Before you begin reading about single ventricle heart defects, please read the explanation of how the normal heart works for a basic understanding of its structure and function.
The heart has four chambers. The upper chambers, called atria, receive blood flowing into the heart. The lower chambers, called ventricles, pump blood out of the heart.
A child with a single ventricle defect is born with a heart with only one ventricle that is large enough or strong enough to pump effectively. Single ventricle heart defects include:
Single ventricle heart defects are also referred to as single ventricle lesions or anomalies.
Our newest video demonstrates the advances in heart care for babies with single ventricle heart defects — from before birth through adulthood. Learn how early detection and diagnosis can make a world of difference in the lives of infants born with single ventricle heart defects.
In most cases, children with single ventricle heart defects require intensive medical intervention soon after birth. Symptoms vary depending on the severity and type of defect. They include:
In many cases, single ventricle defects are diagnosed before the baby is born through fetal echocardiography. The Fetal Heart Program at CHOP will monitor baby and mother throughout the pregnancy and create a plan for labor and care after the birth. CHOP has its own delivery unit, on the same floor as cardiac operating rooms and cardiac patient care units. Newborns can be in the care of pediatric cardiologists and specially trained cardiac nurses immediately. The Fetal Heart Program team will discuss delivery in the Garbose Family Special Delivery Unit with mother and family.
Sometimes single ventricle heart defects aren’t recognized until the baby is born. The newborn might look blue or have trouble breathing. The pediatrician at the birth hospital might recognize a heart murmur (an abnormal sound in the heart beat) and order an echocardiogram, which will show the defect.
In these cases, newborns are transported to CHOP by ambulance or helicopter and admitted to the Tabas Cardiac Intensive Care Unit (CICU).
The types of single ventricle heart defects are very different. For instance, in hypoplastic left heart syndrome (HLHS) the left side of the heart doesn’t work correctly, while in tricuspid atresia it is the right side. Even though the defects vary from child to child, the treatment is similar for all.
Single ventricle defects require a series of open heart procedures, over several years, called “staged reconstruction.” Surgeons reconfigure the heart and circulatory system.
A normal heart is a two-sided pump with four chambers. The right chambers receive deoxygenated blood or “blue” blood from veins and pump it to the lungs. The left chambers receive oxygenated blood or “red” blood from the lungs and pump it to the body.
After the operations for single ventricle defects, the heart functions like a one-sided pump with two chambers. The heart no longer receives deoxygenated blood from the veins. Instead, this blood flows directly to the lungs. The heart receives oxygenated blood from the lungs and pumps it to the body.
This is called Fontan circulation.
Here are brief descriptions of the surgeries required to create Fontan circulation:
The Cardiac Center team will explain the procedures to you in detail, based on your child’s heart anatomy.
Frequent appointments with your child’s cardiologist in infancy and early childhood are important to minimize risk factors associated with single ventricle heart defects. Your child will need a customized series of diagnostic tests between the planned stages of surgery.
Once the surgeries are complete, your child will have appointments with a pediatric cardiologist once a year or more frequently.
As “single ventricle survivors” get older, doctors are recognizing that, while some do fine, many experience complications, including lung, liver and gastrointestinal diseases. The Cardiac Center at CHOP created the Single Ventricle Survivorship Program to bring together doctors from different specialties to care for single ventricle survivors.
In addition, as a group, children with complex congenital heart defects who have had open heart surgery as infants are at a higher risk for neurodevelopmental issues when compared to children without congenital heart defects. The Cardiac Center at CHOP created the NeuroCardiac Care Program (NCCP) to provide evaluation, screening and clinical care for children with complex congenital heart disease who are at risk for neurodevelopmental problems.
Questions families often ask include:
Children with Fontan circulation must continue to see a cardiologist as an adult. We will help with the transition to an adult cardiologist.
The Philadelphia Adult Congenital Heart Center, a joint program of The Children's Hospital of Philadelphia and the University of Pennsylvania, meets the unique needs of adults who were born with heart defects.
Contact the Cardiac Center at The Children's Hospital of Philadelphia for a second opinion or for more information.
Reviewed by: David J. Goldberg, MD
Date: September 2013
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