Managing Patients with Fontan Circulation

Dr. Francois Fontan first performed the Fontan procedure in 1971 on children with tricuspid atresia, or lack of a tricuspid valve. Fontan redirects venous blood from the right atrium to the pulmonary arteries, without pumping it from the lungs to the heart.

The Fontan Procedure is a palliative surgery; in other words, it doesn’t focus on curing the disease, rather, it reduces the symptoms and severity of it, resulting in an improved quality of life for the patient. It’s generally performed on children with complex congenital heart defects. Specifically, it’s used when a child only has one effective ventricle.

The Fontan is typically done as a two-stage repair. The first stage is referred to as a Bidirectional Glen procedure, or Hemi-Fontan. In this stage, oxygen-poor blood is redirected from the upper part of the body to the lungs. The pulmonary arteries are disconnected from their blood supply and the superior vena cava is removed from the heart and directed into the pulmonary arteries. The inferior vena cava transports blood from the lower body and remains connected to the heart. This redirection allows the single ventricle of the heart to do much less work. The second stage is known as Fontan completion, and it also redirects blood from the inferior vena cava to the lungs.

Although there are many different types of Fontan operations, they all serve a common purpose: to cause one effective ventricle to pump oxygen-rich blood to the aorta and into the body.

Patients who undergo Fontan surgery require life-long management to address any problems that may occur, such as heart rhythm issues, a weakened ventricle, or blockages and/or narrowing in the Fontan circulation. In many cases, children who undergo these procedures will require some form of Fontan revision surgery later in life.

It’s difficult to predict exact outcomes for Fontan children—in some cases patients will be able to participate in sports and vigorous activity; however, others may be severely limited in terms of exercise. Because this procedure is still relatively new, there are still many questions as to how well single ventricle heart/Fontan patients will fare as they enter their 30s and 40s, which is why ongoing monitoring is so crucial.

Please feel free to post any questions or comments you may have regarding this or other congenital heart defect procedures in the comments section.

About Michael McConnell, M.D.:

Dr. McConnell specializes in Pediatric Cardiology and general Pediatrics. His area of clinical interest is in cyanotic congenital heart disease management, syncope, and single ventricle patient management. Dr. McConnell completed his residency at the Children’s Hospital of Alabama, and his fellowship was held at the Children’s Hospital Medical Center in Cincinnati, OH. He’s been practicing at Emory since 2000.

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  • Heather T.

    Enjoyed this article. I appreciate all your knowledge. I’m a patient of yours & Dr Book. I just wanted to let you know my son will be a patient of yours soon. He is 15 now and has Holt oram, as myself & my daughter do. But he is an over-comer; he is in jr ROTC and can do all the physical exercises as well as runs 3 miles a day! Thanks goes to doctors as you for his healthy life as a congenital heart patient. Please keep the great work and may God bless you all!

    • Heather,

      Thank you so much for your comment. I look forward to meeting you and your son soon! He sounds like a fantastic young man and a great athlete as well. Thank you for your support of Emory and the adult congenital heart center. Please let us know if you need anything prior to your visit.