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Pulmonary hypertension (PH) is high blood pressure in the lung’s arteries. This increased pressure happens when the arteries in the lungs are too narrow or are not there. PH may be present at birth, or it may develop later.
When the arteries in the lungs are too narrow, the lungs don’t receive enough oxygen-rich blood from the heart. Oxygen is then unable to get to organs and tissues.
PH can affect newborns, babies and children, depending on the type and cause.
Three different types of PH can affect babies and children. The types describe the cause of PH.
Persistent pulmonary hypertension in the newborn (PPHN) occurs in babies soon after birth. It usually affects babies born at full term or near full term.
PPHN happens because the baby’s lungs haven’t adjusted to life outside of the womb. Before babies are born, their lungs don’t need much blood. As a result, some blood vessels leading to the lungs are closed.
When the baby is born, the change in air pressure prompts these blood vessels to open. But in PPHN, the vessels don’t open like they should, leading to low oxygen levels.
Babies who have PPHN may have:
Risk factors for PPHN include:
Secondary PH means that a different health condition caused the PH. Conditions that lead to secondary PH include:
PH without a known cause is called idiopathic PH. Children who have idiopathic PH don’t have any known health conditions that would cause PH. Idiopathic PH is the rarest type.
Symptoms of PH tend to start gradually and get worse over time. If your child is showing signs of PH, seek immediate medical care. If your child has secondary or idiopathic PH, they may experience:
To diagnose PH, doctors use a physical exam and medical tests. Some of these tests include:
The first step in treating PH is to figure out the type and cause. For instance, a child who has congenital heart disease may need surgery or other treatments to correct the heart defect. A child who has sickle cell disease may require treatment to address sickle cell and PH.
There is no cure for idiopathic PH, but treatments can help slow the progression of all types of PH disease. Doctors often use medications to decrease the pressure in the lung’s arteries. These medicines can improve a child’s quality of life. Your child’s medicines may be:
A newborn who has PPHN may need lifesaving treatment in a newborn and infant critical care unit (NICCU). In the NICCU, the baby may receive:
To learn more about our PH expertise and care, visit our Pediatric Pulmonary Hypertension Program.