News & Events

Added to My Toolbox
Removed from My Toolbox
Added to My Toolbox
Removed from My Toolbox
Aug 03, 2019

Experts Recommend Lifelong Monitoring in Patients After Fontan Procedure

The American Heart Association has developed a toolkit for managing patients with a rare congenital heart defect.

While the Fontan procedure is lifesaving for children with a rare congenital heart defect, patients should be continually monitored to improve outcomes, based on a statement released by the American Heart Association. The statement was recently published in Circulation and helped address the long-term monitoring of patients with hypoplastic left heart syndrome.

Hypoplastic left heart syndrome or HLHS is a rare congenital heart defect that affects an estimated 960 babies in the U.S. each year. It occurs when the left side of the heart doesn’t grow as it should, reducing blood flow to the rest of the body. Without surgery, the condition is fatal. However, the Fontan procedure has boosted survival rates since its introduction in the 1970s. More patients are living longer than ever before thanks to the lifesaving surgery.

The Fontan procedure works by rebuilding parts of the heart to redirect blood flow. It creates what experts call “Fontan circulation” and restores normal blood flow to the rest of the body. It’s estimated that 70,000 patients worldwide are living with Fontan circulation and these numbers are expected to double in the next twenty years.

As individuals born with HLHS continue to grow older, the focus has shifted to long-term prevention and treatment. And the recent statement from the American Heart Association helps provide some much needed guidance on monitoring.

Most importantly, the statement provides a surveillance “toolkit” for patients after the Fontan procedure. It recommends testing every 3-4 years in young children and every 1-2 years in to help flag health complications that may develop. Common complications include circulatory failure, abnormal heart rhythms, liver problems, as well as cognitive and behavioral issues.

But not all providers are equipped to monitor and treat patients who have undergone the Fontan procedure, argue authors. The procedure is still relatively rare and both monitoring and treatment of patients with HLHS remains complex. To ensure proper treatment, the statement recommends surveillance by a specialized health care team that is experienced in this field.

Authors also encourage further research on management of patients with Fontan circulation, as the prevention and treatment of complications are still unclear. Experts hope that together, these steps will enhance treatment of patients with HLHS and continue to improve both survival and quality of life.

Questions for You to Consider

  • What are congenital heart defects?
  • Congenital heart defects refer to a number of different conditions that can occur when a baby’s heart is forming or at birth. Although most defects are found during pregnancy or in early childhood, some defects aren’t discovered until adulthood. Survival rates depend on the severity of the heart defect, but most individuals with congenital heart defects live long and healthy lives.
  • How common are congenital heart defects?
  • Congenital heart defects are the most common type of birth defect, affecting roughly 1% of all births in the United States. Thanks to advancements in treatments, it’s estimated that 1 million adults are now living with a congenital heart defect.

Infographic: Congenital Heart Defects


Peripartum Cardiomyopathy Explained

Learn about this uncommon condition triggered in women by pregnancy.

Positive Emotions Can Trigger Mysterious Heart Condition

“Broken heart syndrome” is triggered by both joyful and negative events, finds study.

New Drug Offers Hope for Patients with a Rare Form of Cardiomyopathy

Breakthrough therapy appears both safe and effective in patients with a rare and fatal heart condition called transthyretin amyloid cardiomyopathy.

Cardiomyopathy Increases Heart Risks in Pregnant Women

Women who develop diseases of the heart muscle during pregnancy have the greatest risk of complications.