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Neonatology · Dextro-Transposition of the Great Arteries

Why Do Babies on Prostaglandin E1 Need Intubation?

At a Glance

Prostaglandin E1 (PGE1) is a life-saving medication for babies with D-TGA, but it can temporarily suppress the brain's breathing center, causing pauses in breathing (apnea). Doctors often place a breathing tube to safely manage this side effect and secure the airway before heart surgery.

When a baby is born with dextro-transposition of the great arteries (D-TGA), they are given a life-saving medication called Prostaglandin E1 (PGE1). Delivered as a continuous infusion through an IV or an umbilical line, this medication keeps a fetal blood vessel called the ductus arteriosus open. This allows oxygen-poor and oxygen-rich blood to mix until heart surgery can be performed, which typically happens within the first week of life [1][2].

Seeing your newborn in the intensive care unit with a breathing tube (intubated) can be terrifying. It is natural to worry that this means their heart is failing. However, the breathing tube is frequently placed to safely manage a common side effect of PGE1 called apnea (pauses in breathing), rather than because of heart failure [2]. The tube is placed for airway safety, ensuring your baby breathes steadily while they wait for their operation. While the tube is in place, your baby will be given sedation medications to keep them comfortable, sleepy, and free from pain.

How PGE1 Affects Breathing

Breathing is automatically controlled by a part of the brain called the brainstem. As PGE1 travels through the bloodstream, it can interact with this control center. Research suggests that PGE1 alters chemical signals in the brainstem, which can temporarily suppress the central drive to breathe [3]. This causes the baby’s brain to briefly “forget” to trigger a breath, leading to an apneic event (apnea) [4][2]. On the monitors, an apneic event may look like a sudden drop in heart rate or oxygen levels, often triggering alarms before the care team intervenes.

Who is Most at Risk?

Respiratory depression (slower or paused breathing) is a recognized side effect, but it does not affect every infant.

  • In general, about 12% of babies receiving PGE1 experience respiratory depression [5].
  • The risk is significantly higher in premature babies or infants with a low birth weight (under 2.0 kg), reaching up to 42% [5][4].

To lower this risk, medical teams often use the lowest effective dose of the medication, which can help maintain the open blood vessel while minimizing the chance of apnea [6][2]. In some cases, respiratory stimulants like caffeine may also be considered to counter the medication’s effect on the brainstem [3].

Why Proactive Intubation is Used

Because apneic pauses can happen suddenly and without warning, doctors often take a “safety first” approach. This involves placing a breathing tube down the airway and connecting it to a ventilator—a process known as prophylactic intubation [7].

There are several reasons doctors choose to intubate a baby on PGE1:

  • Airway Security: If a baby stops breathing, the ventilator automatically steps in to provide oxygen [2].
  • Safe Transport: Babies with D-TGA often need to be moved to specialized pediatric cardiac centers. Placing a breathing tube guarantees that the airway is secure during the bumpy and unpredictable environment of an ambulance or helicopter transport [7].
  • Pre-Surgical Stability: The tube ensures the baby stays in the best possible condition, with optimal oxygen levels, as they head into heart surgery.

While the medication-induced apnea resolves once PGE1 is stopped, it is important to know that your baby will not have their breathing tube removed immediately after surgery. They will remain intubated for a period of time to safely recover from the stress of open-heart surgery. The care team will remove the tube only when your baby is stable and breathing strongly on their own.

