Persistent Pulmonary Hypertension in the Neonate (PPHN)
Diagram showing normal circulation through heart and lungs after the baby is born.
This condition may also be known as persistent pulmonary hypertension in the newborn (PPHN) and/or persistent fetal circulation syndrome.
What is PPHN?
Persistent pulmonary hypertension in the neonate (PPHN) refers to a serious breathing problem in newborns. It usually happens in full-term babies or babies who were born at 34 weeks or more. These babies often have other types of breathing conditions.
How does PPHN occur?
During pregnancy, the baby gets its oxygen from its mother
and the placenta. Very little blood goes to the lungs because
the blood vessels in the baby’s lungs are mostly closed. The
blood vessels only open after birth when the baby takes his
or her first breaths. The vessels then allow blood to travel
to the lungs to get oxygen. PPHN happens when the blood
vessels do not open up enough, which means that there is
a limit on how much oxygen is sent to the brain and organs.
This is why PPHN is so dangerous.
What causes PPHN?
The cause of PPHN is not known.
In PPHN, failure of lung vessels to open enough can cause “back-pressure” and force the heart to keep using “fetal” (pre-birth) circulation pathways. These shortcut ― the ductus arteriosus (DA) and the foramen ovale (FO) ― allow low-oxygen blood to mix in with blood pumped to the rest of the baby’s body.
What are the risk factors for PPHN?
Although the cause is unknown, certain factors increase the infant’s risk of developing PPHN. These factors include:
meconium aspiration—this happens when the baby breathes in its own sticky poop (meconium)
respiratory distress syndrome (RDS)—this breathing difficulty happens in infants who do
not have fully developed lungs
lack of oxygen before or during birth
What are symptoms of PPHN?
Symptoms of PPHN include:
Breathing problems such as rapid or slow breathing, grunting, and retracting
Blue color to the skin
Hands and feet that are cool to the touch
Low blood pressure throughout the body
Low blood oxygen levels
How is PPHN diagnosed?
The following tests may be used to diagnose PPHN:
Pulse oximetry (a noninvasive way to measure oxygen levels in the blood)
Echocardiogram (a detailed ultrasound of the baby’s heart).
How is PPHN treated?
The treatment of PPHN may include:
Use of oxygen
Use of nitric oxide
Use of a special ventilator that breathes for the baby at a very fast rate
Temporary heart-lung bypass
How long will it take to recover from PPHN?
After treatment for pulmonary hypertension, your baby’s lungs will take weeks or even months to recover fully. It will be important to help protect your baby from catching colds or flu bugs. You can help with this by practicing good hand washing and keeping your baby away from sick people and large crowds. It will also be especially important for your baby to see his or her pediatric healthcare provider and other specialists regularly to be screened for normal developmental milestones.
National Association of Neonatal Nurses. PPHN Parent Info Page.
Steinhorn RH. Neonatal pulmonary hypertension. Pediatr Crit Care Med. 2010;11(2 Suppl):S79-84.
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