Double-inlet left ventricle (DILV) is a very rare congenital (present at birth) heart defect in which a child’s heart has an intact left ventricle pumping chamber and a small right ventricle. Both of the upper chambers of the heart, the right and left atria, empty into the left ventricle. Cardiothoracic surgeons typically classify double-inlet left ventricle as a type of single ventricle defect, and treat the defect with several surgeries over the course of many years.

Children with this heart defect may have other heart issues as well, such as:

• Coarctation of the aorta
• Pulmonary atresia
• Pulmonary valve stenosis

The complex surgeries used to fix double-inlet left ventricle require experience, skill and training. Norton Children’s Heart Institute, affiliated with UofL School of Medicine, has pediatric subspecialists with the experience to map out a detailed plan to treat children with this type of congenital heart disease.

Norton Children’s Heart Institute is the leading provider of pediatric heart care in Louisville and Southern Indiana.

Norton Children’s Heart Institute has a network of remote diagnostic and treatment services in Kentucky and Southern Indiana..

What Happens in a Double-inlet Left Ventricle Repair?

Your child will need a number of surgeries to improve blood circulation throughout the body and into the lungs. Each surgery will depend on your child’s current health, size and any other issues he or she has.

The series of surgeries to repair double-inlet left ventricle includes:

• Blalock-Taussig 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.

A child who has a double-inlet left ventricle repair will need lifelong care with a cardiologist to look for any complications and issues that may show up as the child grows.

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.