Congenital pericardial defect

Congenital pericardial defect was first described over 400 years ago by Columbus (quoted from Ellis, Leeds and Himmelstein, 1959). Chang and Amory, in 1965, referring to previous publications from 1961 (Chang and Leigh) compiled 112 cases from the medical literature, including their own recent one. The present case report, together with three other recent ones (Baker, Schlang and Ballenger, 1965; Rogge, Mischkin and Genovese, 1966; Varriable, Rossi and Grace, 1967), brings the total number of published cases to 116. Up to the last decade the majority of instances described were observed incidentally at autopsy or during various surgical procedures. In the last decade, however, with the increasing awareness of the lesion, radiological investigations have been the means of detecting such cases. Recognition of this anomaly is of importance because the resulting radiologic appearance may simulate different serious pathological conditions. Two types of congenital pericardial defects have been described in the literature: a complete form, where there is absence of the pericardium on one side of the heart, left or right; and a partial form, where the defect is localised to a certain area of the heart. Complete agenesis of the pericardium is not included because it is usually associated with multiple malformations, not compatible with life. All the reported cases have had either one or the other type of defect. The case to be described here is unique because both types were probably present, each with its characteristic radiological features which will be discussed in detail.