Fetal constrictive pericardial defect with pulmonary capillary hemangiomatosis

Pericardial defect is a rare anomaly that has a variable presentation. Prompt recognition and early surgical intervention are associated with a favorable outcome. The authors present an unusual case of unexpected fetal demise in a 31-week-gestational-age girl who had a large pericardial defect forming a constrictive ring around both ventricular chambers. The defect coexisted with pulmonary capillary hemangiomatosis, an association that has not been reported in cases of pericardial defect.