Persistent Pulmonary Hypertension in a Newborn. PPHN is a serious breathing condition in a newborn. You usually don’t need to be a doctor to see it. The baby often turns blue and is struggling to breathe.
The problem is the lung vessels do not open wide enough. This limits blood flow and oxygen and puts the baby at risk.
PPHN stands for persistent pulmonary hypertension in the newborn (also called persistent fetal circulation syndrome). PPHN is a rare but very serious circulatory condition in newborn babies that can cause difficulty breathing. PPHN is most common in full-term babies and it occurs in about 1.9 out of every 1,000 births.
PPHN occurs when a baby’s blood vessels do not open properly after birth to allow for normal blood circulation into the lungs which is necessary for the baby to begin breathing independently. During gestation, the baby is supplied with maternal oxygen through the placenta. The lungs are still developing and not in use so vessels that permit blood to circulate to the lungs remain closed. After birth, these blood vessels should open up to immediately allow blood to flow into the lungs so the baby can start using them.
In babies with PPHN, these blood vessels fail to open. This is very dangerous because it means that the baby will be unable to get enough oxygen on its own. Oxygen deprivation can lead to major brain injuries and things like cerebral palsy. PPHN can also be deadly. Even if a diagnosis of PPHN is made within the first 72 hours, the chances of survival are between 10-50%.
Over 50% of newborns with a pneumothorax requiring a chest tube develop PPHN. Sepsis/pneumonia will release inflammatory mediators further inducing vasoconstriction and pulmonary hypertension.
Progress is being made in treating PPHN. A few decades ago, the risk of death was as high as 40%. Today, the mortality rate is 5-10%.
The exact cause of PPHN is not fully understood. However, there are many well-known risk factors that can increase one’s chance of having a child with this “rare” but very concerning condition. PPHN is a disease but it’s almost always secondary to some event. Risk factors for PPHN include:
If your child is at potential risk for PPHN, the NICU staff should act accordingly. When your child is born with PPHN, they may appear to have ‘burned skin’ or dry, flaky appearance that looks like it could be peeled off. Their skin could also have a cyanotic or bluish appearance and struggle to breathe.
A quick diagnosis can be made with an echocardiogram (ultrasound of the heart), measuring oxygen levels and conducting a physical examination. Once PPHN has been confirmed, immediate intervention with some combination of continuous oxygen therapy, CPR, suction of the mouth and orifices, ventilation of the lungs, IV antibiotics and administration of surfactant, a medication that prevents the lungs from sticking together, and Milrinone/Primacor will occur. This medication allows the lungs to properly expand, oxygenate and close placental blood flow. This moment, especially for any parent, can be quite overwhelming. You have to hope that your baby’s care in the right hands and hearts of the professionally trained staff attending to him or her and they know how best to treat your baby.
Oxygen is a solution to PPHN but it is not without risk. Oxygen is a drug and, like any drug, even brief exposure to oxygen when not indicated should be avoided if possible. Still, considering what PPHN can do to a child, it is hard for a doctor to argue that that baby should not be on oxygen if there is any doubt. (We are talking about oxygen without a ventilator.)There is also a lot of controversy now about whether cooling is the standard to care to treat newborns who have PPHN. Many newborns are passively cooled to 33-34 degrees in the 6-hour window prior to total body cooling shortly after birth. Does this help? The studies are all of the map on this.
There are possible long-term complications of PPHN that may not be seen until later in your child’s life. These complications can include hearing deficits, stroke, heart failure, kidney failure, brain hemorrhage, seizures, cerebral palsy, oxygen dependence, and pulmonary disease. These long-term issues can lead to increased chances of both physical and mental disabilities.
PPHN Medical Literature
Throughout this page, we provide hyperlinks to the most recent and/or most important PPHN medical studies. Here are more significant articles in the medical literature that help us better understand PPHN.