Persistent Pulmonary Hypertension of the Newborn
(PPHN; Persistent Fetal Circulation [PFC]; Syndrome of Pulmonary Hypertension of Newborn [SPHN])
by Diana Kohnle
Oxygen is needed in every cell of the body. It first enters the body through the lungs. The oxygen is then picked up by the blood flowing by the lungs. The blood brings oxygen to the rest of the body. In newborns with persistent pulmonary hypertension (PPHN), the blood does not flow by the lungs.
The baby's lungs are not used during pregnancy. Instead, oxygen passes from the mother to the baby through the umbilical cord, bypassing the lungs. After the baby is born, the baby's lungs should take over. When this does not occur, most of the blood flow bypasses the lungs, does not pick up oxygen, and the body does not get enough oxygen.
PPHN can be a serious condition. It can cause both immediate and long-term health problems.
PPHN can be caused by a variety of factors, including:
Factors that may increase your baby’s chance of PPHN include:
PPHN may cause:
You will be asked about your baby’s symptoms. Your pregnancy history may also be reviewed. A physical exam will be done.
Your baby's bodily fluids may be tested. This can be done with:
Images may be taken of your baby's bodily structures. This can be done with:
Treatment for PPHN is typically given by a doctor who specializes in newborn illness.
Treatment begins with correcting any related conditions. These conditions can include low blood sugar, low oxygen levels, low blood pressure, and low blood pH. Treatment may include:
Extra oxygen is the most important treatment for PPHN. Oxygen will relax blood vessels to the lungs and improve the flow of blood to the lungs. It may be given with nasal prongs or a hood.
A ventilator is a machine that will help the baby breathe. It may be used if the baby can not get enough oxygen in other ways. A tube will be placed in the baby's throat. The tube is connected to the ventilation machine. The ventilator will deliver air and oxygen with gentle pressure. The pressure will help keep the lungs open.
Nitric oxide is a gas. It may be delivered before or during ventilation. Nitric oxide may relax blood vessels. This will also improve the flow of blood in the lungs.
Medications may be given to relax the baby, especially if a ventilator is needed.
There are a number of new medications to treat PPHN that are being reviewed. One example is sildenafil, which is used to treat erectile dysfunction. Small studies have shown positive results with sildenafil. However, larger studies are needed to confirm the benefits and safety of the medication.
Extracorporeal Membrane Oxygenation (ECMO)
ECMO is a machine that can take over the job of the lungs. It requires major surgery. ECMO may be done if your child has a severe case of PPHN that is not responding to other treatments. This procedure is done to take some stress off of the baby's body. It can give the baby some time to heal.
Most cases of PPHN have no clear cause or are caused by uncontrollable events. For these cases there are no clear preventative steps. Some cases of PPHN may be prevented with proper prenatal care and good health of the mother during pregnancy. General tips for a healthy pregnancy include:
Healthy Children—American Academy of Pediatrics
Kids Health—Nemours Foundation
Extracorporeal membrane oxygenation. Cincinnati Children's Hospital website. Available at:
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Updated May 2015. Accessed September 25, 2017.
Persistent pulmonary hypertension of the newborn (PPHN). EBSCO DynaMed Plus website. Available at: http://www.dynamed... . Updated March 28, 2017. Accessed September 25, 2017.
PS Shah, A Ohlsson. Sildenafil for pulmonary hypertension in neonates. Cochrane Database Syst Rev 2007; 3:CD005494.
Last reviewed September 2018 by EBSCO Medical Review Board Kari Kassir, MD
Last Updated: 5/28/2015
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