Single Ventricle Program

The Single Ventricle Clinic is designed specifically for children and young adults born with single ventricle heart defects who have had the Fontan surgery.

What is Fontan surgery?

The Fontan procedure is often the final surgery in a series of three operations for children with single ventricle heart disease. The goal of the surgery is to separate the oxygen-rich blood from the oxygen-poor blood — just like the circulation in a normally structured heart. 

After surgery, the oxygen-poor blood from the lower body goes directly to the lungs, and the blood with high amounts of oxygen goes to the heart and then is pumped into the body. This new way of circulating blood is called Fontan circulation. Most children are between the ages of 3 and 4 when they have Fontan surgery. They do well and lead normal lives afterward. In fact, more than 80% are living more than 30 years after their surgery.

What complications can arise from single ventricle disease?

The abnormal circulation, as well as other abnormalities caused by single ventricle heart disease, can cause complications over time such as:|

•    Decreased heart function
•    Heart valve problems
•    Abnormal heart rhythms
•    Protein losing enteropathy (PLE)
•    Plastic bronchitis

Children may also experience growth problems, kidney and liver dysfunction, and deficits with learning and emotional and behavioral health. 

How can the Single Ventricle Clinic help?

The goal of the Single Ventricle Clinic is to care for each Fontan patient holistically so potential complications can be addressed before they cause problems. By minimizing the burden of heart disease, Fontan patients have the opportunity to reach their full potential and experience an improved quality of life.

The ideal time to start participating in the Single Ventricle Program is around 10 years of age. After the initial appointment, most patients return to our clinic every two to three years for a repeat evaluation.

What does a day at the Single Ventricle Clinic include?

A visit to the Single Ventricle Clinic typically includes multiple appointments that all take place within the hospital during a single day. While each patient’s day is different, the morning usually includes diagnostic testing that focuses on the heart, liver, lungs and kidneys. Tests may include:

•    Blood test and/ or urine tests
•    CT scans
•    MRIs
•    Ultrasounds
•    Pulmonary function tests

The afternoon is spent in the Cardiology Clinic where an exercise test and echocardiogram may be performed. After all testing is completed, results and recommendations are reviewed with patients and their families and shared with the patient’s primary cardiologist.