Abstract
Objective: Congenital absence of the pericardium (CAP) is a rare heart disorder, frequently misdiagnosed due to an
unspecific clinical picture and leading to diagnostic challenges. The purpose of this case presentation is to show how
cardiac imaging methods can aid in accurate diagnosis of CAP.
Case presentation: A case of the complete congenital absence of the pericardium in a 40-year-old man with
complaints on dyspnea and fatigue is presented. Echocardiography revealed a dilated right ventricle with good
contractility; normal dimensions and function of the left ventricle, and normal heart valve function. Pulmonary
hypertension and atrial septal defect were excluded during echocardiography. Computed tomography revealed
abnormal heart axis rotation leftward and posteriorly, raising suspicion of CAP. The diagnosis was confirmed by
cardiac magnetic resonance imaging . The diagnostic flowchart for the CAP is discussed.
Conclusion: Multimodality cardiac imaging provides clues to the diagnosis of CAP.