At Cook Children's Heart Center, we take an individualized approach to diagnosing and providing comprehensive care for neonates, infants, children and adults with rare and complex congenital heart anomalies. Our multidisciplinary group of specialists focus on early intervention and delivering comprehensive treatments to improve the patients' quality of life.
Once a patient is diagnosed, our cardiothoracic surgical team, led by Vincent Tam, M.D., medical director, Cook Children's Cardiothoracic Surgery, works closely with cardiology colleagues to determine the best course of action for a patient; taking into consideration both the short- and long-term implications for the child.
Our surgeons take an individualized approach to treating complex conditions that other facilities either treat in multiple surgeries (instead of combining multiple procedures into one), or may shy away from treating all together.
Below is a list of the more complex procedures that Cook Children’s cardiothoracic team performs on a regular basis. Please note, this list is not exhaustive.
We take an individualized approach to repairing our patient’s congenital heart defects so the below is an explanation of the various procedures we do perform but our cardiothoracic surgical team will create a customized treatment plan specifically for you or your child’s diagnosis.
Aortic translocation/Nikaidoh procedure
The aortic translocation is also called the Nikaidoh procedure, named after Cook Children's surgeon, Dr. Hisashi Nikaidoh.
This is a surgery that can be used to correct congenital heart defects such as a double outlet right ventricle or the transposition of the great arteries with stenosis (narrowing) of the pulmonary valve. The surgery involves the "translocation" of the transposed aorta over the correct, left, ventricle. The outflow of the right ventricle is then reconstructed with either a right ventricle to pulmonary artery or a graft.
Together, Cook Children's surgeons, Drs. Nikaidoh and Tam, have the largest combined experience of aortic translocations in North America.
Arterial switch repair
Also known as the Jatene procedure, the arterial switch repairs the transposition of the great arteries. Done within the first few weeks of life, this is an open-heart procedure that requires a temporary stopping of the heart while a heart-lung machine keeps blood circulating and baby breathing.
During the procedure, the pulmonary artery and the aorta are switched back to their normal positions. The coronary arteries are attached to the new aorta in the correct positions. The hole between the atria, which may have been enlarged by the balloon atrial septostomy, is also closed and the patent ductus arteriosus is tied off.
Atrial switch with Nikaidoh procedure (Double switch repair)
This procedure is often performed when a child has congenitally corrected transposition of the great arteries" (CCTGA), a less common and more complex form of transposition of the great artery. The proposed surgery to fix this congenital defect is termed the "double switch" where surgeons redirect blood flow from one side of the heart to the other. The heart's major arteries are also switched during this operation. This corrects the circulation so that the heart's left side serves the body and the right side serves the lungs. This is a very complex operation and is usually performed in infants and children.
Meet Ivy, a Cook Children’s cardiology patient born with this condition. Learn how she was diagnosed before birth, see a video about her surgeries, and discover the advanced technology and teamwork that hsa given her a bright future.
Atrioventricular canal repair
Complete atrioventricular (AV) canal defects, require surgery, usually within the first two or three months of life. The surgeon will close the large hole with one or two patches. The patches are stitched into the heart muscle, and as the child grows, the tissue grows over the patches.
The surgeon will also separate the single large valve into two valves and will reconstruct the valves so they are as close to normal as possible, depending on the child's heart anatomy.
Partial atrioventricular canal defects also require surgery, whether they are diagnosed in childhood or adulthood. The surgeon will patch or stitch the atrial septal defect closed. The mitral valve will be either repaired or replaced with either an artificial valve or a valve from a donated organ.
Several types of procedures can be used to surgically treat Ebstein's anomaly, and our surgeons look at each case individually to see what will be best. These include:
Tricuspid valve repair
Surgeons can reduce the size of the valve opening and allow existing leaflets to come together to work correctly. A newer form of tricuspid valve repair is called cone reconstruction in which surgeons separate the leaflets of the tricuspid valve of the heart muscle where they are then rotated and reattached.
Tricuspid valve replacement
If the existing valve cannot be repaired, the surgeon may replace the valve by removing the defective valve and inserting either a biological tissue or mechanical valve.
Closure of the atrial septal defect
If you have fast heart rhythms, the surgeon may make small incisions in the upper chambers of your heart to create a pattern (maze) of scar tissue. This is because scar tissue doesn’t conduct electricity so it purposefully interferes with stray electrical impulses that cause some types of fast heart rhythms.
