Sudden cardiac death in a case of undiagnosed pericardial agenesis

Isolated congenital absence of the pericardium (CAP) encompasses a range of congenital pericardial defects from a small foramen in the pericardium to a complete absence of the entire pericardium.1 Complete absence of the entire pericardium or absence of the whole left or right side usually has an excellent prognosis. On the other hand, partial absences are more dangerous because entrapment of parts of the heart through the defects may lead to fatal myocardial strangulation, myocardial ischemia and sudden death. Most patients are asymptomatic, but some may have non-specific chest pain.2 Complete absence of the left pericardium usually results in a characteristic chest radiograph, including a leftward shift of the cardiac silhouette. Electrocardiographic ST-T abnormalities are rarely described.