Surgery, Gastroenterology and Oncology

Congenital absence of the pericardium is a rare condition, which can be isolated, but most frequently associated with other cardiac or extracardiac congenital anomalies.
Generally, most the patients with pericardial absence are asymptomatic. In some cases, partial pericardial defects can be life threatening, because of heart and lung herniation and ventricular entrapment. The pericardium has a supporting and protecting role for the heart and the proximal great vessels (ascending aorta and pulmonary arterial trunk), so absence of the pericardial layers allows important thoracic displacement of the heart. Diagnosis is established by the relevant radio-imaging findings suggestive of complete absence of the pericardium, with characteristic cardiac silhouette abnormalities.

We present a case with complete absence of the left pericardium in an asymptomatic young male patient. A review of literature focusing on congenital pericardial defects and the radiological findings of this condition, focused on cardiac- MR imaging, is also presented.

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