Persistent Pulmonary Hypertension of the Newborn (PPHN): A Clinical Guide
Updated: 2 days ago
Medically reviewed by Dr. Baraa Alnahhal, MD · Last reviewed: October 2026
Persistent pulmonary hypertension of the newborn (PPHN) is a life-threatening breathing problem that occurs when a baby's circulation does not adapt to life outside the womb. The lung blood vessels do not open enough, which limits the oxygen that reaches the brain and other organs [1]. It affects about 2 in every 1,000 live births and needs immediate intensive care, often with specialized ventilation and nitric oxide. Most babies recover within weeks, but long-term follow-up is essential for survivors [1].
Quick Answer: What is persistent pulmonary hypertension of the newborn (PPHN)?
Persistent pulmonary hypertension of the newborn (PPHN) is a life-threatening condition in which a newborn's lung blood vessels stay constricted after birth, so blood cannot pick up enough oxygen. It occurs in 2 out of every 1,000 live births and causes rapid breathing and bluish skin. Treatment includes oxygen, nitric oxide and ventilation, and most babies survive. [1]
What the research says
Inhaled nitric oxide reduced the need for ECMO but not the death rate. A Cochrane review of 17 randomized trials in term and near-term infants with hypoxic respiratory failure found that inhaled nitric oxide reduced the combined outcome of death or ECMO use (high-quality evidence). The reduction came from less ECMO use (number needed to treat for one additional benefit, 5.3), while mortality was not affected. Oxygenation improved in about 50% of treated infants, with the oxygenation index falling by a mean of 15.1 within 30 to 60 minutes. Outcomes were not improved in babies with diaphragmatic hernia, and disability and deafness rates were similar in treated and untreated survivors (Barrington et al., Cochrane Database Syst Rev, 2017).
Late-pregnancy SSRI exposure was linked to PPHN, but the absolute risk stayed low. A meta-analysis of seven studies found that SSRI exposure in early pregnancy was not associated with PPHN (odds ratio 1.23; 95% CI, 0.58 to 2.60), while exposure in late pregnancy was (odds ratio 2.50; 95% CI, 1.32 to 4.73). The absolute risk difference was 2.9 to 3.5 per 1,000 infants, so an estimated 286 to 351 women would need to take an SSRI in late pregnancy for one additional case to occur (Grigoriadis et al., BMJ, 2014).
Bedside echocardiography allows early, definitive diagnosis. A review describes PPHN as a disorder of circulatory transition with high pulmonary vascular resistance and right-to-left shunting outside the lungs that causes low blood oxygen. It notes that easy access to bedside echocardiography helps establish an early diagnosis, understand the hemodynamics, and monitor the response to treatment (Singh & Lakshminrusimha, Clin Perinatol, 2021).
Research summaries are based on article abstracts retrieved from PubMed.
How does newborn circulation work, and what is PPHN?
During pregnancy, a fetus gets oxygen through the placenta and umbilical cord rather than the lungs. In this fetal state the pulmonary arteries are mostly closed, so very little blood goes to the lungs and blood bypasses them to flow to the rest of the body. After birth, the first breath should open these vessels so blood can flow into the lungs and pick up oxygen [1].
PPHN occurs when this transition fails. The lung blood vessels do not open enough, which limits how much oxygen reaches the baby's brain, heart, kidneys, and gut. PPHN is a medical emergency that can happen shortly after birth [1].
What are the symptoms and warning signs of PPHN?
Symptoms of PPHN are often immediate and severe, so healthcare providers watch newborns closely for signs of breathing distress and poor oxygenation [1].
Symptom Category | Clinical Indicators |
Respiratory | Rapid or slow breathing, grunting, and chest retractions. |
Circulatory | Rapid heart rate and extra or abnormal heart sounds (heart murmur). |
Oxygenation | Bluish skin color (cyanosis) and low blood oxygen levels. |
Systemic | Low blood pressure (hypotension). |
What causes PPHN in newborns, and who is at risk?
Researchers do not know the exact cause of PPHN. Incorrectly developed or underdeveloped lung blood vessels, or an inability of the vessels to open after birth, may cause the condition. Several factors raise the risk [1]:
Meconium aspiration: the baby breathes in their own sticky first stool.
Infection: including pneumonia and bloodstream infections.
Respiratory distress syndrome: breathing difficulty in babies whose lungs are not fully developed, usually premature babies.
Birth asphyxia: a lack of oxygen before or during birth.
Diaphragmatic hernia: a hole in the diaphragm that lets abdominal organs push into the chest.
Other congenital heart and lung abnormalities: such as smaller-than-normal lungs or a blocked heart valve.
Certain medicines during late pregnancy: NSAIDs and SSRIs may have an effect [1] [3].
How is PPHN diagnosed in newborns?
A combination of laboratory tests and imaging is used to diagnose PPHN and look for its cause. Early diagnosis helps prevent organ damage from low oxygen [1].
Pulse oximetry and arterial blood gas: measure blood oxygen and how well the body is delivering oxygen.
Echocardiogram: a detailed ultrasound of the heart that shows blood flow to the heart and lungs [4].
Chest X-ray: looks for an enlarged heart or lung disease.
Complete blood count, electrolyte tests, and lumbar puncture: help find infection and check the balance of minerals in the blood.
