Pediatric Care for Single Ventricle Heart Defects

Children with single ventricle heart disease may encounter complications such as:

• Fatigue and difficulty exercising
• Abnormal heart rhythm
• Liver problems
• Learning difficulties
• Behavioral issues
• Abnormal clot formation
• Loss of protein in the stool causing diarrhea
• Abnormal airway plugs causing chronic cough

A number of congenital cardiac conditions are single ventricle defects, including:

• Some Atrioventricular septal defects (AV canal)
• Double inlet left ventricle
• Some double outlet right ventricles
• Some Ebstein’s anomalies
• Hypoplastic left heart syndrome (HLHS)
• Hypoplastic right heart syndrome (HRHS)
• Some forms of pulmonary atresia
• Tricuspid atresia

How is a Single Ventricle Defect Diagnosed and Treated?

Single ventricle heart defects are usually diagnosed before birth via a fetal echocardiogram, a special type of ultrasound test that looks at the baby’s developing heart. In most cases, the condition requires intensive medical and surgical intervention soon after delivery. To address the defect, children typically undergo a series of staged surgeries during the first three to four years of life called single ventricle palliation.

Management Pediatric Single Ventricle Patients after the Fontan Operation

UH Rainbow Babies & Children’s Congenital Heart Collaborative offers the Comprehensive Pediatric Fontan Clinic at the hospital’s main campus location in University Circle. This specialized clinic is for all pediatric single ventricle patients who have had their Fontan procedure.

The Congenital Heart Collaborative is committed to providing state-of-the-art care for all of our patients with single ventricle heart conditions. Early optimized medical management is key to preventing and delaying many of the complications that can develop as these children grow older. The Comprehensive Pediatric Fontan Clinic provides advanced care to improve long-term outcomes and promote overall patient health and well-being.

Though cardiac care is a major priority for patients who have had Fontan surgery, close monitoring of the liver, kidney and lung health, as well as potential learning disabilities, is extremely important. Our clinical staff provides multidisciplinary care for single ventricle patients and all of their unique medical concerns, collaborating closely with many other pediatric medical specialties and services, including our colleagues who specialize in the care of the liver, intestines, kidneys, lungs and other organs. We will also coordinate evaluations and visits with our neurodevelopmental specialists.

Specialized testing we offer includes:

• Blood work testing
• Echocardiogram
• Electrocardiogram (ECG/EKG)
• Exercise testing with metabolic testing
• 24-hour or longer home ECG/EKG
• 24-hour home blood pressure monitoring
• Chest x-ray
• Liver ultrasound and other liver image testing
• Neurodevelopmental screening/testing
• Dietary counseling

Our team uses evidence-based evaluation and treatments for patients as they move from childhood into teenage years. If your child already sees a primary cardiologist, we will work with them to make sure your child receives the best single ventricle care. As our patients move through their teenage years, we also help prepare them for the transition into our comprehensive adult congenital heart disease program.

Improved Quality of Life with a Single Ventricle Defect

Children who undergo surgical repair for single ventricle defects require lifelong follow-up care by a pediatric cardiologist and may need to take certain medications for the rest of their life. Sometimes additional procedures will be required throughout the patient’s lifetime.

You can promote your child’s health and minimize their risk of complications by encouraging them to:

• Avoid smoking
• Eat a healthy diet consisting of lots of fruits, vegetables and whole grains, but low in fats, sweets and salt
• Maintain a “normal” BMI
• Maintain a normal blood pressure
• Maintain excellent oral health
• Maintain regular clinic follow-up
• Participate in some form of exercise as prescribed by the cardiologist or nurse practitioner
• Take their medications as prescribed