Cardiac cirrhosis: a rare manifestation of an uncorrected primum atrial septal defect

Atrial septal defects constitute the second most common cardiac congenital abnormality. These defects when undiagnosed during childhood, present in adulthood with symptoms of right heart failure. We describe a case of uncorrected ostium primum atrial septal defect that presented as liver failure in a 43-year-old woman. The patient was found to have liver cirrhosis and a complete work-up of hepatic causes for cirrhosis was negative. Further examination revealed an atrial primum septal defect. Liver cirrhosis in this patient was thought to be secondary to chronic congestion from the right heart failure resulting from the atrial septal defect. Although right heart failure is a known cause of cirrhosis, liver cirrhosis resulting specifically from an atrial septal defect is uncommon. In fact, an ostium primum atrial septal defect presenting as liver cirrhosis has not been well described. We present such a case to highlight this manifestation of atrial septal defect and also to reiterate that cardiac causes should always be considered in the differential diagnosis whenever a patient presents with liver cirrhosis. J Cardiovasc Med 11:689-691 (C) 2010 Italian Federation of Cardiology.