Congenital absence of left atrial appendage (LAA) is an extremely rare condition and its physiological consequences are unknown. We present two cases of incidental finding of a congenitally absent LAA in a 79-year-old male who presented for routine transesophageal echocardiogram (TEE) to rule out intracardiac thrombus prior to placement of biventricular implantable cardioverter-defibrillator and a 54-year old female who presented for TEE prior to radiofrequency ablation of atrial fibrillation. Characterization of patients with such an absence is important because congenitally absent LAA may be confused with flush thrombotic occlusion of the appendage.
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