What Parents Need to Know About Persistent Pulmonary Hypertension of the Newborn

When a baby is developing, they receive oxygen from the placenta through the umbilical cord. Their lungs do not exchange oxygen and carbon dioxide while they are in the womb, and the blood vessels in their lungs are almost completely closed.

Within minutes of being born, the blood vessels in the lung should widen as the newborn begins to breathe air using their lungs. When these blood vessels widen, it causes a rapid increase in blood flow to the lungs. As blood flow increases, it flows through the lungs so the lungs can use the oxygen before the blood travels to the rest of the baby’s body.

If this doesn’t happen and a newborn’s circulation system continues to bypass the lungs, it is known as PPHN. It is also called persistent fetal circulation.

Some common symptoms of PPHN include:

• rapid breathing and shortness of breath
• low Apgar scores
• low blood oxygen levels even after receiving 100% oxygen
• high heart rate
• low blood pressure
• cold hands and feet
• signs of distress like moaning, grunting, or flaring nostrils while breathing
• retractions (pulling in the skin under the ribs while breathing hard)

PPHN can be caused by issues with the blood vessels in a newborn’s lungs. They may be atypically formed or underdeveloped. The blood vessels may also have a hard time adjusting to the air for a variety of reasons, such as illness or severe distress during delivery.

PPHN is typically diagnosed soon after a baby’s birth.

In addition to checking the baby’s delivery and health history, the doctor may request:

• a chest X-ray to see if there are issues with the heart or lungs
• blood tests
• oxygen level checks on different parts of the body, which are then compared to see whether oxygen is making its way to the lungs
• an echocardiogram

Treatment of PPHN focuses on:

• getting oxygen to all of the baby’s organ systems
• maintaining typical blood pressure
• opening up the blood vessels in the lungs

Treatment depends on the newborn’s general health, their symptoms, and how severe the condition is.

Treatment options can include:

• supplemental oxygen through a mask or hood
• endotracheal (ET) tube
• ventilator
• nitric oxide to expand the blood vessels in the lungs

IV medications given through a vein may also be used to help treat some underlying causes and symptoms. These medications may include:

• blood pressure medications
• antibiotics

If all other treatments do not help, doctors may use extracorporeal membrane oxygenation (ECMO) to take over the functions of the heart and lungs.

Newborn babies are at a higher risk of PPHN if they have:

• aspirated meconium (when baby breathes in their first bowel movements while in the placenta)
• atypical heart or lung development
• an infection like pneumonia
• difficult birth
• birth asphyxia (not receiving enough oxygen while being born)
• risk factors related to the birthing parent, such as
  • having obesity
  • having diabetes
  • experiencing pre-eclampsia
  • experiencing chorioamnionitis
  • smoking
  • taking selective serotonin reuptake inhibitors
  • taking nonsteroidal anti-inflammatory drugs

While most newborns do not experience PPHN, when it happens, PPHN is a very serious medical event with a death rate of between 7.6 to 10.7%.

With current treatment options, more babies with PPHN survive. For infants with no other conditions and who have mild symptoms, the outlook is better, but they are more likely to need to go back into the hospital at some point during infancy.

Follow-up care for infants following PPHN is important because there can be long-term complications, such as:

• developmental delays
• cognitive delays
• hearing loss

Researchers in 2022 noted that Black babies had more adverse or poor outcomes from PPHN, followed closely by Hispanic and Asian infants. One reason for this may be systemic racism and inequities in prenatal care, healthcare, and access to care.

How long will it take a baby with PPHN to recover?

It may take your baby’s lungs weeks or even months to recover from PPHN. It’s important to note that even after the lungs have had the chance to heal, the long-term effects of PPHN can continue.

Can older children get PPHN?

PPHN technically only occurs in newborns, but older children can develop pulmonary hypertension from medical conditions like congenital heart and lung diseases.

What can I do to help prevent my baby from having PPHN?

There is no way to completely remove the risk of PPHN, but getting regular prenatal care during pregnancy can increase the chances of having a healthy baby.

PPHN is a potentially fatal condition that will typically be diagnosed quickly after a baby’s birth. It occurs when a newborn’s circulation system does not adjust for breathing outside the womb. It’s important to get medical help right away if you notice signs of PPHN.

Treatment focuses on ensuring the baby’s organs get sufficient oxygen. This is important because long-term health complications can develop when this does not occur.