ABSTRACT
Congenital absence of the pericardium, also known as pericardial agenesis, is an extremely rare anomaly. Although most cases are asymptomatic, some experience chest pain, dyspnea, dizziness, and syncope. A few sudden death cases have been reported. We report a case of congenital complete left-sided absence of the pericardium that is not related to the cause of death. In the autopsy of a skinny 45-year-old deceased man, there was no left pericardium, and the heart had direct contact with the left lung. Inflammation and adhesion around the heart, torsion of great vessels, structure abnormality of the heart, and histologic lesion of the myocardium were absent. Due to the chemical analysis result and morphology, we suspected that the cause of death was related to starvation. The congenital absence of the pericardium is usually accompanied by myocardial infarction, aortic dissection, and variable congenital anomalies in the heart and other organs. Additionally, both complete and partial defects can cause myocardial infarction. Therefore, we suggest that precise gross examination should be performed to determine the ischemic lesions in the heart and other anomalies if congenital absence of the pericardium is noted in the autopsy.