The goal, the PHA said, is to help identify the disease right away, so as to start early treatment to prevent complications for the baby.
The new guide covers the frequency, symptoms, risk factors, treatment, and recovery of PPHN, as well as how a diagnosis is determined.
This rare form of pediatric pulmonary hypertension (PH) occurs at birth, when blood vessels in the newborn’s lungs fail to fully open.
“Without free flow of blood through the pulmonary vessels after delivery, low oxygen levels can cause damage to a baby’s brain, body, heart and lungs,” the guide said, noting that, without treatment, “the condition can be life threatening.”
Until birth, a baby obtains oxygen from the mother through the placenta. The pulmonary blood vessels remain closed during this time, opening only when the newborn takes his or her first breath.
The PHA estimates that about two in 1,000 live births result in PPHN, which is more common among babies born at full-term, or at 37 weeks of pregnancy or later.
Generally, treatment consists of supporting the respiratory and circulatory systems so that blood flow improves. It’s typically given in a hospital’s neonatal intensive care unit, or NICU.
Some babies require therapies for pulmonary hypertension — such as inhaled nitric oxide or phosphodiesterase type 5 inhibitors — and a treatment that delivers oxygen to the blood. That treatment, known as extracorporeal membrane oxygenation, or ECMO, is usually used in more severe cases. Other interventions may include receiving supplemental oxygen, mechanical ventilation, antibiotics, intravenous (into-the-vein) fluids, or sedation.
Recovery can take weeks to months, and often requires specialist care throughout. Babies at higher risk of developmental delays also may need to be seen by developmental specialists and neurologists.
Although PPHN shares similarities with adult pulmonary hypertension, the differences between the two mean that infants require specialist care. There are eight pediatric PH care centers in the U.S. that have been accredited by the PHA. More information about these centers can be found here.
Families who do not live close to any of the centers are encouraged to use the PHA’s directory of doctors who treat pulmonary hypertension. While not specifically endorsed by the PHA, all of the doctors on the lists are members of Pulmonary Hypertension Clinicians and Researchers, or PHCR, the organization’s own network. To find a doctor outside of the U.S., visit www.PHAEurope.org.
Symptoms of PPHN include fast breathing and heart rate, low blood pressure, and bluish lips, hands, and feet. The bluish tinge is a common sign of low blood oxygen levels.
Risk factors include having underdeveloped lungs, inhaling stool and amniotic fluid — collectively known as meconium — during delivery, and infections. Pre-birth exposure to some medications taken by the mother, including certain antidepressants, also can be a risk factor.
PPHN occurs in males and females, but is more common among newborn boys. It also occurs more frequently in babies born to Black and Asian mothers.
Additional information and resources are available at the PHA website for pediatric PH.
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