Giant Eustachian valve and left ventricular systolic dysfunction in a patient with non-dilated amyloid cardiomyopathy

Amyloid cardiomyopathy is characterized by non-dilated thick-walled left ventricular, thickening of interventricular septum and right ventricular free wall, biatrial enlargement associated with granular 'sparkling' appearance of the myocardium. Typically, decreased left ventricular compliance results in abnormal diastolic functions but left ventricular systolic functions are preserved until late in the course of the illness when left ventricle starts to dilate culminating into dilated cardiomyopathy. We present a 77-year-old patient who had typical echocardiographic features of amyloid heart disease, a giant Eustachian valve resembling cor triatriatum dexter and left ventricular systolic dysfunction without associated left ventricular dilatation.