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Single Ventricle Program

The Single Ventricle Program is for children who are born with only one pumping chamber (single ventricle) in their heart, instead of the usual two.

A broad range of congenital cardiac conditions are associated with having a single ventricle, including

About Single Ventricle Congenital Heart Defects

Children born with single ventricle congenital heart defects have a series of operations during childhood, including Norwood, Glenn and Fontan operations. Specific complications that need to be monitored include ventricular dysfunction, atrioventricular valve regurgitation, arrhythmia, cyanosis, thrombosis, protein-losing enteropathy and plastic bronchitis. Liver fibrosis and renal dysfunction are common and may progress over time, and cognitive, neuropsychological and behavioral deficits are also highly prevalent.

The normal human heart has main pumping chambers called ventricles. These are the most important chambers in the heart. The right ventricle pumps blood to the lungs, and the left ventricle pumps blood to the rest of the body.

There are many forms of congenital heart disease in which the child is born with only one good ventricle. The other ventricle may be too small, poorly functioning or not exist at all. As a group, these ventricles are known by the broad term single ventricle or functional single ventricle.

There are many different subtypes of single ventricle, including tricuspid atresia, mitral atresia, pulmonary atresia/intact septum, double inlet ventricle and hypoplastic left heart syndrome. They all have in common that the one good ventricle that is present must do the required pumping work to maintain life. In most of these cases, the child will require some form of surgery in order to allow the one ventricle to be able to maintain this work and, in many cases, several operations are required as the child grows. The various operations are described below, including the ages we generally perform them.

Shunt Operations

A newborn or infant with single ventricle may need a shunt. A shunt is a small tube of Gore-Tex (the same material that rain jackets are made of) that is sewn to the blood vessels to allow blood to flow in a certain direction. This allows us to direct blood in one direction or the other in order to assist the one ventricle in keeping the child’s oxygen levels at a proper level. This sometimes must be done in the first few days of life.

For children who require a shunt, the operations may require cardiopulmonary bypass and several weeks of recovery in the hospital. We usually require the child to be on aspirin while the shunt is in place so that blood clots do not form and block the shunt. Sometimes patients with shunts form blockages and many times, these can be unblocked without surgery; this is done with a minimally invasive technique using wires and IVs during a catheterization.

At Rady Children’s Hospital-San Diego, we have been performing shunt operations in children for more than 30 years and have performed hundreds of different types of shunts for all the possible different diagnoses. Risk level for these operations varies depending on the diagnosis, but in general, the operations are over 90 percent successful. The individual risk for your child will be discussed with you during the surgical consultation.

Most newborns and infants who require shunts will go on to require one or two more operations; these are known as bidirectional Glenn (BDG for short) and the Fontan operation.

Our program seeks to improve survival and quality of life for children and young adults with single ventricle physiology. We seek to educate parents regarding the care of their child and provide resources.

This program provides comprehensive and interdisciplinary care, screening, evaluation and coordinated care, and referrals for the following areas:

Team

Cardiology

Jose Alfonso Silva Sepulveda, M.D., Director, Single Ventricle Program and Transitional Program, Assistant Clinical Professor of Pediatrics, UC San Diego

Howaida El-Said, M.D., Ph.D., Director, Cardiac Catheterization Laboratory; Clinical Professor of Pediatrics, UC San Diego

Shylah Haldeman, M.S.N., N.P.-C, CCTC

Jessica Haley, M.D., Director, Home Monitoring Program; Assistant Clinical Professor of Pediatrics, UC San Diego

Gabrielle Vaughn, M.D., Heart Failure/Heart Transplant,; Assistant Clinical Professor of Pediatrics, UC San Diego

Matthew Williams, M.D., Director, Cardiac Pacing; Clinical Assistant Professor of Pediatrics, UC San Diego

Cardiothoracic Surgery 

John J. Nigro, M.D., Division Chief; Director, Heart Institute; Director, Cardiac Transplantation and Ventricular Assist Device Program

John Vossler, M.D. Assistant Professor of Surgery, UC San Diego

Endocrinology

Michael Gottschalk, M.D., Ph.D., Division Chief; Associate Director, Pediatric Diabetes Research Center; Clinical Professor of Pediatrics, UC San Diego

Gastroenterology

Kathleen Schwarz, M.D., M.P.H., Associate Physician Diplomate, UC San Diego

Gynecology

Akilah Weber, M.D., Associate Physician, UC San Diego

Nephrology

Caitlin Carter, M.D.

Pulmonology

Aparna Rao, M.D., Associate Clinical Professor of Pediatrics, UC San Diego

Other team members include a neurodevelopmental practitioner, social workers and registered dietitian.

Resources

National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC): Patient and Family Resources
https://npcqic.org/resources-parents-and-families

Locations

Rady Children’s Hospital-San Diego (Map it)
Rose Pavilion, 1st Floor South
3020 Children’s Way
San Diego, CA 92120
Phone: 858-966-5855
Fax: 858-966-7903

Contact Us

For more information about the program and other cardiology services, contact the Cardiology division at 858-966-5855.