The Adult Fontan Patient: Update for 2011

Background

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.¹ 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.² The most recent information, published in 2004, estimated 787,800 to 1.3 million adults with CHD living in the United States.^{3, 4} 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.^{2, 3, 5}

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.² It is estimated that 1-2% of children with CHD have single-ventricle physiology.^{6, 7} 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.