Born-free congenitally absent pericardium

Abstract

Objectives: Congenital absence of pericardium (CAP) is a very rare condition, with an incidence of <1 in 10,000 and occurs in isolation or associated with various congenital cardiac or systemic disorders. We describe the two cases of incidentally diagnosed left-sided congenitally absent pericardium and evaluate the presentation, diagnosis, and management with recent work done in this area. Atrial septal defects have been associated with the absence of pericardium similar to one of our case. We also found this entity associated with aortic coarctation which has not previously been described. Materials and Methods: Using our database, we retrospectively went through the details of patients operated in our center between January 2015 and December 2019 to identify the cases with CAP. Results: Out of 2193 cardiac surgeries, 478 patients got operated for congenital heart disease out of which 2 were found with pericardial defects intraoperatively. No surgical intervention for the pericardial defects was done. Both patients did well after surgery for their primary disease. Conclusions: Congenital defects of the pericardium are very rare. Although there are characteristic radiology findings, yet without a suspicion, these can be easily missed and are discovered during surgery. The associations with many cardiac conditions have been seen; however, association with aortic coarctation is extremely rare as we could not find any such association in literature.