Partial absence of the left pericardium. Diagnosis by two-dimensional echocardiography

The partial absence of the left pericardium is an unusual cause of syncope and chest pain in children. The identification of this lesion is important because of potential herniation of the left side of the heart through the defect, resulting in fatal strangulation. Initial suspicion of the diagnosis should be raised by the typical cardiac contour on the chest roentgenogram. Two-dimensional echocardiography and cineangiography at cardiac catheterization are additional helpful studies in identifying partial pericardial defects.