The Fontan Circulation: The Search for Targeted Therapy
The Fontan operation has been the final palliation for children born with congenital heart defects with a functional single ventricle for more than four decades1, 2. In the early days of the Fontan operation, focus was rightly on technical modifications and alterations in strategy in an attempt to decrease morbidity and mortality related to the operation itself3. In recent years that focus has shifted toward the long-term implications of a total cavopulmonary connection4. It is clear that while the Fontan operation can create a stable circulation through early adulthood, there are inherent limitations to this physiology that impact other organ systems leading to diminished functional capacity over time5. This has led to an intensified search for therapies that specifically and effectively target the deficiencies in the Fontan physiology.
- Received October 16, 2014.
- Accepted October 16, 2014.