A small number of newborns suffer from a complex and potentially fatal congenital defect known as tetralogy of Fallot with pulmonary atresia and major aorto-pulmonary collaterals. In infants with this defect, the blood vessels that should connect the heart to the lungs instead connect the lungs to the aorta, and the heart itself has a hole in the wall separating its lower chambers (ventricles). Use our interactive 3-D animation to learn more about the heart and unifocalization, a surgical technique invented by our Chief of Cardiothoracic Surgery to address these rare heart defects.
In the past, surgeons could repair this complex and life-threatening defect only with several separate surgeries, each of which required the chest to be opened and the heart stopped. Unifocalization—developed and pioneered by Moore Children’s Heart Center Director Frank Hanley, MD—repairs the complete defect with only one surgery, in the majority of patients.
In unifocalization, the blood vessels which are not directed properly are rerouted into a single vessel (or into the pulmonary artery if it is present), which is then attached to the right ventricle of the heart through a homograft (tissue that comes from a human). This restores normal circulation from lungs to heart. Next, the hole in the ventricle wall is repaired.
Because unifocalization is complex, the procedure takes approximatively six to 10 hours, and is followed by hospitalization of up to 14 days.
The benefits of unifocalization to the patient are significant. If your child undergoes this surgery, know that the unifocalization procedure decreases overall hospitalization time for your child, and it reduces the number of major surgeries, anesthesias, and incisions, sparing your child additional pain and trauma. In addition, this procedure makes it more likely that the heart can be repaired before your child’s condition worsens and makes surgery either more difficult or, worst of all, impossible.
How unifocalization works:
Operating on premature newborns with both congenital heart disease and very low (less than 1,500 grams, or 3 pounds 4 ounces) or extremely low birth weight (less than 1,000 grams, or 2 pounds 3 ounces) can be a difficult challenge because of their small size and immaturity. Conventional wisdom holds that it is better to wait for the child to develop further before undertaking heart repair. However, waiting carries serious risks, because the heart defect leaves the child highly vulnerable to potentially fatal complications such as infection or lung disease.
Since fixing the heart defect as soon as possible gives the infant the best chance of living normally, the Betty Irene Moore Children’s Heart Center has developed surgical techniques that allow congenital heart surgery even in extraordinarily small newborns, including successful repair in the smallest, youngest infant known. As a result of this experience, the Moore Children’s Heart Center is among the world leaders in this demanding specialty. Contact us for more information in case your premature or very small newborn was referred for heart surgery.
The Moore Children’s Heart Center is one of the leading heart and heart-lung transplantation programs in the world and the only one that had the highest volume of pediatric heart transplants in California for nine straight years. To learn more about this treatment option, visit our heart failure and heart transplant program (PACT) page.
This highly-complex heart surgery is performed on children born with a single heart ventricle. To correct this defect, a two-step heart surgery is often required within the first three years of life. The first surgery is the Glenn procedure and the second surgery is the Fontan procedure. The goal of the Fontan is to add a shunt (pattern of blood flow in the heart) to allow blood to flow from the inferior vena cava (the main artery from the lower body) to the heart, and from there to the pulmonary artery and the lungs.
There are four different types of Fontan procedures. At Stanford Medicine Children’s Health, we perform a unique off-pump extracardiac conduit Fontan without the use of a heart-lung machine (bypass), which results in better outcomes. Our survival rate for the Fontan procedure is higher than 97%, despite treating children with very complicated needs who have often been deemed untreatable by other heart centers. Nearly 30% to 40% of our patients are referred to us from other heart centers around the country.
While the Fontan surgery does not return the heart to a perfectly normal state, it allows oxygen-rich blood to flow through the body, empowering children born with one heart ventricle instead of two to live an active, high-quality life. Have questions? Contact us for more information.
Congenitally corrected transposition of the great arteries (CCTGA) is a rare and complex congenital heart defect that affects the structure of the heart. In babies born with CCTGA, the right and left ventricles are switched in position and in roles. As a result, the right ventricle must take on the more demanding role of pumping the blood to the body at high pressure. This puts a strain on the right ventricle, which in many cases leads to serious heart problems.
Most children born with CCTGA eventually need heart surgery, and 90% of children with CCTGA have additional heart defects. For CCTGA, we carry out a complicated surgery that reroutes the blood within the heart and restores the roles of the right and left ventricles. We are one of a few programs nationwide that offer an anatomical “double-switch repair,” which our team of renowned heart specialists has been performing for the past two decades.
Our modified approach to the double-switch is unique and innovative. We not only reroute the blood in the heart but also repair other related heart conditions that your child may be experiencing at the same time. To increase success, we also train (strengthen) the left ventricle before surgery.
Our unique approach to the double-switch repair, based on years of experience and research, has been shown to prevent or delay known major complications of the traditional double-switch repair. With our approach, your child can likely experience a better quality of life for longer and in some cases delay the need for another heart surgery.
The Moore Children’s Heart Center is actively involved in exploring new approaches to the surgical repair of pediatric heart disease and developing evidence-based guidelines for clinical care. Current efforts focus on three areas: