Conditions

Cyanotic Heart Disease

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What is cyanotic heart disease?

Cyanotic heart disease is the name for a group of heart defects that prevent your child’s body from getting enough oxygen through their blood. It is sometimes called by other names — critical heart disease (CCHD), cyanotic congenital heart disease, congenital cyanotic heart disease or cyanotic heart defect.

What causes cyanotic heart disease?

Drawing of a normal heart showing the 4 chambers, the valves that let blood flow in and out of the chambers, and the major blood vessels going to and from the heart

This is a normal heart. It sends oxygen-poor blood to the lungs and oxygen-rich blood to the body. In cyanotic heart disease, a heart defect changes how blood moves through the heart and lungs. As a result, the body does not get enough oxygen-rich blood.

Typically, the heart works this way:

The heart is made of 4 spaces, or chambers. The 2 on top are called atria, and the 2 on the bottom are ventricles.
Your body uses oxygen from your blood.
The blue (oxygen-poor) blood returns to the right atrium.
t goes into the right ventricle and is pumped to the lungs, where it picks up oxygen from your breathing.
Red (oxygen-rich) blood returns from the lungs to the left atrium.
It goes into the left ventricle and is pumped out to the body.
Heart valves act like doors. They let blood move from one place to the next and keep it from leaking backward. The heart has 4 valves: tricuspid valve, pulmonary valve, mitral valve and aortic valve.

In cyanotic heart disease, blood doesn’t move through the heart and lungs in the typical way. There may be a defect that lets blue and red blood mix, so the heart pumps some blue blood out to the body. A valve may be blocked, preventing the full amount of blood from being pumped to the lungs. Problems with the way blood moves from the lungs back into the heart can also cause low oxygen levels. Finally, severe heart defects can keep the chambers of the heart from developing fully ().

Conditions like these can allow blue blood to be pumped out to the body, or they can limit the overall blood flow to the body. In either case, your child’s body may not get as much oxygen as it needs.

Types of Cyanotic Heart Disease

There are three main types of cyanotic heart disease.

Right heart obstructive lesions

These are problems that affect blood flow between the heart and lungs, such as:

Left heart obstructive lesions

These are problems that affect blood flow between the heart and the rest of the body, such as:

Mixing lesions

These are problems that let red and blue blood mix in the heart, such as:

Heart Center at Seattle Children's

Our pediatric cardiologists and cardiac surgeons have more advanced training and experience operating and performing procedures on babies, children and teens than at any other children's hospital in the Pacific Northwest.

What are the symptoms of cyanotic heart disease?

If your child’s body is not getting enough red blood, they may have cyanosis. Cyanosis is the medical name for a change in the color of the skin caused by low oxygen in the blood. The skin may look blue or purple tinged, mottled (different shades or colors), grayish or paler than usual. The lips, mouth, gums, fingernails or toenails may look bluish.

Depending on the defect causing cyanotic heart conditions, children may have other symptoms, too, such as:

Trouble breathing, or fast or irregular breathing
Trouble feeding, sweating or crying while feeding, or getting very tired while feeding
Lower appetite, eating less or taking longer to feed than they used to
Not gaining as much weight or growing as much as is typical
Being more irritable than is typical
Becoming less active or feeling tired all the time
Fainting
Puffy face or puffiness around the eyes
Ends of the fingers or toes that are larger than typical, along with changes to the nails (clubbing)
Fast heart rate

How is cyanotic heart disease diagnosed?

Sometimes doctors can diagnose heart defects in a developing baby before birth. They might first see something different about your baby’s heart on a standard prenatal . To learn more, you might have a fetal echocardiogram (an ultrasound done to check the heart more closely). Seattle Children’s Fetal Care and Treatment Center team can care for you when you are pregnant if your developing baby has a known or suspected problem.

Often, babies who are not diagnosed prenatally are diagnosed as newborns, before they go home from their birth center, because they show symptoms. A simple newborn screening test () can also show their body is not getting as much oxygen as it needs. If this screening test shows a concern, your child’s team will do more detailed testing.

To diagnose cyanotic heart disease after birth, your child’s doctor will examine your child and use a stethoscope to listen to their heart and lungs.

The doctor will consider your child’s symptoms, their health history and your family health history.

Your child will also need tests that provide information about how their heart and blood vessels look and work. These may include

How is cyanotic heart disease treated?

Treatment for cyanotic heart disease depends on the heart defect and how serious the effects are. Some cyanotic infants will be very ill and need to stay in the hospital after birth to start treatment right away. Other children may not have signs or symptoms or need treatment at first, but they may need care later if their oxygen level drops too low over time. Your child’s care team can tell you more about the outlook for your child’s condition (prognosis).

