Single Ventricle Anomalies in Children

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In a normal heart, the right and left sides of the heart work by collecting blood and pumping it to the lungs or the rest of the body. In babies born with a single ventricle anomaly, one of these sides or ventricles does not develop properly. This inherited birth defect can appear in both mild and severe forms, and include:

  • Tricuspid atresia — when the tricuspid valve doesn’t develop
  • Pulmonary atresia — when the pulmonary valve doesn’t develop
  • Hypoplastic left heart syndrome — when the left side of the heart doesn’t develop
  • Double inlet left ventricle — when the child has a large left ventricle and a small right ventricle

Whatever type of single ventricle anomaly your child has, at UVA, you will find a comprehensive suite of treatments within our congenital heart center. Not only are we the only hospital in Virginia providing pediatric heart transplants, but we provide pediatric cardiology and cardiovascular surgery expertise that has been nationally ranked by U.S. News & World Report.

Symptoms of a Single Ventricle Anomaly in a Child

Signs to look for in your infant or child:

  • Blue or pale, grayish skin color
  • Fast breathing and heartbeat
  • Sweating
  • Poor feeding/poor weight gain

Advanced diagnostic tools can help your pediatrician or cardiologist determine your child’s condition, including:  

  • Echocardiogram
  • Chest X-ray
  • MRI scan
  • CT scan
  • Electrocardiogram
  • Cardiac catheterization

Penn's Beautiful Heart Story

Penn's heart didn't fully develop as a baby. But, today, it's a normal size, thanks to a procedure that opened her right ventricle. She was brought to UVA because her sister was also successfully treated here for her heart condition. Watch Jen and Shawn, Penn's parent, tell Penn's story. View Penn's Beautiful Heart Story transcript.

Treating a Pediatric Single Ventricle Anomaly    


The ductus arteriosus, a connection between the pulmonary artery and the aorta, usually closes within a few days after birth. Your pediatric cardiologist may use medicine to keep this connection open and blood flowing, as a temporary treatment for your child’s condition.


Often, your child needs surgery right after birth. Several surgical options exist. They can:

  • Restore connections between the heart, lungs and body, with a shunt
  • Improve circulation, using a band
  • Reroute the way blood travels through the heart and lungs
  • Replace the heart, with a heart transplant

We offer a special Home Single Ventricle Surveillance Program, where a nurse specialist closely follows babies with a single heart ventricle when they return home.

Lifelong Health Considerations

Your child may need to:

  • Have regular exams with a heart specialist
  • Take antibiotics before other medical or dental procedures to prevent a heart infection
  • Limit certain activities