubance in cardiac amyloidosis is a rare presentation in a clinical situation. We report the first case in Korea of cardiac amyloidosis with complete atrioventricular block, which was confirmed by endomyocardial biopsy. A 59-year-old woman was admitted due to drowsy mentality. We observed a severe atrioventricular conduction disturbance on the electrocardiogram and a marked hypertrophied ventricular wall and a granular sparkling appearance within the wall, a characteristic feature in cardiac infiltration of amyloidosis, on the echocardiography. Endomyocardial biopsy was performed and revealed extensive deposition of amorphous eosinophilic materials within the cardiac muscle cells. Polarizing illumination after Congo-red staining showed typical green birefrigence. We performed a insertion of permanent pacemaker(VVI type) for the treatment of complete atrioventricular block.