Persistent Pulmonary Hypertension

What is persistent pulmonary hypertension (PPHN)?

PPHN is a condition in which a newborn baby's circulation changes back to the circulation of a fetus, where much of the blood flow bypasses the lungs. It is sometimes called persistent fetal circulation.

Who is affected by PPHN?

About one in every 1,250 babies develops PPHN. It occurs most often in full-term or post-term babies after a difficult birth, or birth asphyxia (a condition that results from too little oxygen). PPHN is more likely in babies whose mothers took certain medications in late pregnancy, including nonsteroidal anti-inflammatory drugs and selective serotonin receptor inhibitors (SSRIs).

What causes PPHN?

Illustration demonstrating fetal circulation
Click Image to Enlarge

During pregnancy, the baby’s lungs are not used to exchange oxygen and carbon dioxide, so his or her lungs don’t need as much blood. The baby’s circulation sends most of the blood away from the lungs through special connections in the heart and the large blood vessels. After a baby is born when he or she begins to breathe air, this circulation changes a lot. The change in pressure in the lungs helps close those fetal (pre-birth) connections and redirect the blood flow. Now blood is pumped to the lungs to help with the exchange of oxygen and carbon dioxide.

When a baby has lowered oxygen levels or difficulty breathing at birth, these changes may not happen. This causes the baby's circulation to go back to the fetal system when blood is directed away from the lungs. The lung pressure stays high. That is why this condition is called persistent pulmonary hypertension.

Why is PPHN a concern?

When blood is directed away from the baby's lungs, it is difficult for his or her lungs to exchange oxygen and carbon dioxide. Even breathing air with 100 percent oxygen, babies with PPHN have low blood oxygen levels. This can cause serious problems. All of the body's organs need a regular supply of oxygen-rich blood. They can become damaged if they don’t get enough oxygen.

What are the symptoms of PPHN?

Each baby may experience symptoms differently. Some symptoms of PPHN are:

  • Baby appears ill at delivery or in first hours after birth

  • Lips and skin appear blue (Cyanosis)

  • Rapid breathing

  • Rapid heart rate

  • Low blood oxygen levels while receiving 100 percent oxygen

The symptoms of PPHN sometimes look like other conditions or medical problems. Always see your baby's health care provider for a diagnosis.

How is PPHN diagnosed?

  • Your baby’s health care provider will check his or her medical and delivery history and give your baby a physical examination at birth. Certain procedures to check for PPHN may be recommended, including:

  • Chest X-ray

  • Blood tests

  • Oxygen level checks. (When checked at different places on the baby's body, levels can be compared to see if the blood is being circulated to the lungs.)

Treatment for PPHN

The goal of treatment for PPHN is to increase the oxygen to all of the body systems. If your baby’s body doesn’t get enough oxygen, he or she may have long-term health problems.

Your baby's health care provider will figure out the best treatment for your baby based on:

  • How old your baby is,

  • His or her overall health and medical history

  • How sick he or she is

  • How well your baby can handle specific medications, procedures, or therapies

  • How long the condition is expected to last

  • Your opinion or preference

Treatment may include:

  • Supplemental oxygen. Your baby is given 100 percent oxygen by a mask or plastic hood.

  • Endotracheal tube. This is a tube (ET tube) that is put into the baby's windpipe to help him or her breathe.

  • Mechanical breathing machine. The machine does the work of breathing for the baby.

  • Medications. These can help to completely relax the baby's muscles and reflexes so that he or she will better respond to the mechanical ventilator.

  • Nitric oxide. Breathing in this gas can help expand the blood vessels in the lungs)

  • Extracorporeal membrane oxygenation (ECMO). This is a special procedure that may be used when a baby does not respond to maximum medical care. With ECMO, blood from the baby's veins is pumped through an artificial lung where oxygen is added and carbon dioxide is removed. The blood is then returned back to the baby. ECMO is only used in specialized NICUs.

 

Online Medical Reviewer: MMI board-certified, academically-affiliated clinician
Online Medical Reviewer: Trevino, Heather M., BSN, RNC
Last Review Date: 9/29/2014
© 2000-2014 The StayWell Company, LLC. 780 Township Line Road, Yardley, PA 19067. All rights reserved. This information is not intended as a substitute for professional medical care. Always follow your healthcare professional's instructions.