The adult Fontan patient: update for 2011

Congenital heart disease is the most common birth defect, with an estimated incidence of moderate to severe disease of 4-6 per 1,000 live births. Due to the dramatic advances in cardiac surgery and general pediatric cardiology care, approximately 85% of neonates with congenital heart disease (CHD) survive to adulthood. The most recent information, published in 2004, estimated 787,800 to 1.3 million adults with CHD living in the United States. This is expected to increase by 5% per year. It is estimated that within the next decade, 1 in every 150 young adults will have some form of CHD. DUe to the changing therapeutic options, a higher percentage of the adult CHD population will consist of more complicated cardiac disease during the coming years. It is estimated that 1-2% of children with CHD have single-ventricle physiology. Thus, as survival in this complex portion of the CHD population improves, the number of adult patients with previous Fontan palliation will dramatically increase. The goal of this article is to provide a brief background of the Fontan procedure and then discuss the late-term outcomes and complications in this unique patient type. The majority of the article will focus on information needed to adequately care for the adult Fontan patient.