Single Ventricle Heart Defects
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.
- What is a single ventricle heart defect?
- What are the symptoms?
- How is it diagnosed?
- What are the treatment options?
- What kind of follow-up care is required?
What is a single ventricle heart defect?
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 defects include hypoplastic left heart syndrome, tricuspid atresia, double inlet left ventricle, some heterotaxy defects and others.
These are also referred to as single ventricle lesions or anomalies.
What are the symptoms?
In most cases, children with single ventricle defects require intensive medical intervention soon after birth. Symptoms vary depending on the severity and type of defect. They include:
- blue or purple tint to lips, skin and nails (cyanosis)
- difficulty breathing
- difficulty feeding
- lethargy (sleepy or unresponsive)
How is a single ventricle defect diagnosed?
In many cases, single ventricle defects are diagnosed before the baby is born, through fetal echocardiography. The Fetal Heart Program at Children’s Hospital will monitor baby and mother throughout the pregnancy and create a plan for labor and care after the birth. Children’s Hospital 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 Children’s Hospital by ambulance or helicopter and admitted to the Tabas Cardiac Intensive Care Unit (CICU).
What are the treatment options?
The types of single ventricle 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 body’s 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 first operation will occur within several days of birth. The purpose is to ensure that blood-flow is controlled enough to prevent damage to the heart and lungs, and that enough blood is reaching the lungs to keep the child alive until the second operation. There are different surgical procedures for this, depending on the type of heart defect.
- The second operation, called the hemi-Fontan or Glenn operation, usually occurs within six months of birth. During this surgery the superior vena cava, a large vein that carries deoxygenated blood from the upper body into the heart, is disconnected from the heart and attached to the pulmonary artery. After this operation, deoxygenated blood from the upper body goes to the lungs without passing through the heart.
- The third operation, called the Fontan, occurs at approximately one-and-a-half to 3 years of age. During this surgery the inferior vena cava, a large vein that carries deoxygenated blood from the lower body into the heart, is disconnected from the heart and attached to the pulmonary artery. After this operation, all of the deoxygenated blood from the body goes to the lungs without passing through the heart.
The Cardiac Center team will explain the procedures to you in detail, based on your child’s heart anatomy.
What kind of follow-up care is required?
Through 18
Frequent appointments with your child’s cardiologist in infancy and early childhood are important to minimize risk factors associated with single ventricle 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.
Questions families often ask include:
- Will my child need more surgery? It is possible that your child will require additional surgical or catheter therapies, or in rare cases heart transplantation.
- Will my child have to be on medication? Most children will not. We recommend some patients take aspirin every day to “thin” the blood.
- Will my child be allowed to be active or play sports? In most cases, we tell parents to allow the child to do as much as he or she is able to do. Most children will self-limit (stop activity if it begins to stress the body). There are certain sports and activities to avoid. We will discuss this with you based on your child’s unique situation.
- What is my child’s expected life span? We don’t know the expected life span of a person with Fontan circulation. Forty years ago children with single ventricle defects didn’t survive past infancy. Because of advances in surgery, medicine and intensive care capabilities, today the majority survive. There are thousands of people in their twenties and thirties with Fontan circulation. The Single Ventricle Survivorship Program at Children’s Hospital is focused on the care of Fontan patients and research into new treatments, quality of life and life span.
Into adulthood
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:
Jack Rychik, MD
David Goldberg, MD
Date: January 2010
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