An Integrated Approach to the Care of Adult Patients with Prior Atriopulmonary Fontan Surgery

Because of improved survival among children born with heart disease, more adults than children are now living with congenital heart defects, providing a new challenge for ongoing evaluation and care. At Children's Memorial Hospital in Chicago, we have gained extensive expertise in the long-term outcome of patients with repaired single ventricle anatomy, particularly with regard to arrhythmias and impaired hemodynamics, and have developed an integrated approach to their care. This article will summarize (a) single ventricle physiology, (b) evolution of the Fontan operation and the long-term multi-system sequelae, (c) treatment options for patients with prior Fontan surgery, focusing on Fontan conversion with arrhythmia surgery, and (d) unique management of adult patients with prior Fontan surgery. For the foreseeable future, pediatric nurse practitioners will have an important role, including coordination of care and providing continuity in the care of adults with prior Fontan surgery.