Acute cardiac tamponade secondary to congenital factor V deficiency.

Spontaneous cardiac tamponade secondary to a congenital coagulation defect has never been reported. We report a case of acute cardiac tamponade in a patient with a known factor V deficiency. This patient presented with classic signs of acute cardiac tamponade and an enlarged cardiac silhouette. After diagnostic two-dimensional echocardiography and treatment with fresh-frozen plasma, the patient underwent emergent pericardiocentesis followed by complete pericardiectomy.