Common questions in this guide

Why does my baby need a breathing tube while on Prostaglandin E1?
Prostaglandin E1 can cause your baby's brain to temporarily forget to breathe, resulting in pauses called apnea. A breathing tube is placed to ensure your baby receives a steady supply of oxygen and their airway remains secure until heart surgery.
Is my baby's heart failing because they are intubated?
No, seeing your newborn with a breathing tube does not mean their heart is failing. The tube is a proactive safety measure to manage medication-induced apnea and ensure safe oxygen levels before surgery.
Can the Prostaglandin E1 dose be adjusted to prevent breathing pauses?
Medical teams often use the lowest effective dose of Prostaglandin E1 to keep the necessary blood vessel open while minimizing the risk of apnea. In some cases, respiratory stimulants like caffeine are used to help maintain the baby's breathing drive.
Will my baby be in pain while the breathing tube is in place?
While the breathing tube is in place, your baby will receive continuous sedation medications. These medications are carefully managed to keep your newborn comfortable, sleepy, and free from pain in the intensive care unit.
When will the breathing tube be removed?
The breathing tube will remain in place during and immediately after the open-heart surgery to help your baby safely recover from the physical stress. The care team will remove the tube only when your baby is stable and breathing strongly on their own.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.Are you using the lowest effective dose of Prostaglandin E1 to keep the ductus arteriosus open while minimizing side effects?
  2. 2.What medications are you using to ensure my baby is comfortable and not in pain while the breathing tube is in place?
  3. 3.Is my baby's breathing tube placed purely as a precaution for PGE1 side effects, or are there other respiratory concerns?
  4. 4.Could caffeine or other respiratory stimulants be used to help manage my baby's breathing drive while on this medication?
  5. 5.What is the expected timeline for removing the breathing tube after their heart surgery?

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References

References (7)
  1. 1

    Effects of Prostaglandin E1 and Balloon Atrial Septostomy on Cerebral Blood Flow and Oxygenation in Newborns Diagnosed with Transposition of the Great Arteries.

    Cucerea M, Ognean ML, Pinzariu AC, et al.

    Biomedicines 2024; (12(9)) doi:10.3390/biomedicines12092018.

    PMID: 39335532
  2. 2

    Caffeine Citrate for the Prevention of Apnea Associated With Alprostadil Infusions.

    Higgins KL, Buck ML

    The journal of pediatric pharmacology and therapeutics : JPPT : the official journal of PPAG 2020; (25(3)):235-240 doi:10.5863/1551-6776-25.3.235.

    PMID: 32265607
  3. 3

    Caffeine prevents prostaglandin E1-induced disturbances in respiratory control in neonatal rats: implications for infants with critical congenital heart disease.

    Mitchell LJ, Mayer CA, Mayer A, et al.

    American journal of physiology. Regulatory, integrative and comparative physiology 2020; (319(2)):R233-R242 doi:10.1152/ajpregu.00316.2019.

    PMID: 32579854
  4. 4

    Low-dose prostaglandin E1 is safe and effective for critical congenital heart disease: is it time to revisit the dosing guidelines?

    Vari D, Xiao W, Behere S, et al.

    Cardiology in the young 2021; (31(1)):63-70 doi:10.1017/S1047951120003297.

    PMID: 33140712
  5. 5

    Side effects of therapy with prostaglandin E1 in infants with critical congenital heart disease.

    Lewis AB, Freed MD, Heymann MA, et al.

    Circulation 1981; (64(5)):893-8 doi:10.1161/01.cir.64.5.893.

    PMID: 7285304
  6. 6

    Effectiveness of Alprostadil for Ductal Patency.

    Gordon CM, Tan JT, Carr RR

    The journal of pediatric pharmacology and therapeutics : JPPT : the official journal of PPAG 2024; (29(1)):37-44 doi:10.5863/1551-6776-29.1.37.

    PMID: 38332962
  7. 7

    The safety of postnatal transport of newborns prenatally diagnosed with duct-dependent congenital heart disease.

    Shenoy RU, DiLorenzo M

    The journal of maternal-fetal & neonatal medicine : the official journal of the European Association of Perinatal Medicine, the Federation of Asia and Oceania Perinatal Societies, the International Society of Perinatal Obstetricians 2016; (29(12)):1911-4 doi:10.3109/14767058.2015.1067295.

    PMID: 26303726

This page provides educational information about neonatal care and D-TGA. It is for informational purposes only and does not replace professional medical advice from your pediatric cardiology team.

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