Fontan Failure

Fontan FailureSome people are born with a congenital heart defect that has only one ventricle (or pump) instead of two. You may also hear it called a “single ventricle heart.” When there is only one ventricle pumping blood, the blood that is oxygen-rich mixes with the blood that is oxygen-poor. This can make you have a bluish look to your skin, nails, and tongue. In addition, the ventricle has to enlarge as it tries to pump blood both into the body and into the lungs, which can overwork it. <Single Ventricle Heart (from Fontan book)>Fortunately, like with other types of CHD, pediatricians and pediatric cardiac surgeons have discovered ways to allow you to survive and thrive throughout childhood with only one pumping chamber. If you were born with only one pumping chamber, you likely went through several heart surgeries early in your life and may now have a Fontan circulation.  The Fontan circulation is created when a pediatric cardiac surgeon creates a pathway for blood to return to your lungs for oxygen directly, without a pump. This frees up the one pumping chamber you have to do the hard work of pumping your blood out to your body.<Fontan Heart>While the Fontan circulation can be very successful inevitably, since your heart has only one pumping chamber instead of two, the one pump will wear out and you will experience heart failure. If you have a Fontan circulation and are starting to experience heart failure, you might feel like it’s hard to catch your breath or like your heart is beating in a weird rhythm. Your skin may be more blue-tinted than usual or you may have swelling in certain areas of your body. In addition, because pediatric cardiac surgeons had to make some unnatural changes in your body to create the Fontan circulation, you may be prone to chronic liver disease, renal insufficiency/failure, and/or problems with your intestines leading to malnutrition. <Example of a Fontan Heart – Extracardiac Heart_ORIGINAL FILE NEEDS EDITS>  
Some patients with a Fontan will have symptoms of heart failure that can’t be controlled with medications. They may need a VAD to help them get blood to their organs. They can help as a form of long-term therapy or they can help you survive until it’s time for your transplant. Brooklyn & Brandon’s StoriesVideo/Text/photos

Fontan Patient StoryInfographic/Video
As someone who was born with a single ventricle heart, you have special health concerns now as an adult. This book waswritten for patients who had Fontan heart surgery and are now getting care as adults.Fontan BookBook
For more information about Fontan, see the following resources. When you are faced with a lifelong heart condition such as adult congenital heart disease, it can help to connect with others who get what you’re going through. Support groups offer a way to bring people together to share information. This can also help reduce stress and worry, and provide helpful education. 
Resources:Advanced Therapy PageMended Hearts/Mended Little HeartsSisters by HeartConsiderations for Advanced Heart Failure Consultation in Fontan Patients (Fontan Referral)
<ACHD Support and Resources>