While the extracardiac Fontan technique is most commonly used today, there were many historical variations of the Fontan operation, such as the atriopulmonary Fontan procedure, that were performed over the last four decades. More than half of patients undergoing the Fontan procedure eventually develop abnormal heart rhythms, particularly with these older techniques. These patients are prone to developing abnormally fast heart rhythms — supraventricular tachycardia (SVT) or atrial tachycardia (AT) — in adolescence or adulthood. People with earlier forms of the Fontan operation are also likely to develop marked dilation of the top (atrial) chamber of the heart.
These older Fontan patients may be candidates for the Fontan Conversion and Arrhythmia Surgery Program. We have successfully performed more than 150 of these complex surgeries on patients from around the world. Surgical mortality is 2%, and most patients experience a significant improvement in lifestyle and exercise ability.
What to Expect
Patient Medical History Review
Our electrophysiologists and the cardiac team do a comprehensive evaluation before accepting a patient into the program. The patient’s health records (medical, arrhythmia, and surgical history and reports, as well as cardiac images on CDs or DVDs) are reviewed by the team of cardiologists before the patient comes to our center for evaluation and to meet our specialists.
Once a patient is accepted into the program for further evaluation, appointments with pediatric electrophysiologists (EP), adult congenital heart disease specialists and an adult hepatologist are scheduled at Lurie Children’s and Northwestern Memorial Hospital.
Meeting With Specialists & Additional Testing
Patients meet with these specialists to further discuss their medical history. To decide whether a patient is a candidate for surgery, additional testing such as echocardiogram, Holter monitor, blood work, cardiac MRI or CT, cardiopulmonary stress test, cardiac catheterization, and intracardiac electrophysiology study may be performed at Lurie Children’s.
After all tests are complete, the pediatric electrophysiologists, adult congenital heart disease specialist and the cardiac surgeons will meet to review all the data and discuss whether the patient is a Fontan conversion and arrhythmia surgery candidate. This process usually takes about three to five days.
Surgery & Recovery
If surgery is recommended, a surgical date will be chosen, typically a Wednesday. Fontan conversion and arrhythmia surgery usually takes an entire day in the operating room. Almost all patients will receive a cardiac pacemaker during their operation. After the operation, the patient will be admitted to the Regenstein Cardiac Care Center.
Inpatient recovery time is usually about 10 days. Outpatients are then monitored carefully over the next week. Out-of-town patients may stay at Inpatient recovery time is usually about 10 days. Outpatients are then monitored carefully over the next week. Out-of-town patients may stay at Ronald McDonald House Near Lurie Children's. Once cleared by our cardiologists, usually after a week, the patient will return home. Our cardiologists coordinate their continuing care with their local cardiologist.