Medical Conditions

Persistent Pulmonary Hypertension of the Newborn (PPHN)

Medically reviewed by Dr. Michael Okonkwo, MD, FAAP · Board-Certified Neonatologist

Content reviewed against published AAP, NIH, ATS guidelines

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If your baby has been diagnosed with or you suspect persistent pulmonary hypertension of the newborn (pphn), here is what the evidence says.

The short answer

Persistent pulmonary hypertension of the newborn (PPHN) occurs when the blood vessels in the lungs fail to relax after birth, keeping blood pressure in the lungs abnormally high and preventing adequate oxygen exchange. This causes severe respiratory distress and cyanosis (blue skin) typically within the first 24 hours of life. PPHN is a medical emergency treated in the NICU with inhaled nitric oxide, high-frequency ventilation, and in severe cases, ECMO (extracorporeal membrane oxygenation).

Key takeaways

  • Persistent pulmonary hypertension of the newborn (PPHN) occurs when the blood vessels in the lungs fail to relax after birth, keeping blood pressure in the lungs abnormally high and preventing adequate oxygen exchange. This causes severe respiratory distress and cyanosis (blue skin) typically within the first 24 hours of life. PPHN is a medical emergency treated in the NICU with inhaled nitric oxide, high-frequency ventilation, and in severe cases, ECMO (extracorporeal membrane oxygenation).
  • Usually normal when: Your newborn had a brief period of blue hands and feet (acrocyanosis) after birth that resolved quickly, which is a normal finding in healthy newborns
  • Call your doctor if: Your newborn appears blue around the lips, tongue, or trunk and is breathing rapidly, grunting, or having difficulty breathing, as this could indicate PPHN or another critical condition requiring emergency evaluation
  • Varies by age — see the age-by-age breakdown below
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Parents everywhere have the same worry. You are doing the right thing by looking into it.

What Parents Should Know

According to AAP, NIH, ATS guidelines, persistent pulmonary hypertension of the newborn (PPHN) occurs when the blood vessels in the lungs fail to relax after birth, keeping blood pressure in the lungs abnormally high and preventing adequate oxygen exchange. This causes severe respiratory distress and cyanosis (blue skin) typically within the first 24 hours of life. PPHN is a medical emergency treated in the NICU with inhaled nitric oxide, high-frequency ventilation, and in severe cases, ECMO (extracorporeal membrane oxygenation). At 0-24 hours, pPHN most commonly presents within the first hours to the first day of life. The newborn may appear blue (cyanotic), breathe rapidly, grunt, flare their nostrils, or have chest retractions. Oxygen saturation levels remain low despite supplemental oxygen. Risk factors include meconium aspiration, pneumonia, sepsis, congenital diaphragmatic hernia, and maternal use of certain medications (such as NSAIDs or SSRIs in late pregnancy). The diagnosis is confirmed by echocardiography showing elevated pulmonary pressures and right-to-left shunting of blood. It is generally considered normal when your newborn had a brief period of blue hands and feet (acrocyanosis) after birth that resolved quickly, which is a normal finding in healthy newborns. However, you should contact your pediatrician promptly if your newborn appears blue around the lips, tongue, or trunk and is breathing rapidly, grunting, or having difficulty breathing, as this could indicate PPHN or another critical condition requiring emergency evaluation.

Sources: [1], [2], [3]

Normal vs. Concerning

Usually Normal
Worth Discussing
Your newborn had a brief period of blue hands and feet (acrocyanosis) after birth that resolved quickly, which is a normal finding in healthy newborns
Your newborn appears blue around the lips, tongue, or trunk and is breathing rapidly, grunting, or having difficulty breathing, as this could indicate PPHN or another critical condition requiring emergency evaluation
Your baby had mild respiratory transition after birth that resolved within a few hours without requiring significant intervention
Your baby recently discharged after PPHN treatment develops worsening breathing difficulty, new cyanosis, or poor feeding, which could indicate rebound pulmonary hypertension
Your baby's oxygen levels are consistently normal on pulse oximetry screening before hospital discharge
Your newborn's oxygen saturation is consistently low on pulse oximetry, which is a sign of a potentially life-threatening condition requiring immediate medical attention

