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Large pericardial defects are exceptional and difficult to diagnose. A 62-year-old man underwent elective coronary surgery which was seriously complicated by the unexpected intrapleural position of the heart due to the absence of a large amount of pericardium on the left. It appeared retrospectively that the patient had presented typical signs of the malformation and these are described in detail.

Congenital absence of the left pericardium with extreme intrathoracic displacement of the heartCase report and review of the literature