For my fellow heart moms... This is from The Children's Hospital of Philadelphia.
 

Single Ventricle Survivorship Program

Single ventricle heart defects

Single ventricle type of congenital heart disease is 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.
These defects may include:

• Tricuspid valve atresia
• Hypoplastic left heart syndrome
• Hypoplastic right-heart syndrome (pulmonary atresia with intact ventricular septum)
• Double-inlet left ventricle
• Heterotaxy syndrome

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 problems such as:

• Difficulty exercising or playing sports
• Abnormal heart rhythm
• Abnormal clot formation with increased risk of stroke
• Liver abnormalities
• Protein losing enteropathy (loss of protein in the stool)
• Plastic bronchitis (cast formation in the airways)

Ongoing care

Improving the quality of life and finding new treatments for patients with Fontan circulation 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 will provide screening, evaluation and coordinated care from a multidisciplinary team of experts that will include:

• Cardiologists
• Nurse practitioner
• Cardiothoracic surgeons
• Gastroenterologists
• Endocrinologists (bone health and growth)
• Hepatologists (liver disease)
• Hematologists (blood disorders and diseases)
• Nurses
• Radiologists
• Other specialists as needed

Research

Patients in this program are also offered the opportunity to take part in research. Creation of a specialized program of care with focus on single ventricle 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 this program CHOP is well positioned to focus its attention on addressing the challenges faced by these patients 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 research, please contact:
Katie Dodds, RN, MSN, Nurse Program Manager
Phone: 267-426-3057
Email: svsp@email.chop.edu