Most of the time, children with cyanotic heart disease need surgery to fix the problem in their heart or change their blood flow. Some heart conditions can be treated with a cardiac catheterization procedure or a hybrid heart procedure, which combines surgery and catheterization.

Before your child has surgery, they may need other steps, including:

Oxygen therapy, such as through a tube under their nose (nasal cannula) or a breathing machine ()
Medicine to help their heart pump, control their heart rhythm, reduce extra fluid in their body or keep blood vessels open that normally close soon after birth

Children born with complex heart conditions are at risk for problems with the growth and development of their brain and central nervous system. At Seattle Children’s, we have a Cardiac Neurodevelopmental Clinic to assess your child and, if needed, create a plan to help them reach their full potential.

Why choose Seattle Children’s for cyanotic heart disease treatment?

When you come to Seattle Children’s, your child will be treated at one of the nation’s top Heart Centers, as ranked by U.S. News and World Report. We have the most experienced pediatric cardiologists and heart surgeons in the Pacific Northwest.

The experts you need are here
- The Heart Center team includes more than 40 pediatric cardiologists who diagnose and treat every kind of heart problem. We have treated many children with cyanotic heart disease, and we provide any ongoing care patients need as they grow into young adults.
- Our pediatric cardiac surgeons perform more than 500 procedures yearly. Each has experience correcting the conditions that can cause cyanotic heart disease in babies, children and teens. We can do surgery even for tiny newborns, which not all heart centers can do.
- For the smallest babies, who may be too small for standard surgery, our surgeons and cardiac catheterization specialists work together to perform hybrid procedures.
- We also have a pediatric cardiac anesthesia team and a Cardiac Intensive Care Unit ready to care for children who have heart surgery.
- Some children need a heart transplant. The heart transplant team at Seattle Children’s does many transplants each year for children with heart problems that cannot be treated with standard medical or surgical treatment. Read more about our Heart Transplant Program and our exceptional outcomes.
- Your child’s team includes other experts from Seattle Children’s based on their needs, like doctors who specialize in newborns (neonatologists) or lung health (pulmonologists).
Among the nation’s top programs
- Our surgical outcomes are among the best in the nation year after year.
Care from fetal diagnosis through young adulthood
- If your developing baby is diagnosed with a congenital heart defect before birth, our Fetal Care and Treatment Center team works closely with you and your family to plan and prepare for the care your baby will need.
- Your child’s treatment plan is custom-made. We plan and carry out their surgery based on the specific details of their heart defect. We closely check your child’s needs to make sure they get the care that is right for them at every age.
- We have a special Adult Congenital Heart Disease Program to meet your child’s long-term healthcare needs. This program, shared with the University of Washington, transitions your child to adult care when they are ready.
Support for your whole family
- We are committed to your child’s overall health and well-being and to helping your child live a full and active life.
- Whatever types of care your child needs, we will help your family through this experience. We will discuss your child’s condition and treatment options in ways you understand and involve you in every decision.
- Our Child Life specialists know how to help children understand their illnesses and treatments in ways that make sense for their age.
- Seattle Children’s has many resources, from financial to spiritual, to support your child and your family and make the journey as smooth as possible.
- Many children and families travel to Seattle Children’s for heart surgery or other care. We help you coordinate travel and housing so you can stay focused on your child.
- Read more about the supportive care we offer.
Research to improve care
- Seattle Children’s takes part in PC4, the Pediatric Cardiac Critical Care Consortium. This is a national group of leaders in treating children with serious heart conditions. PC4 focuses on advancing care by sharing and analyzing data to find what works best.
- We are part of a nationwide group of children’s hospitals called the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC). This group works to improve the outcomes and quality of life for children with hypoplastic left heart syndrome.
- Our experts strive to reduce the number of procedures your child may need. For example, we set goals to reduce the number of procedures a child is likely to need for interrupted aortic arch. After surgery for this condition, some patients develop a narrow spot where their aorta was repaired. This puts extra stress on their heart. It may mean they need a cardiac catheterization procedure or another surgery to open the aorta again. By carefully studying the steps in our surgical approach and the results for our patients, we designed a standard way to repair the aorta with lower risk of getting a narrow spot in the future.
- For children with tetralogy of Fallot, doctors at Seattle Children’s take part in national and international research to study less invasive ways to replace the pulmonary valve, if needed later in life, through cardiac catheterization. With these options, children may be able to avoid further open-heart surgery and recover faster.
- Learn more about heart research at Seattle Children’s.