The failing Fontan: etiology, diagnosis and management

Abstract

While the Fontan operation has facilitated the survival of a generation of children born with congenital heart disease resulting in a functional single ventricle, it does not recreate a normal circulation. Over time, survivors of the Fontan operation are at risk for ventricular dysfunction, plastic bronchitis, protein-losing enteropathy and chronic Fontan failure. New techniques and therapies are emerging to address the long-term risks associated with Fontan physiology, but as the number of survivors continues to grow, the recognition of the limitations of this circulation is increasing. Novel investigations of possible mechanical devices designed to function as a subpulmonary ventricle are underway, but are still many years away from clinical use. In the meantime, continued development of medical therapeutics targeted at the specific problems of the Fontan circulation will be beneficial and might reduce the need for cardiac transplantation.

Keywords::

• cardiac transplantation
• congenital heart disease
• Fontan operation
• heart failure
• pediatrics
• plastic bronchitis
• protein-losing enteropathy

Financial & competing interests disclosure

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

No writing assistance was utilized in the production of this manuscript.