New Long-Term Guidelines for Patients after Fontan

Nov 13, 2019 | Brianna N. Tranby | @briannatranby


“Evaluation and Management of the Child and Adult with Fontan Circulation: A Scientific Statement from the American Heart Association.” Circulation.2019; 140:e234-e284

The American Heart Association (AHA) recently proposed new guidelines for how patients with Fontan circulation should be monitored for potential complications. The article also proposed a “Fontan Circulation Surveillance Testing Toolkit” to guide physicians on which tests should be performed and when.

Lynn Cern, RN, is a nurse research coordinator with the Mayo Clinic HLHS Program. She reviewed the new guidelines and has summarized them below. She also developed a convenient, printable checklist with these questions for your cardiologist that you can find on our website!


Guest Writer – Lynn Cern, R.N.

The medical community does a good job preparing you for the sta20982194[1]ged surgeries that are needed with a functional single ventricle. Those stages are completed when you leave the hospital after the Fontan operation. Then what? Of course, there are the post-operative clinic visits, but what happens when your cardiologist tells you that you don’t need to be seen for another six months, or even a year?! Regular and frequent visits to the cardiologist have been a way of life for at least the first three years of life. But once you’ve gotten through the “planned” or “anticipated” tough stuff, you may start asking “Now what do I do??”


Primary Care

First, make sure you have a Primary Care Physician (PCP). A PCP is a physician (pediatrician, family practice, or general internist), an advanced practice registered nurse (APRN), or a physician’s assistant (PA). You want your PCP to be someone you can trust, communicate well with, and hopefully even like.

Topics that need to be established with your PCP include:

  • The cardiologist: Your PCP must know who your cardiologist is, and accept that it is the PCP’s responsibility to keep communication open in both directions with your cardiologist. Be sure that records of any problems and routine care are sent to your cardiologist’s office. Your cardiologist needs to be informed of any necessary or elective procedures or surgeries. Any new medication also needs to be reported to your cardiologist.
  • Methods of communication: Establish the best way to communicate with your PCP. Should we email or call, or use an online patient portal? Should it be through ancillary staff (nurse or secretary)? How should I communicate if I have an emergency? When do I need to go directly to an Emergency Department? (Have a plan ahead of time and know where the closest Emergency Room is.)
  • In their absence: Who will cover or help in your PCP’s absence?


General Health Topics

Vaccinations, including annual flu shots, must be kept up to date!

Dental care is essential for the rest of your life. Regular cleanings are part of good dental care. Be sure to establish with your cardiologist whether you need to take antibiotics before dental visits. Have your cardiologist or PCP provide a standing prescription to your pharmacy that can be filled when you need it. Similarly, if you need antibiotics before the dentist, you will probably need them before other planned procedures. Always check with your cardiologist if you plan a surgery or invasive procedure, including tattoos and piercings.

Many drugs can compromise heart function or cause heart rhythm problems. This includes non-prescription (over-the-counter) medications and recreational substances. Patients after Fontan should have their cardiologist assess any new medications or substances.

Plan ahead: Whether traveling or relocating, have a plan to inform a new caregiver about yourself. Keep copies of current EKGs and echocardiograms. Make a list of all your surgeries and all medications you are taking, and keep it updated.

Your Cardiologist: If your cardiologist is a pediatric cardiologist, you may need to transition to an adult congenital cardiologist at some point. Some congenital cardiologists are board certified or trained to care for both children and adults, and can care for you into adulthood.

Staying Fit! It is crucial that post-Fontan patients stay physically fit! Find the method of exercise that works best for you, one that you enjoy. Follow healthy eating guidelines. Overweight or obese patients after Fontan have an increased risk of developing some of the problems known in patients after Fontan.


Long-Term Assessment of the Adult Patient after Fontan

The following topics are areas/body systems that need to regularly be assessed in a patient after Fontan. Your cardiologist should monitor and you should report any symptoms associated with these areas:

  • Atrioventricular valve regurgitation – the valve between your atrium (upper chamber) and ventricle (lower chamber) can have problems due to leaking.
  • Arrhythmias – You may or may not feel palpitations (your heart beating fast or irregularly). This can lead to shortness of breath, lightheadedness, or passing out.
  • Brain and Neurocognitive Development – Several factors contribute to brain and neurocognitive development, which put the patient after Fontan at increased risk for deficits. Early testing and intervention help identify tools needed to optimize the Fontan patient’s potential.
  • Cyanosis – Your cardiologist will monitor your oxygen saturation. After Fontan operations, the typical person has an oxygen saturation of 90-94%. If it is lower, you are “blue” or more cyanotic. This can lead to complications and the cardiologist may discuss further testing, such as cardiac catheterization.
  • Exercise and FitnessExercise is essential for patients after Fontan!!!! Overweight Fontan patients do not do well long term. Regular exercise and fitness will help the Fontan patient grow, develop, and maintain good bone health, Be sure to discuss specifics with your cardiologist.
  • Ventricular dysfunction – Ventricular function is generally assessed on echocardiogram, or other imaging testing. If your ventricular function decreases, your medications may need to increase or change.
  • Protein-losing enteropathy (PLE) & Plastic bronchitis – PLE is the abnormal loss of protein from the body, usually in your stool. Plastic bronchitis causes a thick material to build up in the breathing tubes of the lungs causing a chronic productive cough and increased cyanosis.
  • Renal dysfunction – Fontan circulation, toxic medications, and many surgeries can lead to decreased kidney function.
  • Contraception and Pregnancy – Early identification of whether a female Fontan patient should become pregnant is essential. Preventing a pregnancy by prescribing low-risk contraception is also crucial, as well as genetic counseling. ALL women with a Fontan need to discuss these issues with their cardiologist BEFORE thinking about becoming pregnant.


Other adult illnesses: Just like other adults, the adult patient after Fontan will encounter other health problems: gall bladder issues, appendicitis, orthopedic issues, and gastrointestinal issues to name a few. How should they be handled? Make sure your cardiologist is always involved, and that any provider who cares for you has your complete and current medical history.

Converting YOUR Fontan: Was your Fontan completed before 1990? You may be a candidate for a revision of that surgery.

Transplantation: Though it is something no one likes to think about, cardiac transplantation can be an option for the failing Fontan, especially for those suffering from PLE. Your cardiologist should monitor your heart function to incorporate all other possible treatments to delay or prevent the need for transplant. However, if transplant is needed, your cardiologist should have an open and honest discussion with you about which hospitals specialize in transplant and have the highest volumes and best outcomes.



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