Best Bi-directional Glenn and Fontan descriptions I've found...

Without grabbing a napkin and pen, I still find I have a hard time concisely explaining in words the surgeries Luke has had and still needs to people who are curious. It's such a simple question: "So, how do they fix that??" But the answer is confusing, even after four years of reading and researching! I thought I would pass on this explanation of the staged Fontan palliation for other heart families to use, or for those reading this blog who understandably don't understand the single ventricle heart!

"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."