Persistent Pulmonary Hypertension

During fetal development, most of your baby’s blood bypasses the developing lungs because the placenta exchanges oxygen and carbon dioxide. The placenta no longer supports your baby’s oxygen exchange after birth, so blood must go to the lungs to pick up oxygen so it can be delivered to the rest of the body. If there is abnormally high blood pressure in the lungs, or pulmonary hypertension, there can be a lack of blood flow to the lungs. This can result in a lack of oxygen delivery to the organs of the body.

Persistent pulmonary hypertension of the newborn (PPHN), or persistent fetal circulation, is a condition in which a newborn's normal blood circulation maintains the circulation of a fetus. It is one of the causes of pulmonary hypertension in a newborn. It is most likely to occur after a difficult birth or if a baby has lowered oxygen levels during birth from another complication.

Each baby with pulmonary hypertension may experience symptoms differently. Symptoms include:

The baby appears ill or unwell at delivery or within a few hours after birth
The lips and/or skin appear blue (cyanosis)
Rapid heart rate
Rapid breathing
Low blood oxygen levels, even when receiving 100 percent oxygen

Diagnosis of Pulmonary Hypertension

Your child's pediatrician will perform a physical exam immediately after birth to check for any signs of illness. If pulmonary hypertension is suspected, a diagnosis can be confirmed with the following exams and tests:

Chest X-ray. A chest X-ray helps rule out other causes for your baby's symptoms.
Blood tests. A complete blood count (CBC) test can also tell the doctor if your baby has another condition causing the symptoms.
Oxygen level checks. The doctor will check the level of oxygen in your baby's blood. A low blood oxygen level may be due to pulmonary hypertension.

Treatments

In the case of pulmonary hypertension in newborns, the goal of treatment is to improve blood flow to the lungs so that oxygen can be delivered throughout the body. If your baby does not have enough oxygen circulating to support the organs (including the brain) for a prolonged period of time, he or she may develop long-term health problems.

Pulmonary hypertension in newborns is treated with the following at Riley at IU Health:

Intubation and mechanical ventilation. A thin tube is inserted into your baby’s windpipe through the mouth so that a specialized machine can move air into and out of the lungs. Types of mechanical ventilation include:
- Conventional ventilation. This moves air in and out of the lungs in a similar quantity to when babies breathe on their own.
- High frequency jet ventilation. This moves smaller quantities of air into and out of the lungs with greater frequency to protect your baby’s lungs from overexpansion.
- High frequency oscillatory ventilation. Similar to high frequency jet ventilation, this moves small quantities of air into and out of the lungs often with even greater frequency, which is safer for babies with severely underdeveloped lungs.
Inhaled nitric oxide. This is a special gas that is delivered through a breathing tube that can help the blood vessels in the lungs relax and work better.
Extracorporeal membrane oxygenation (ECMO). A machine acts as an artificial lung and moves deoxygenated blood out of the body. It then removes carbon dioxide from the blood, adds oxygen and warms the blood back to body temperature before returning it to circulate through the body.

Due to the specialized nature of these treatments, your baby will undergo treatment in the neonatal intensive care unit (NICU). An expert care team of doctors, respiratory therapists and pediatric specialists will monitor your baby’s condition around the clock and provide immediate care if there are any changes.

When it is safe for your baby to leave the NICU, you will meet with a multidisciplinary team to discuss follow-up care. Hearing problems and developmental delays can be common in babies who have had pulmonary hypertension. If your baby is expected to have any added health concerns or neurodevelopmental delays because of pulmonary hypertension, you will meet with specialists who can help manage those conditions, such as an audiologist (hearing specialist), a speech therapist or a developmental pediatrician.

Key Points to Remember

Pulmonary hypertension occurs when a baby’s circulation maintains fetal circulation, resulting in a lack of oxygen throughout the body.
Symptoms such as rapid breathing, rapid heart rate and a blue color to the skin may indicate pulmonary hypertension.
Pulmonary hypertension can be treated with intubation and mechanical ventilation and other specialized breathing treatments.
Treatment for pulmonary hypertension takes place in the neonatal intensive care unit (NICU).

Support Services & Resources

Visit the trusted websites below to learn more about persistent pulmonary hypertension of the newborn.

Riley at IU Health offers a broad range of supportive services to make life better for families who choose us for their children's care.

Learn More About Riley Support Services

Pulmonary Hypertension Association

This nonprofit organization works to improve the lives of those affected by pulmonary hypertension by promoting education and research and connecting patients to local support groups.

National Heart, Lung, and Blood Institute

The National Institutes of Health provides an in-depth overview of pulmonary hypertension, including what it is, who is at risk and what it is like living with the condition.

MedlinePlus

Learn more about pulmonary hypertension and view illustrations of the respiratory system and heart.

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