Medical treatments for single ventricle anomalies

At Stanford Medicine Children’s Health, we treat all types of inherited single ventricle heart defects in our nationally leading pediatric heart center, where we have performed more than two and a half times the national average of pediatric cardiac surgeries over three recent years. Heart surgery quickly after birth, followed by two more surgeries later on, is often the treatment to repair single ventricle defects and restore adequate blood flow to sustain life. Our well-respected heart surgeons are known for caring for children with highly complex single ventricle anomalies with remarkable outcomes.

The types of surgery performed vary based on the condition, but common surgeries for children with a single ventricle include the following:

Norwood procedure. This surgery is done on newborns. The mission is to build a new aorta, the main artery that carries blood from the heart to the rest of the body. A separate connection (shunt) is created to allow blood from the aorta to reach the lungs and pick up oxygen. The shunt varies, depending on the exact defect present in your child, and can be a modified Blalock-Taussig shunt, a Sano shunt, or a central shunt. Our survival rates for the Norwood procedure are 90%, higher than the survival rates of other peer hospitals.

Glenn procedure. This surgery is typically completed on babies ages 3 to 6 months. A new connection (shunt) replaces the first shunt. The shunt connects the large blood vessel from the top of the body to the heart (superior vena cava) to the pulmonary artery. This allows oxygenated blood to get to the lungs. Our survival rates for bidirectional Glenn procedure are 97%.

Fontan procedure. This surgery is typically completed from ages 2 to 3. A second connection (shunt) is made. It allows blood from the large blood vessel from the lower part of the body to the heart to flow to the pulmonary artery. This surgery varies depending on the exact defect, as well as other factors. Our survival rates for the Fontan procedure are outstanding, at 100%.

Hybrid procedure. In this procedure for newborns, our cardiothoracic surgeons and cardiac catheterization specialists work together to make sure that blood flows from the single ventricle to both the body and the lungs, without your child needing to be connected to a cardiopulmonary bypass pump. Our cardiac catheterization cardiologists put a stent in the ductus arteriosus (a blood vessel that connects the pulmonary artery to the aorta) to make sure that blood reaches the lungs. Next, the cardiothoracic surgeon performs a pulmonary artery banding procedure to ensure that the flow of blood to the lungs is just right (not too much, not too little).

Heart transplant. A heart transplant is performed when medical treatments are no longer sufficient. We work in tandem with our Pediatric Advanced Cardiac Therapies program team, which manages both heart failure and transplant patients in a single program, for better patient outcomes. Our team has over 40 years of experience in performing pediatric heart transplantation and we’ve completed the most in California.

Ventricular assist device (VAD). As one of the first hospitals to petition the FDA for the use of external pumps for children awaiting transplant, we are nationally recognized as a leader in VAD placements. A VAD performs the role of the heart by mechanically pumping blood out of the heart and to the rest of the body. It is often a lifesaving procedure.

Besides heart surgeries, we coordinate a wide range of medical interventions and therapies for your child’s heart, including medications and cardiac catheter lab procedures. By including experts from across our hospital, we care for all of your child’s bodily systems, which optimizes organs to function better. For example, we bring in subspecialists such as gastroenterologists to address feeding concerns, hepatologists to ensure liver health, and endocrinologists to treat diabetes or obesity and to promote bone health.

Holistic treatments for single ventricle conditions

All children with a single ventricle undergo heart surgery, but our care does not stop there. Our pediatric Single Ventricle Program offers a variety of services to care for your whole child. We accompany children as they go through treatment and walk with them through life as they face other challenges that are often related to single ventricle anomalies. Our multidisciplinary team includes a dedicated group of neurodevelopmental psychologists, school liaisons, registered dietitians, and social workers who know and understand the single ventricle care journey and the unique challenges your child faces. By providing comprehensive, holistic care, we give your child with a single ventricle a platform to flourish.