When to Seek Immediate Care

  • Your newborn appears blue around the lips, tongue, or trunk and is breathing rapidly, grunting, or having difficulty breathing, as this could indicate PPHN or another critical condition requiring emergency evaluation
  • Your baby recently discharged after PPHN treatment develops worsening breathing difficulty, new cyanosis, or poor feeding, which could indicate rebound pulmonary hypertension
  • Your newborn's oxygen saturation is consistently low on pulse oximetry, which is a sign of a potentially life-threatening condition requiring immediate medical attention

By Age

What to expect by age

0-24 hours

PPHN most commonly presents within the first hours to the first day of life. The newborn may appear blue (cyanotic), breathe rapidly, grunt, flare their nostrils, or have chest retractions. Oxygen saturation levels remain low despite supplemental oxygen. Risk factors include meconium aspiration, pneumonia, sepsis, congenital diaphragmatic hernia, and maternal use of certain medications (such as NSAIDs or SSRIs in late pregnancy). The diagnosis is confirmed by echocardiography showing elevated pulmonary pressures and right-to-left shunting of blood.

1-7 days

Babies with PPHN require intensive care in the NICU. Treatment focuses on lowering pulmonary vascular resistance and improving oxygenation. Inhaled nitric oxide is the primary targeted therapy and works by selectively dilating the lung blood vessels. High-frequency oscillatory ventilation may be used to optimize lung expansion. If the baby does not respond to these treatments, ECMO (a heart-lung bypass machine) may be needed as a rescue therapy. Most babies with PPHN who survive the acute phase recover well.

1-4 weeks

As PPHN resolves, babies are gradually weaned from respiratory support, nitric oxide, and supplemental oxygen. The weaning process must be done carefully, as too-rapid reduction can cause rebound pulmonary hypertension. Some infants may need supplemental oxygen at home for a period after discharge. Follow-up echocardiography is performed to confirm resolution of elevated pulmonary pressures. Parents should be counseled about the importance of follow-up appointments to monitor respiratory and developmental progress.

1 month+

Most babies who recover from PPHN do well long-term, though some may have ongoing respiratory issues or developmental concerns depending on the severity and duration of their illness. Babies who required ECMO or had prolonged periods of low oxygen should have developmental follow-up. Hearing screening should be repeated, as some PPHN treatments and the condition itself can affect hearing. If your baby had PPHN, keeping all follow-up appointments is important for monitoring their continued recovery.

What to Tell Your Pediatrician

  • Describe when you first noticed persistent pulmonary hypertension of the newborn (pphn) and how it has changed over time.
  • Note your baby's current age and which age-specific patterns you are seeing.
  • Mention if your baby who recovered from PPHN seems to breathe faster than expected or has intermittent episodes of looking dusky during feeds or crying.
  • Mention if your baby had PPHN and you are concerned about their developmental progress at follow-up visits.
  • Let your doctor know if you have noticed any related concerns, such as changes in feeding, sleep, or movement patterns.
  • Bring a list of any questions or observations you want to discuss at the appointment.

What Should You Do?

When to take action

Probably normal when...
  • Your newborn had a brief period of blue hands and feet (acrocyanosis) after birth that resolved quickly, which is a normal finding in healthy newborns
  • Your baby had mild respiratory transition after birth that resolved within a few hours without requiring significant intervention
  • Your baby's oxygen levels are consistently normal on pulse oximetry screening before hospital discharge
Mention at your next visit when...
  • Your baby who recovered from PPHN seems to breathe faster than expected or has intermittent episodes of looking dusky during feeds or crying
  • Your baby had PPHN and you are concerned about their developmental progress at follow-up visits
  • You are pregnant and taking medications that have been associated with PPHN risk and want to discuss this with your obstetrician
Act now when...
  • Your newborn appears blue around the lips, tongue, or trunk and is breathing rapidly, grunting, or having difficulty breathing, as this could indicate PPHN or another critical condition requiring emergency evaluation
  • Your baby recently discharged after PPHN treatment develops worsening breathing difficulty, new cyanosis, or poor feeding, which could indicate rebound pulmonary hypertension
  • Your newborn's oxygen saturation is consistently low on pulse oximetry, which is a sign of a potentially life-threatening condition requiring immediate medical attention

