What are cyanotic spells?

Cyanotic spells, also known as blue spells or blue baby syndrome, are episodes where a baby turns a bluish or purplish color due to a lack of oxygenated blood flow. These spells are typically seen in infants with certain congenital heart defects.

The spell occurs when there is a temporary imbalance or shunting of deoxygenated blood from the right side to the left side of the heart and into the systemic circulation. This decrease in oxygenated blood causes the bluish discoloration of the skin, lips and nails.

Cyanotic spells are associated with heart defects that involve:

• Obstruction to blood flow from the right ventricle to the lungs (e.g. tetralogy of Fallot)
• Abnormal blood vessel connections between the aorta and pulmonary artery (e.g. truncus arteriosus)

During a spell, the infant may:

• Turn a blue/purple color, especially around the lips and nailbeds
• Become agitated, irritable or cry intensely
• Have breathing difficulties or respiratory distress
• Appear limp or lose consciousness in severe cases

Spells can be provoked by anything that increases the infant’s oxygen demand, like crying, feeding or defecation. They typically resolve on their own within 10-15 minutes but require emergency care if prolonged.

Positioning the baby in a knee-to-chest position, giving oxygen, IV fluids and medications to improve heart function may help terminate the spell.

Cyanotic spells are considered a medical emergency as they indicate significantly decreased oxygenation. Surgical repair by your healthcare provider of the underlying heart defect is eventually required.

What are pallid spells?

Pallid spells, also known as pale tet spells or pallid syncope, are a type of neurological event that occurs primarily in infants and young children with severe congenital heart defects. The main features are:

Pallor/Paleness

• The child’s skin, lips and mucous membranes turn very pale or white in color due to reduced blood flow and oxygenation.

Loss of Muscle Tone

• The child becomes floppy, limp and lacks muscle tone during the spell.
• May lose consciousness or appear to “faint.”

Unresponsiveness

• The child is unresponsive and difficult to arouse during the episode.
• May have a blank stare or appear unconscious.

Rapid Onset and Recovery

• Pallid spells come on abruptly without warning.
• They typically resolve spontaneously within 1-2 minutes.

Associated Symptoms

• Sweating, dilated pupils, nausea/vomiting may occur with some spells.

Pallid spells are caused by a sudden change or interruption in blood flow through congenital heart defects like tetralogy of Fallot. This reduces oxygenated blood supply to the brain, causing the symptoms.

While frightening, isolated brief spells are not immediately life-threatening if the child recovers quickly. However, frequent or prolonged episodes require emergency treatment.

Potential triggers include crying, defecation, fear or any event that increases the infant’s oxygen demand.

Pallid spells are less common than cyanotic (blue) spells seen with some heart defects. They generally occur in infants under 2 years old before surgical heart repair.

What is the treatment for breath-holding spells?

Breath-holding spells in children are generally benign and self-resolving events that do not require specific medical treatment. However, some management strategies can help prevent or respond to these spells:

1. Stay calm – Remaining calm and avoiding giving the child excessive attention during a spell can discourage inadvertent reinforcement of the behavior.
2. Distraction – Distracting the child with a toy, book, or activity when a spell seems imminent may help abort it.
3. Gentle stimulation – Lightly tapping, blowing in the face, or sprinkling water on the face when the child loses consciousness can trigger a gasp and resumption of breathing.
4. Lay child down – If unconsciousness occurs, laying the child down flat can help blood flow return to the brain faster.
5. Do not restraint – Avoid restraining the child as this may prolong the spell.
6. Observe and monitor – Most spells are brief, but seek emergency care if unconsciousness persists beyond 1 minute or there are seizure-like movements.
7. Identify triggers – Note circumstances that trigger spells to help avoid or prepare for future events.
8. Behavior modification – For frequent, attention-seeking spells, using positive reinforcement for desired behaviors may help reduce their occurrence over time.
9. Iron supplementation – For iron deficiency anemia, treating the underlying condition may reduce spell frequency.
10. Anti-anxiety medication – In very severe, refractory cases, medications like selective serotonin reuptake inhibitors may be prescribed.

While alarming, breath-holding spells are usually outgrown by ages 5-8 and do not cause lasting harm. Educating parents on proper response and avoiding reinforcement of the behavior is key.