Babies who have serious heart conditions requiring multiple surgeries, including single-ventricle heart defects, receive specialized care and monitoring.
Babies with certain congenital heart defects such as single ventricle defects will need at least two surgeries while they are still very young, often within the first six months of life. This time between surgeries is considered a higher-risk period and it is critical that they receive expert, comprehensive care to monitor their health and development between surgeries.
Families receive seamless coordination of care and a consistent point of contact during this time in the Cardiac High Risk Monitoring Program at Johns Hopkins All Children’s Hospital in St. Petersburg, Florida.
Our team generally meets with families during the prenatal period to assist with coordination of care through delivery, surgery, hospitalization and beyond. We provide expert, compassionate care not only for your baby, but for your entire family.
Our program is also part of the National Pediatric Cardiology Quality Improvement Collaborative, which performs quality research focused on children with single ventricle heart defects. This keeps our team on the cutting edge for standard of care in our treatment of patients with higher-risk conditions.
Why Choose Johns Hopkins All Children’s
Families have 24-hour access to our team through a dedicated phone line for any concerns or questions they may have between their child’s appointments.
Families receive the technology they need to track their child’s vital signs, feedings, and any other important information between appointments. This home-monitoring technology provides our team with information on your baby in real-time between appointments.
We work closely with other members of the Heart Institute team to ensure children receive comprehensive care from diagnosis through follow-up. Surgeries are performed by our board-certified pediatric heart surgeons. Children may need cardiac catheterization to diagnose or treat their congenital heart defect, including ongoing treatment throughout their lives. These procedures use a long, thin tube called a catheter to examine the heart and treat certain heart conditions, and are performed by our expert interventional cardiology team.
We also work closely with other providers in specialties like gastroenterology, speech-language and feeding, and neurodevelopment to coordinate your child’s care. Families can also receive support from the hospital’s nutrition specialists and social work team as needed.
Conditions We Manage
We provide care between surgeries for children with single ventricle defects and other conditions that require multiple surgeries and a higher level of monitoring between surgeries. Some of these conditions include:
- Single ventricle defects including:
- Double inlet left ventricle (DILV)
- Hypoplastic left heart syndrome
- Mitral valve atresia
- Pulmonary atresia with intact ventricular septum (PA/IVS)
- Some heterotaxy defects
- Tricuspid atresia
- Unbalanced atrioventricular canal defect (AV Canal)
- Tetralogy of Fallot
Single Ventricle Heart Defects
Many of the patients we treat in the Cardiac High Risk Monitoring Program are receiving care for a single ventricle heart defect. The ventricles are the two lower chambers of the heart that help pump blood out of the heart. Single ventricle heart defects occur when one of the ventricles of the heart does not form normally.
Single ventricle heart defects may sometimes be diagnosed before birth through a fetal echocardiogram. This is a type of ultrasound that is used to take pictures of an unborn baby’s heart and examine the heart’s structure, function and rhythm. The team in our Fetal Heart Program specializes in diagnosing congenital heart defects in babies before birth using fetal echocardiogram.
Single ventricle defects may also sometimes be diagnosed after birth. Symptoms vary depending on the condition, but babies with a single ventricle defect may have a blue or purple tint to their skin and lips (this is called “cyanosis”) and may have trouble breathing or feeding on their own.
While there are several different types of single ventricle defects, treatment for these types of defects is similar. Babies with single ventricle defects will need early intervention and treatment beginning soon after birth. Single ventricle defects often require a series of open-heart surgeries over several years to repair the defect.
This is a “staged reconstruction” and involves procedures called the Norwood procedure, the Glenn procedure and the Fontan procedure. The first procedure (the Norwood) typically occurs within several days of birth, with the second (the Glenn) at about 6 months of age, and the third (the Fontan) between 1½ to 3 years of age. The goal of the surgeries is to reconstruct the heart’s circulatory system.
Additional Follow-Up Clinics for Patients
Johns Hopkins All Children’s also has several specialized follow-up clinics that provide care for our patients:
Cardiac Neurodevelopment Clinic
The team in the cardiac neurodevelopment clinic works with families to help patients reach developmental milestones. The clinic includes neuropsychologists, occupational and physical therapists, and speech pathologists.
The team in the feeding clinic monitors patients closely to make sure children are getting the nutrition they need. For children with feeding tubes, we teach families how to confidently use the tubes at home.
Longitudinal Fontan Clinic
As patients get older, we also have a longitudinal Fontan clinic for children who have undergone this procedure. The clinic team has expertise in how the organ systems of patients who have undergone the Fontan procedure may be impacted as they grow. The clinic team works with each patient’s primary cardiologist to offer a specialized multi organ system approach to their care.
For more information or to make an appointment, please call 727-767-3333. We serve families throughout the greater Tampa Bay area and beyond.
Baby Asher was born with a rare form of congenital heart disease that required major surgery during his first week of life, and another surgery at 5 months old. Now, he and his family celebrate his first birthday.