What You Can Do at Home

  • Keep track of when you notice persistent pulmonary hypertension of the newborn (pphn) — noting the time of day, duration, and any triggers can help your pediatrician.
  • Remember that your newborn had a brief period of blue hands and feet (acrocyanosis) after birth that resolved quickly, which is a normal finding in healthy newborns — this is generally within the range of normal.
  • At 0-24 hours, focus on observation rather than intervention unless your pediatrician advises otherwise.
  • Follow any care instructions from your pediatrician. Keep a written log of symptoms to bring to appointments.
  • While monitoring at home, seek immediate care if your newborn appears blue around the lips, tongue, or trunk and is breathing rapidly, grunting, or having difficulty breathing, as this could indicate PPHN or another critical condition requiring emergency evaluation.

Frequently asked questions

Is persistent pulmonary hypertension of the newborn (pphn) normal?
Persistent pulmonary hypertension of the newborn (PPHN) occurs when the blood vessels in the lungs fail to relax after birth, keeping blood pressure in the lungs abnormally high and preventing adequate oxygen exchange. This causes severe respiratory distress and cyanosis (blue skin) typically within the first 24 hours of life. PPHN is a medical emergency treated in the NICU with inhaled nitric oxide, high-frequency ventilation, and in severe cases, ECMO (extracorporeal membrane oxygenation).
When should I call the doctor about persistent pulmonary hypertension of the newborn (pphn)?
Your newborn appears blue around the lips, tongue, or trunk and is breathing rapidly, grunting, or having difficulty breathing, as this could indicate PPHN or another critical condition requiring emergency evaluation Your baby recently discharged after PPHN treatment develops worsening breathing difficulty, new cyanosis, or poor feeding, which could indicate rebound pulmonary hypertension Your newborn's oxygen saturation is consistently low on pulse oximetry, which is a sign of a potentially life-threatening condition requiring immediate medical attention
When is persistent pulmonary hypertension of the newborn (pphn) normal?
Your newborn had a brief period of blue hands and feet (acrocyanosis) after birth that resolved quickly, which is a normal finding in healthy newborns Your baby had mild respiratory transition after birth that resolved within a few hours without requiring significant intervention Your baby's oxygen levels are consistently normal on pulse oximetry screening before hospital discharge
What causes persistent pulmonary hypertension of the newborn (pphn)?
Persistent pulmonary hypertension of the newborn (PPHN) occurs when the blood vessels in the lungs fail to relax after birth, keeping blood pressure in the lungs abnormally high and preventing adequate oxygen exchange. This causes severe respiratory distress and cyanosis (blue skin) typically within the first 24 hours of life. PPHN is a medical emergency treated in the NICU with inhaled nitric oxide, high-frequency ventilation, and in severe cases, ECMO (extracorporeal membrane oxygenation). Common explanations include: Your newborn had a brief period of blue hands and feet (acrocyanosis) after birth that resolved quickly, which is a normal finding in healthy newborns. Your baby had mild respiratory transition after birth that resolved within a few hours without requiring significant intervention.
What should I mention to my pediatrician about persistent pulmonary hypertension of the newborn (pphn)?
You should mention persistent pulmonary hypertension of the newborn (pphn) at your next visit if: Your baby who recovered from PPHN seems to breathe faster than expected or has intermittent episodes of looking dusky during feeds or crying. Your baby had PPHN and you are concerned about their developmental progress at follow-up visits. You are pregnant and taking medications that have been associated with PPHN risk and want to discuss this with your obstetrician.
Is persistent pulmonary hypertension of the newborn (pphn) normal at 0-24 hours?
PPHN most commonly presents within the first hours to the first day of life. The newborn may appear blue (cyanotic), breathe rapidly, grunt, flare their nostrils, or have chest retractions. Oxygen saturation levels remain low despite supplemental oxygen. Risk factors include meconium aspiration, pneumonia, sepsis, congenital diaphragmatic hernia, and maternal use of certain medications (such as NSAIDs or SSRIs in late pregnancy). The diagnosis is confirmed by echocardiography showing elevated pulmonary pressures and right-to-left shunting of blood.
Is persistent pulmonary hypertension of the newborn (pphn) normal at 1-7 days?
Babies with PPHN require intensive care in the NICU. Treatment focuses on lowering pulmonary vascular resistance and improving oxygenation. Inhaled nitric oxide is the primary targeted therapy and works by selectively dilating the lung blood vessels. High-frequency oscillatory ventilation may be used to optimize lung expansion. If the baby does not respond to these treatments, ECMO (a heart-lung bypass machine) may be needed as a rescue therapy. Most babies with PPHN who survive the acute phase recover well.
Should I go to the ER for persistent pulmonary hypertension of the newborn (pphn)?
Seek emergency care if your newborn appears blue around the lips, tongue, or trunk and is breathing rapidly, grunting, or having difficulty breathing, as this could indicate PPHN or another critical condition requiring emergency evaluation, or if your baby recently discharged after PPHN treatment develops worsening breathing difficulty, new cyanosis, or poor feeding, which could indicate rebound pulmonary hypertension. When in doubt, call your pediatrician's after-hours line for guidance.
Does persistent pulmonary hypertension of the newborn (pphn) go away on its own?
In many cases, persistent pulmonary hypertension of the newborn (pphn) resolves on its own, especially when your newborn had a brief period of blue hands and feet (acrocyanosis) after birth that resolved quickly, which is a normal finding in healthy newborns. By 1 month+, most babies who recover from PPHN do well long-term, though some may have ongoing respiratory issues or developmental concerns depending on the severity and duration of their illness. Babies who required ECMO or had prolonged periods of low oxygen should have developmental follow-up. Hearing screening should be repeated, as some PPHN treatments and the condition itself can affect hearing. If your baby had PPHN, keeping all follow-up appointments is important for monitoring their continued recovery.

