Cyanosis (blue spells) in congenital heart disease

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Blue spells occur when a child's lungs are not receiving enough blood to carry oxygen to the rest of the body. Find out what to do in case of a blue spell.

Key points

Cyanosis means there may not be enough oxygen being carried to tissues in the body.
During cyanosis, your child may suddenly feel uncomfortable; be cranky or less alert; breathe faster and deeper; turn blue, especially around the mouth and face; faint or feel faint.
If your child is a baby, place them on their back and bring their knees up to touch their chest. Comfort and soothe them.
If your child is older, place them on their side and bring their knees up to their chest.
If cyanosis lasts longer than one minute and/or your child is less alert, or if your child faints, call 911.

Introduction

What is cyanosis (blue spell)?

Cyanosis, or blue spells, is when a reduced amount of blood flows into the lungs. Since blood carries oxygen, less oxygen is delivered to the body. As a result, a child's skin, face, tongue or lips may appear blue or bluish.

The colour is caused by high levels of reduced (deoxygenated) hemoglobin in the blood near the surface of the skin. Hemoglobin is the molecule that allows red blood cells to carry oxygen. Oxygenated hemoglobin (hemoglobin that is carrying oxygen) is bright red, while deoxygenated hemoglobin (hemoglobin that is not carrying oxygen) is bluish.

Cyanosis caused by congenital heart disease is often seen soon after birth, but sometimes it does not appear until a baby is older.

Signs and symptoms

Signs and symptoms of cyanosis

A child diagnosed with congenital heart disease, may be experiencing cyanosis if they show the following signs and symptoms:

suddenly feeling uncomfortable, cranky or less alert
fast or deep breathing
blue tinge to their skin, especially around the mouth and face
fainting or feeling like they are about to faint

Causes, risk factors and prevalence

What causes cyanosis?

Cyanosis may mean that the lungs are not oxygenating the blood properly, or that not enough oxygenated blood is getting to the body. Cyanosis can be seen in many conditions. It can occur because of breathing or lung problems, seizures, cold temperature, dehydration, severe anemia or shock in babies and children. It can also occur because of congenital heart disease.

The most common cause of cyanosis due to a heart problem is a sudden narrowing of the path between the heart and the lungs. This narrowing causes problems with blood flow to and/or from the lungs: this may be due to abnormal connections of the blood vessels between the heart and lungs, or obstruction of the blood vessels. This narrowing lowers the amount of blood going to the lungs, where blood gets oxygenated.

The children most often affected by cyanosis are those with tetralogy of Fallot (a form of congenital heart disease). However, cyanosis may also occur in children with other forms of heart disease in which blood flow to the lungs is reduced. Cyanosis can be seen with congenital heart conditions that allow oxygen-poor blood from the right side of the heart to mix with oxygen-rich blood in the left side of the heart, so the blood that is pumped out to the body is low in oxygen; these defects are known as right-to-left shunts

Congenital heart disease that causes cyanosis includes transposition of the great arteries, pulmonary atresia, tetralogy of Fallot, tricuspid atresia, hypoplastic left heart syndrome, truncus arteriosus and interrupted aortic arch.

Cyanosis in babies with some types of congenital heart disease is most common:

when they cry strongly
soon after waking
soon after a feeding
soon after a bowel movement
if they are dehydrated

Helping your child

Helping your child during a cyanotic or blue spell

If your child has a cyanotic or blue spell, do not panic. To help your child, follow these steps:

For babies

Place your baby on their back.
Bring the baby's knees up to touch the chest and hold them firmly in place. You can also do this while holding your baby.
Comfort and settle your baby by holding and rocking, giving a soother and gentle cuddling.
Remain calm. Your baby can sense when you are upset, which will make it harder to settle the baby.

For older children

Child on their side with knees raised to the chest and hands held under cheek

Place your child on their side, bringing the knees up to the chest and hold them firmly in place.
Some children will automatically squat during a blue spell. This is their way of bringing their knees to the chest on their own.
Make sure you can see your child's face so you can watch for colour changes and check the degree of alertness and length of the spell.

When to seek medical attention

When you should call a health-care provider

If this is your child's first blue spell, tell your child's health-care provider.
If the frequency of your child's blue spells increases, tell your child's cardiologist.
If the blue spell lasts longer than one minute, take your child to the nearest emergency department.

If the blue spell lasts longer than one minute and there is also a change in your child's level of alertness or if your child faints, call 911.

Your child may show physical changes when their condition is serious or when their condition gets worse. Parents and caregivers can learn how to spot these signs in order to seek help from a health-care provider.

Last updated: August 28th 2024