Head ultrasound: looks for bleeding in the brain.
How is PPHN treated in intensive care?
The main goal of treatment is to increase oxygen flow to the baby's organs and prevent serious complications. Most babies are treated in a neonatal intensive care unit (NICU), and treatment is matched to how severe the condition is [1].
Life Support and Medical Interventions
Supplemental oxygen: up to 100% oxygen through a nasal cannula, nasal prongs, or a mask.
Mechanical ventilation: a breathing tube in the windpipe connected to a ventilator, sometimes with high-frequency oscillatory ventilation.
Nitric oxide: an inhaled gas that dilates the lung blood vessels and improves blood flow to the lungs [2].
Blood pressure support: medicine given through a vein.
ECMO: a machine that temporarily delivers oxygen to the brain and body when the heart or lungs are failing.
What is the long-term outlook and recovery for PPHN?
Most cases of PPHN resolve within one to two weeks, though some take months to recover fully. Between 7% and 10% of newborns with PPHN die from complications, and up to 1 in 4 survivors have lasting effects from a lack of oxygen to the brain [1].
Long-term effects can include developmental delays, learning disabilities, hearing problems such as deafness, and a decreased ability to perform physical activities. Regular visits with the baby's healthcare provider and specialists are important to screen for developmental milestones. To protect the baby from colds, the flu, and other viruses, practice good handwashing, avoid large crowds and sick people, and make sure all household members get their annual flu vaccine [1].
When should you seek immediate care for PPHN?
After discharge, seek immediate medical care if your baby develops a fever, is not feeding well, sucks in the chest or flares the nostrils while breathing, makes wheezing or grunting sounds, or begins to turn a bluish or grayish color [1].
Conclusion
PPHN is a serious and life-threatening challenge, but modern neonatal care has improved outcomes for affected infants. Early detection and prompt treatment are the cornerstones of management, and regular follow-up care helps children reach the best possible long-term health [1].
Frequently Asked Questions
What exactly happens in the lungs during PPHN?
The blood vessels in a newborn's lungs stay narrow and constricted, so blood cannot flow into the lungs to pick up enough oxygen [1].
Is PPHN more common in premature babies?
PPHN happens most often in full-term babies (born between 37 and 42 weeks) and late-term babies (born after 42 weeks). It occasionally occurs in premature babies as well [1].
Can PPHN be prevented during pregnancy?
Researchers do not know why PPHN occurs, so it cannot be prevented. You can reduce risk by avoiding certain medicines during pregnancy, so talk with your healthcare provider about which medicines are safe. Do not stop a prescribed medicine on your own [1].
What is the survival rate for babies with PPHN?
Between 7% and 10% of newborns with PPHN die from complications of the condition, which means about 90% to 93% survive. Of those who survive, up to 1 in 4 have lasting effects from a lack of oxygen to the brain [1].
How long does a baby usually stay in the NICU for PPHN?
Most cases resolve within one to two weeks, but some babies take longer, sometimes months, to recover fully [1].
What is nitric oxide therapy?
Nitric oxide is a gas that helps open (dilate) the blood vessels in the baby's lungs and improves blood flow to them. It is delivered through a ventilator [1].
Does PPHN cause permanent brain damage?
Not every baby is affected, but a lack of oxygen reaching the brain can lead to developmental delays or learning disabilities in some children [1].
Can a baby with PPHN have hearing loss?
Yes. Hearing problems, including deafness, are possible long-term effects in babies who survive PPHN [1].
What is ECMO?
ECMO stands for extracorporeal membrane oxygenation. It is a machine that temporarily delivers oxygen to the baby's brain and body when the heart or lungs are failing [1].
Related Resources
External resource for deeper reading:
Cleveland Clinic. Persistent Pulmonary Hypertension in the Neonate (PPHN). Cleveland Clinic Health Library. Last updated May 15, 2023. https://my.clevelandclinic.org/health/diseases/16020-persistent-pulmonary-hypertension-in-the-neonate-pphn
Related Reading
Learn more in our guide to Apgar Score: How It's Scored and What Your Baby's Score Means.
References
Cleveland Clinic. Persistent Pulmonary Hypertension in the Neonate (PPHN). Cleveland Clinic Health Library. Last updated May 15, 2023. https://my.clevelandclinic.org/health/diseases/16020-persistent-pulmonary-hypertension-in-the-neonate-pphn
Barrington KJ, Finer N, Pennaforte T, et al. Nitric oxide for respiratory failure in infants born at or near term. Cochrane Database Syst Rev. 2017;1(1):CD000399. PMID: 28056166. DOI: 10.1002/14651858.CD000399.pub3
Grigoriadis S, Vonderporten EH, Mamisashvili L, et al. Prenatal exposure to antidepressants and persistent pulmonary hypertension of the newborn: systematic review and meta-analysis. BMJ. 2014;348:f6932. PMID: 24429387. DOI: 10.1136/bmj.f6932
Singh Y, Lakshminrusimha S. Pathophysiology and management of persistent pulmonary hypertension of the newborn. Clin Perinatol. 2021;48(3):595-618. PMID: 34353582. DOI: 10.1016/j.clp.2021.05.009
Medical disclaimer
This article is for informational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have about a medical condition.
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