What Our Heart Surgeons at Stanford Medicine Children’s Health Are Known For

Unifocalization: Repairing a fourfold defect all at once

A small number of newborns suffer from a complex and potentially fatal congenital defect known as tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries. 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). Our interactive 3-D animation helps you learn more about your child’s heart and unifocalization, a novel surgical technique, which we are number one in the world for performing.

In the past, heart surgeons could only repair this complex and life-threatening defect with several separate surgeries, each of which required the chest to be opened and the heart stopped. Unifocalization—developed and pioneered by Frank L. Hanley, MD, executive director of the Betty Irene Moore Children’s Heart Center—repairs the complete defect with only one surgery in the majority of patients.

How unifocalization works

  • In unifocalization, the blood vessels that 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, anesthetics, 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 heart surgery either more difficult or, worst of all, impossible.
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Learn more about unifocalizaiton

View the difference between a healthy heart and one with tetralogy of Fallot with pulmonary atresia and how our surgeons treat it with unifocalization. 

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 can carry serious risks at times, 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 can give your infant the best chance of living normally, the Betty Irene Moore Children’s Heart Center has developed surgical techniques for congenital heart defects even in extraordinarily small newborns, including successful repairs in the smallest, youngest infants known. As a result of this experience, the Moore Children’s Heart Center is among the world leaders in this demanding specialty. 

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 has had the highest volume of pediatric heart transplants in California for nine straight years. We specialize in providing transplants to children with complex health needs. To learn more about this treatment option, please visit Pediatric Advanced Cardiac Therapies (PACT) Program.

The highly complex Fontan 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.

About the Fontan procedure

The goal of the Fontan is to add a shunt (a small tube that improves 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.

Our novel and superior approach to Fontan

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 98%, despite treating children with very complicated needs who have often been deemed untreatable by other heart centers. Approximately one-third 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.

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.

About CCTGA

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.

Our unique approach to CCTGA

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 innovative, modified approach to the double switch not only reroutes the blood in the heart but also repairs other related heart conditions that your child may be experiencing at the same time. To increase success, we 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, it can delay the need for another heart surgery.

We are actively investigating ways of diagnosing heart rhythm problems while the fetus is still in the womb and performing corrective surgeries before birth. The Betty Irene Moore Children’s Heart Center is the world’s leading site for research in this area, led by Frank Hanley, MD.

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. We are often on the cutting edge of new devices, better surgical techniques, and ways to increase quality of life for children who undergo heart surgery.

Learn more about our groundbreaking research and innovation >