References

  1. [1]Steinhorn RH. Neonatal Pulmonary Hypertension. Pediatric Critical Care Medicine, 2010. Referenced in AAP NeoReviews. AAP
  2. [2]National Heart, Lung, and Blood Institute. Persistent Pulmonary Hypertension of the Newborn. NIH, 2023. NIH
  3. [3]Abman SH, et al. Pediatric Pulmonary Hypertension: Guidelines from the American Heart Association and American Thoracic Society. Circulation, 2015. ATS

Doctor Visit Checklist

Bring this checklist to your next pediatrician visit to discuss Persistent Pulmonary Hypertension of the Newborn (PPHN).

Things to mention

  • Describe when you first noticed persistent pulmonary hypertension of the newborn (pphn) and how it has changed over time.
  • Note your baby's current age and which age-specific patterns you are seeing.
  • Mention if your baby who recovered from PPHN seems to breathe faster than expected or has intermittent episodes of looking dusky during feeds or crying.
  • Mention if your baby had PPHN and you are concerned about their developmental progress at follow-up visits.
  • Let your doctor know if you have noticed any related concerns, such as changes in feeding, sleep, or movement patterns.
  • Bring a list of any questions or observations you want to discuss at the appointment.

Observations to share

  • Your baby who recovered from PPHN seems to breathe faster than expected or has intermittent episodes of looking dusky during feeds or crying
  • Your baby had PPHN and you are concerned about their developmental progress at follow-up visits
  • You are pregnant and taking medications that have been associated with PPHN risk and want to discuss this with your obstetrician

Urgent signs to report immediately

  • Your newborn appears blue around the lips, tongue, or trunk and is breathing rapidly, grunting, or having difficulty breathing, as this could indicate PPHN or another critical condition requiring emergency evaluation
  • Your baby recently discharged after PPHN treatment develops worsening breathing difficulty, new cyanosis, or poor feeding, which could indicate rebound pulmonary hypertension
  • Your newborn's oxygen saturation is consistently low on pulse oximetry, which is a sign of a potentially life-threatening condition requiring immediate medical attention

My notes

From ismybabyalright.com — free, evidence-based baby health guides

All content follows our editorial policy and is reviewed against published clinical guidelines.

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Bottom line

Most cases of persistent pulmonary hypertension of the newborn (pphn) are normal. Talk to your pediatrician if your newborn appears blue around the lips, tongue, or trunk and is breathing rapidly, grunting, or having difficulty breathing, as this could indicate pphn or another critical condition requiring emergency evaluation.

Trust your instincts. If something feels wrong, reach out to your pediatrician. Worrying about your baby means you care — that is a good thing.

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