Role of Multimodality Imaging in a Case Series of the Partial Absence of the Pericardium: A Case Report

The congenital absence of the pericardium is a rare cardiac entity which could be manifested in isolation or in association with other anomalies. Usually, affected cases are asymptomatic. Hereby, we describe adults with recent palpitations who were diagnosed with the partial absence of the pericardium on chest computed tomography (CT). The first case was a 49-year-old woman, who presented with palpitations, especially in the left decubitus position, of 1 year’s duration. Her physical examination revealed nothing of significance, except for tachycardia. The second case was a 37-year-old woman, who presented with dyspnea. Transthoracic echocardiography suggested the partial absence of the pericardium. The definite diagnosis was made using multidetector CT, which was compatible with the echocardiography findings. An abnormally leftward rotated heart without a tracheal deviation, an elongated and flattened left ventricular border (Snoopy’s sign), the absence of the pericardium, an excessive cardiac motion, a prominent pulmonary artery, and cardiac pulsations were seen on the scan slices. A lucent area between the diaphragm and the heart or the aorta and the pulmonary artery was also seen due to an interposed lung tissue. The final case was a 40- year-old man, who presented with dyspnea. In addition to the above findings, dextrocardia and a persistent left superior vena cava were noticeable. Palpitations and sinus tachycardia in adults may be a mere presentation of the partial absence of the pericardium, and multimodality imaging can be applied for the proper identification of this entity. (Iranian Heart Journal 2018; 19(4): 54-57)