Most children are born with two ventricles that pump blood to the lungs and the body. Complex single ventricle is a term that describes a group of rare heart defects in which the heart has a single main pumping chamber (ventricle) that performs the work of both ventricles, having to pump blood to both the lungs and body. Without surgery, most children with single ventricles will not survive to their first birthday.

Complex single ventricle defects include:

• Double-inlet left ventricle
• Hypoplastic left heart syndrome
• Tricuspid atresia
• Some variations of double-outlet right ventricle

A complex single ventricle repair is tailored to the type of defect and many other factors. It takes experience, skill and training to match the right treatment with your child’s unique condition.

Norton Children’s Heart Institute has a network of remote diagnostic and treatment services in Kentucky and Southern Indiana. Our specialists at Norton Children’s Heart Institute will be able to identify a treatment plan that is specially designed for your child’s type of complex single ventricle.

Norton Children’s Heart Institute, affiliated with the UofL School of Medicine, is the leading provider of pediatric heart care in Louisville and Southern Indiana.

What Happens in a Complex Single Ventricle Repair?

A complex single ventricle defect is a serious heart condition that causes one ventricle of the heart to have to do the workload of two ventricles. Patients also have lower oxygen levels than expected. A child with a complex single ventricle may need two or three heart surgeries during the first three years of life.

The overall goal of the surgeries is to separate the body’s two circulation paths, allowing the child’s oxygen saturation to become normal, or as close to normal as possible. After all repair stages are completed, the patient’s single ventricle will pump blood to the body and blood will passively return to the lungs without the use of a “right-sided” ventricle.

What Procedures Are Used to Repair Complex Single Ventricles?

Depending on a child’s specific condition, the following procedures may be used:

• Blalock-Taussig or central shunt: Heart specialists will perform this procedure in the first week or two of life. The shunt is a small tube used to create an alternative route of blood flow to the lungs. This allows for a consistent blood supply to the lungs. In addition, the added flow to the pulmonary arteries enhances the growth of these blood vessels and prepares them for operations that will be done later.
• Pulmonary artery band: If a child has too much blood flow to the lungs, banding of the pulmonary arteries can precisely limit the amount of blood flow going to the lungs. This prevents the pulmonary blood vessels from being damaged.
• Glenn procedure: This surgery detours blood from the upper body to the pulmonary artery directly. This also will decrease the workload that the single ventricle is performing. Cardiothoracic surgeons perform this surgery when a child is around 4 to 6 months old.
• Fontan procedure: This is the final surgery to repair a complex single ventricle. Cardiothoracic surgeons connect the pulmonary artery and the inferior vena cava, effectively separating the body’s two circulations. Now, the patient’s single ventricle will pump blood to the body and blood will passively return to the lungs without the use of a “right-sided” ventricle.

Complex Single Ventricle Repair Complications and After Care

After having the procedures that separate the two body’s circulations, oxygen saturation levels in the blood will become normal or near normal. However, these patients are still left with a single-ventricle heart. This means the heart will have difficulty pumping the amount of blood the body needs (cardiac output). Children with a single ventricle can live active and eventful lives, but they may not be able to exert themselves like children with two ventricles.

A child who has had a complex single ventricle defect repaired will need lifelong care with a cardiologist. The cardiologist will look for developing complications and issues over time, such as abnormal heart rhythms or congestive heart failure. Because they have only one ventricle, these patients are at risk for heart failure early in life and may need to be treated with various medications, or even a heart transplant if their heart failure is severe.

Why Choose Norton Children’s Heart Institute

• Norton Children’s Hospital has been a pioneer in pediatric cardiothoracic surgery, performing Kentucky’s first pediatric heart transplant in 1986 and becoming the second site in the United States to perform an infant heart transplant.
• The American Board of Thoracic Surgery has certified our cardiothoracic surgeons in congenital heart surgery.
• The Adult Congenital Heart Association has accredited Norton Children’s Heart Adult Congenital Heart Disease Program as the only comprehensive care center in Kentucky and Indiana treating adults born with a heart defect.
• More than 17,000 children a year visit Norton Children’s Heart Institute for advanced heart care.
• Norton Children’s Heart Institute has offices across Kentucky and Southern Indiana to bring quality pediatric heart care closer to home.
• The Jennifer Lawrence Cardiac Intensive Care Unit (CICU) at Norton Children’s Hospital is the largest dedicated CICU in Kentucky, equipped with 17 private rooms and the newest technology available for heart care.