Cardiac Center

Single Ventricle Survivorship Program

Single ventricle heart defects

Single ventricle heart defects are one of the most complex and challenging forms of congenital heart disease to treat.

Approximately 3,000 children are born in the United States each year with complex heart defects in which one of the heart’s ventricles is too small or weak to pump blood effectively to the body and/or lungs.

Single ventricle heart defects may include:

Forty years ago, single ventricle heart defects were fatal. Today, with early intervention in infancy, most children with single ventricle defects are surviving. Many of these patients undergo a series of surgeries in childhood to reconfigure the heart and circulatory system into an altered system, called Fontan circulation. The Fontan operation establishes a normal direction of blood flow: “blue” poorly-oxygenated blood to the lungs and “pink” highly-oxygenated blood to the body.

Many of these patients lead highly functional and active lives after single ventricle surgery in childhood. There are however certain health problems, not limited to the heart, being recognized with increasing frequency. Survivors may encounter complications such as:

Ongoing care: The Single Ventricle Survivorship Program

Improving the quality of life and finding new treatments for patients with single ventricle heart defects is one of the most pressing challenges in pediatric cardiology. The Single Ventricle Survivorship Program has been created to focus attention on the challenges faced by these patients to improve quality and duration of life.

Led by Jack Rychik, MD, this program provides screening, evaluation and coordinated care from a multidisciplinary team of experts that includes:

Single ventricle heart defect research

Patients in the Single Ventricle Survivorship Program are also offered the opportunity to take part in research. Creation of a specialized program of care with focus on single ventricle heart defects will attract patients from around the nation further increasing the number of those treated. This will strengthen our experiences and increase our knowledge base concerning this relatively uncommon disease.

With the Single Ventricle Survivorship Program, CHOP is well positioned to focus its attention on addressing the challenges faced by patients with single ventricle heart defects and to develop innovative solutions to continue to improve quality of life for these patients.

Contact us

If your child has a single ventricle heart defect, and you’re interested in learning more about becoming a patient and/or taking part in congenital heart defect research, please contact:

Katie Dodds, RN, MSN, Nurse Program Manager
Phone: 267-426-3057

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Single Ventricle Malformations Video

Learn how experts in the Fetal Heart Program diagnose and monitor single ventricle malformations before birth, allowing effective treatment to begin right after delivery.

Watch the video »