Fontan Clinic
About the Program
Recognizing the extraordinary care that this special group of heart patients require, Children’s Hospital of New Orleans has created a multidisciplinary Fontan Clinic. The Fontan operation is a special operation for patients with single ventricle physiology. Patients with Fontan require extra-specialized care across many different service lines.
Our multi-disciplinary team includes specialists in cardiology, electrophysiology, both pediatric and adult congenital heart disease cardiothoracic surgery, cardiac intervention, and liver disease. This clinical team collaborates to provide family-centered and individualized patient evaluation and management for our Fontan patients.
The team also works closely with the Pelican Krewe, an interdisciplinary team of doctors, nurses, social work, and spiritual care who provide an extra layer of support during serious illness—helping with communication, care coordination, pain and symptom management, with the ultimately goal of optimizing quality of life.
In addition, because these patients require such specialized care, the
clinic also has specialists in pulmonary medicine, endocrinology, radiology,
lung disease, genetic, pregnancy and contraceptive counseling, social
work, anticoagulation, nutrition, and mental health and neurodevelopment.
Children who have complex congenital heart disease and have a Fontan procedure, require follow-up and evaluation by a pediatric hepatologist with experience and knowledge on the Fontan circulation. Years after this surgery, some children might develop long-term issues associated with this type of circulation including Fontan associated liver disease and protein-losing enteropathy.
Is very important for children who have this surgery, to be followed in a comprehensive multidisciplinary clinic that includes Cardiology and Hepatology. Our Heart Center has developed a comprehensive multidisciplinary Fontan clinic to provide this type of care to the children of Louisiana and the Gulf South.
Children's Hospital has acquired technology including ultrasound with elastography that allows a to evaluate Fontan associated liver fibrosis noninvasively.
About the procedure
This Fontan surgery is done when your child is 2 to 4 years old. The Fontan procedure is the third operation in single ventricle palliation—a series of surgeries that treats single ventricle defects. This part of the surgery is done to separate the circulation of blood in the heart. This is so oxygen-poor blood does not mix with oxygen-rich blood in the single ventricle. The surgeon will do one of these 2 repairs:
Extracardiac Fontan
- The IVC is sewn into a tube that is put outside the heart. The tube goes
straight to the pulmonary artery. This lets oxygen-poor blood flow straight
to the lungs to get oxygen. A fenestration may also be used in this case.
Since the Fontan procedure requires open heart surgery, your toddler will
be on a heart-lung machine to temporarily take over blood circulation
and breathing. The procedure will last about five hours. Fontan surgery
recovery usually requires a two- or three-week hospital stay that will
start in the cardiac intensive care unit (CICU).
Lateral Tunnel Fontan - The inferior vena cava (IVC) is a large blood vessel. It brings oxygen-poor blood to the right atrium. A tube is built within the right atrium to make a baffle (tunnel). This is so that blood flows from the IVC directly to the pulmonary artery. This sends blood straight to the lungs to get oxygen. Sometimes, a small hole is left in the tunnel (fenestration). This hole acts like a pop-off valve, preventing pressure from building up in the lungs too much after surgery.
Who do we treat?
Our clinic treats both children and adults who have had a Fontan procedure with the goal of monitoring these patients as they grow to catch developing complications and to provide ongoing care for all Fontan-related health concerns. This clinic does not replace the regular cardiologist visits these patients will need throughout their lives.
Because Children’s has established a multi-disciplinary clinic, our patients can see multiple specialties at one appointment and all the patient’s physicians collaborate on a treatment plan customized for each patient.
What makes us different?
- A Team and Family-Centered Approach – it’s in our mission statement, “The Heart Center exists to provide exceptional and compassionate care to all patients with congenital heart disease, using a team and family-centered approach.” We know how instrumental families are in helping with care. Yes, we are the experts in medicine, but the family members are the experts on the family. Together, as both a medical team and a family team, we develop an individualized plan of care for the patient.
- Expert Cardiology Care – The Heart Center provides care in all its subspecialities. These include cardiac intervention, imaging, electrophysiology, cardiac surgery and heart failure. We are members of the National Pediatric Cardiology – Quality Improvement Collaborative which is actively sharing ways to help improve the management of patients with a Fontan operation.
- Collaborative, multi-disciplinary Care – The Heart Center is a part of an even greater picture – the Children’s Hospital of New Orleans. Our hospital is a tertiary/quaternary facility that not only provides excellent heart care but also care in all pediatric specialties. This is critical in the management of the patient with a Fontan operation. If our patient has a lung, gastrointestinal, endocrine, liver or psychological problem, we have the leading specialists in the country to bring in to help manage those problems.
- Advanced Imaging – Usually, patients with Fontan physiology require more advanced imaging than echocardiography. The Heart Center and Children’s Hospital has experts in advanced cardiac imaging including CT and MR imaging as well as 3D imaging. These modalities give the necessary information to our physicians to strive for the best outcome
- Convenient Care – Patients with a Fontan operation often require care from doctors in many specialties. All these physicians are under the one roof of Children’s Hospital of New Orleans. This allows for “one-stop” visit and care for the patient and family.
Single ventricle conditions we treat using the Fontan procedure:
- Hypoplastic left heart syndrome (HLHS)
- Tricuspid atresia
- Double outlet right ventricle
- Heterotaxy syndrome heart disease
- Pulmonary atresia with intact ventricular septum (PAIVS)
- Unbalanced atrioventricular canal
- Double inlet left ventricle
- Univentricular heart
