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A single ventricle defect is a congenital (present at birth) heart condition, and one of the most complex heart defects. It means that only one of the heart’s two ventricles is large enough or strong enough to effectively pump blood. Single ventricle defects occur in five out of every 100,000 live births. This type of defect develops as the heart grows during the first eight weeks of pregnancy. It might be caused by a combination of genes and factors in the baby’s and mother’s environment during this early stage of development.
Single ventricle defects may include:
As the leading providers of pediatric heart care in Louisville and Southern Indiana, the cardiothoracic surgeons at Norton Children’s Heart Institute, affiliated with the UofL School of Medicine, are experienced with successfully repairing single ventricle defects.
The board-certified and fellowship-trained specialists at Norton Children’s Heart Institute have the skills and experience to provide a pinpoint diagnosis and develop a customized treatment plan for you and your child.
A single ventricle defect causes mixing of oxygen-rich blood with oxygen-poor blood in the heart. A newborn with this condition can have:
These signs can start a few hours to a day or so after the birth. Without treatment, the baby’s blood pressure will fall too low to meet the body’s needs.
Specialists at Norton Children’s Heart Institute diagnose single ventricle defects either during pregnancy with a fetal echocardiogram or shortly after birth with an echocardiogram, a test that uses ultrasound to build pictures of the heart. However, it is possible that some children with single ventricles will be diagnosed at a later time.
The following tests may be used to find more details about a child’s specific condition:
In some cases, a doctor may order the following additional tests to confirm the diagnosis:
The cardiothoracic surgeons at Norton Children’s Heart Institute will repair single ventricle defects with a combination of procedures and surgeries based on short-term and long-term needs.
Balancing the amount of blood flow between the body and the lungs is the first short-term solution. It requires one of these procedures:
Sometimes, a newborn with a single ventricle defect won’t need a repair right away. Surgery will be necessary, but can wait until the child is older.
In complex cases, surgery such as a Norwood procedure, used in hypoplastic left heart syndrome, is necessary to reconstruct the way blood flows to or from the heart. All newborns need unobstructed blood flow to the body with a proper amount of blood flow going to the lungs.
In order to prepare for a long-term solution, a cardiac catheterization will help evaluate the structures of the heart and measure blood pressures within the heart and lungs. The catheterization will help determine if the child is able to have the next-stage surgery, called the bidirectional Glenn procedure.
Two large veins bring unoxygenated blood back from the body, one from the upper body and one from the lower body. In the bidirectional Glenn procedure, usually done when the child is 6 to 8 months old, the superior vena cava vein from the upper body is connected directly to the lungs which decreases the work load on the single ventricle while also increasing oxygen saturations. The Fontan procedure, usually done between ages 2 to 4 years, is the third modification in single ventricle treatment. In this procedure, blood from the inferior vena cava from the lower body is rerouted from the heart to the pulmonary artery. This further decreases the volume load on the single ventricle and again increases oxygen saturations. After completion of the Fontan procedure, the patient may have normal saturations, or lower saturations if a fenestration or hole was left connecting the Fontan tunnel with the heart. Your pediatric cardiologist will decide if the fenestration, if present, may be closed at a later time.
After completing the staged repairs for a single ventricle, children may lead a productive life. However, complications can occur, including heart rhythm issues that require medication or pacemaker placement. Lung issues also can occur, including a decrease in blood oxygen levels. Digestive issues can occur due to protein loss in the stool, which results in swelling and water retention. Some patients, despite doing well after surgeries, may need a heart or lung transplant later in life
The hope for children with single ventricle defects is that they will live into adulthood with as few long-term complications as possible. They will need lifelong follow-up care with a pediatric cardiologist. Monthly visits will be needed during their first few years. After having the Fontan procedure, visits may go down to once or twice a year. Most children who have had a single ventricle defect repaired will take medicines for the rest of their lives. Children who need the Fontan procedure can exercise normally, but many will have limits on how active they can be.