No parent wants to find out that their child has a heart condition, but fortunately, children with ventricle defects can be helped with many different types of research-backed treatments and operations. If your child was just diagnosed with a single ventricle heart defect, or you suspect that your child might have symptoms that point to a congenital heart condition, it’s important to speak to your doctor as soon as possible.
In the meantime, learn more about single ventricle defects and treatments for these heart conditions that are often diagnosed within the first weeks of a child’s life.
What Is a Single Ventricle Heart Defect?
The heart consists of four chambers: two upper and two lower. The upper chambers are called the atria, and the lower chambers are the ventricles. As the heart pumps, the atria are filled with blood, and the ventricles send the blood out of the heart and back into the bloodstream. If a person has any problem with any one of these four chambers, it could spell trouble for their heart, their energy level, and even their life.
Single ventricle physiology defects are congenital conditions that develop well before a baby is even born. In fact, they tend to occur around the time the baby’s heart forms — during the first two months of pregnancy.
As the baby’s heart grows, blood passes through it. The single abnormal ventricle doesn’t have the opportunity to practice pumping blood like the other four chambers, so it is not able to fully develop. There are several different classifications of single ventricle defects, but they all originate in the womb.
Why Is a Single Ventricle Defect a Problem?
A single ventricle defect poses a major problem for babies born with it, as their hearts aren’t able to circulate enough oxygen in their bloodstream to sustain their life. A baby or young child might appear blue, have a higher risk for heart infections like endocarditis, or experience blood clots and abnormal heart rhythms as a result of their one good ventricle working too hard. Endocarditis is a major risk for many people who have had heart surgery, and these patients often need to take antibiotics before undergoing dental procedures to reduce the risk to their heart.
Even as an adult, someone who had heart surgery as a child might have one or more chronic health problems. They often won’t be able to exercise like their peers, and they might need to take several medications every day to remain healthy.
What Are Different Types of Single Ventricle Defects?
Though a single ventricle (as opposed to both ventricles) is involved in all cases of this condition, the ventricle isn’t always affected in the same way. The following conditions are the most common single ventricle defects.
Hypoplastic left heart syndrome (HLHS). In HLHS, both the left ventricle and the aorta branching off this ventricle are too small. The aorta is a large artery that delivers oxygenated blood to the body, and when it doesn’t work correctly, it can cause major problems and often result in the baby’s death if it isn’t immediately treated within the first few days to weeks of life.
Tricuspid atresia. In this type of atresia, the body is missing the tricuspid valve. This valve brings the blood from the body into the heart to receive more oxygen. Not having this valve poses a problem for the body because it isn’t able to replenish the blood with enough oxygen to sustain the body.
Pulmonary atresia. In this condition, the child is born without a pulmonary valve. The pulmonary valve functions as a gate between the heart and the rest of the body as well as between the left ventricle and right atrium. Children with this condition have trouble getting enough oxygen-rich blood into their lungs because this blood mixes with oxygen-poor blood due to the absence of the pulmonary valve.
How Is a Single Ventricle Defect Diagnosed?
When a baby has a congenital heart condition, it may be apparent as soon as they're born. The baby might look ashen or blue, depending on the type of ventricle defect.
Older babies, toddlers, and children are examined by a cardiologist to determine whether or not the problem is in the heart or somewhere else. Your child might undergo several different types of tests, like a physical examination, a chest X-ray, an electrocardiogram, or a cardiac MRI.
What Are Single Ventricle Treatment Options?
While there is no overall cure for this condition, single ventricle prognosis isn't always bleak. Your child might undergo several surgeries or a heart transplant if the condition worsens over time.
A heart transplant is the closest thing to a cure — but your child might have a difficult road to recovery, and they will need to take medication to prevent their body from rejecting the new heart. Your cardiologist will probably discuss one or more of the following options with you.
Operation. Depending on the type of single ventricle problem, your child will probably undergo at least one operation to treat it. Surgeons will place your child under anesthesia to place a catheter, or a small tube, to help the blood in your child’s heart flow where it's supposed to flow. Another option is called Fontan surgery: During this procedure, the surgeon will reroute the oxygen-poor blood through the pulmonary artery to give it more oxygen.
Heart transplant. Sometimes the only way to fix a ventricle defect is to replace the entire heart. If your doctor has recommended a pediatric heart transplant, there’s a good chance that your child will feel much better and be able to live a healthy, productive life afterward while taking medication to make sure that their body doesn’t reject the heart. You and your child will meet with several professionals involved in the transplant process, including the following people:
- A cardiologist (you likely already know this doctor from previous appointments)
- Heart surgeons
- Nurses
- A transplant coordinator
- Mental health professionals like social workers and child psychologists who will counsel you and your child throughout the transplant process
Can a Child With a Single Ventricle Defect Live a Normal Life?
A child with a single ventricle defect that has not yet been repaired by surgery is in danger of developing serious health problems and even dying (especially in the case of hypoplastic left heart syndrome) if the condition is not treated.
It’s possible to live into adulthood with this type of condition. Most kids with single ventricle defects have multiple surgeries in childhood, and their condition is closely monitored for years.
Teens and adults should follow up with a cardiologist regularly throughout their lives to make sure that the results of their childhood surgeries are still benefiting them. Living a full life with a heart condition is possible, but it’s good to keep in mind that your child may need to be more cautious than other children when it comes to running, playing sports, and roughhousing.