When a child is diagnosed with hypoplastic left heart syndrome, doctors typically treat this congenital heart defect with a three step procedure, the first one being the Norwood procedure.
This is usually done within the first two weeks of life. During this procedure, surgeons build a new aorta and place a tube to the lung blood vessels called a shunt.This redirects the right ventricle's output to go to both the body and the lungs instead of going only to the lungs.
Vincent Tam, M.D., medical director of Cardiothoracic Surgery, trained under William Norwood, M.D., the developer of the Norwood procedure in the late 1990s.
The Ross–Konno operation is a complex procedure for patients with complex left ventricular outflow tract obstruction that includes an aortoventriculoplasty with pulmonary autograft. It consists of using the patients' pulmonary valve as a neoaortic autograft (Ross procedure), combined with the opening of a narrowed left ventricular outflow tract (LVOT) by incising the outflow septum.
The Ross-Konno procedure is an excellent technique for the treatment of complex multilevel left ventricular outflow tract obstruction with severe annular hypoplasia and a dysplastic aortic valve. The operation can be performed earlier in life, thus avoiding repeated surgical re-interventions, which may provide only short-term palliation and potentially exacerbate ventricular function.
The Ross-Konno procedure increases the therapeutic choices for neonates or infants with critical aortic stenosis, who show unacceptable results following open valvotomy or balloon valvotomy. The pulmonary autograft demonstrates durability without the likelihood of developing aortic stenosis or progressive dilatation and a low incidence of developing aortic insufficiency.
Despite the technically demanding nature of the operation, the Ross-Konno procedure is the method of choice for the multilevel type of left ventricle outflow tract obstruction, especially in newborns and infants.
This procedure repairs a complex and potentially fatal congenital defect known as tetralogy of Fallot with pulmonary atresia and major aorto-pulmonary collaterals in just one procedure. This procedure recreates the child's pulmonary arteries, making it more likely that the heart can be repaired before the child's condition worsens which can cause the surgery to be more difficult, or even impossible.
Cook Children's Heart Center is one of a few pediatric heart programs in the country to perform the unifocalization procedure, specifically on neonates and infants.
We take an individualized approach to repairing our patient's congenital heart defects so the below is an explanation of the various procedures we do perform but our cardiothoracic surgical team will create a customized treatment plan specifically for you or your child's diagnosis.
Conditions we treat
Our cardiothoracic surgery team's services include the repair of complex congenital heart defects, such as:
- Hypoplastic left heart syndrome
- Transposition of the great arteries
- Single ventricle abnormalities
- Total anomalous pulmonary venous connection
- Tetralogy of Fallot
- Pulmonary Atresia with VSD and MAPCAs
- Double outlet right ventricle
- Truncus arteriosus
- Anomalous origin of the coronary arteries
- Corrected transposition of the great arteries
- Ebstein's anomaly
- Atrioventricular canal defect
Harper's Heart Center story
Harper was diagnosed with hypoplastic left heart syndrome before she was born. Watch her story.
Cardiothoracic surgery team
Procedure volumes and outcomes
Our surgeons take an individualized focus on treating complex conditions that other facilities either treat in multiple surgeries (instead of combining multiple procedures into one) or may shy away from treating altogether. Our surgeons perform almost 500 procedures annually.
We participate in the Society of Thoracic Surgeons (STS) national pediatric cardiac database and actively pursue continuous quality improvement. This allows us to track our outcomes compared to the average of other pediatric cardiology care centers throughout the nation. It is difficult to benchmark pediatric outcomes because of the variety of types of congenital heart surgery.
The STS mortality rates listed below, as well as the Cook Children's specific volumes and outcomes, reflect Jan. 2019-Dec. 2022.
Are you prepared for your child's heart surgery? – Knowing what to expect before the day of surgery can help to ease some of your anxiety. From scheduling a preoperative visit to talking to your child to what you need on the day of and the days following surgery, you'll find everything you need to be prepared, here.
Appointments and referrals
Rare and complex congenital heart defects don't always present a clclear-cutethod of treatment. Often, there are multiple ways of approaching the diagnosis. Our physicians also are available to provide second opinions for families seeking alternative treatment options. If you would like to schedule an appointment, refer a patient or speak to our staff, please call our offices at 682-885-2140.