This disorder causes severe trouble breathing (respiratory distress) in full-term or postterm newborns.
Breathing is rapid, and the skin and/or lips may be bluish or may be pale and grayish.
The diagnosis is confirmed by an echocardiogram.
Treatment involves opening (dilating) the arteries to the lungs by giving high concentrations of oxygen, often while supporting the newborn’s breathing with a ventilator.
To help dilate the arteries in the lungs, sometimes nitric oxide is added to the gas that the newborn is breathing.
Extracorporeal membrane oxygenation is sometimes used in the most severe cases.
(See also Overview of General Problems in Newborns Overview of General Problems in Newborns Problems in newborns may develop Before birth while the fetus is growing During labor and delivery After birth About 10% of newborns need some special care after birth due to prematurity, problems... read more .)
Normally, the blood vessels to the fetus's lungs are tightly constricted before birth. The lungs do not need much blood flow before birth because the placenta rather than the lungs eliminates carbon dioxide and transports oxygen to the fetus. However, immediately after birth, the umbilical cord is cut and the newborn’s lungs must take over the role of oxygenating the blood and removing carbon dioxide. To achieve this process, it is necessary for the fluid filling the air sacs (alveoli) in the lungs to be replaced by air and for the pulmonary arteries, which bring blood to the lungs, to widen (dilate) so that an adequate amount of blood flows through the lungs to be oxygenated.
Sometimes the blood vessels to the lungs do not widen (dilate) after birth as they normally should. When the blood vessels to the lungs do not widen, blood pressure in the pulmonary arteries is too high (pulmonary hypertension Pulmonary Hypertension Pulmonary hypertension is a condition in which blood pressure in the arteries of the lungs (the pulmonary arteries) is abnormally high. Many disorders can cause pulmonary hypertension. People... read more ), and blood flow to the lungs is insufficient. Because of this insufficient blood flow, not enough oxygen reaches the blood.
There are many reasons the blood vessels may not widen, including
Severe distress during delivery (such as from meconium aspiration syndrome Meconium Aspiration Syndrome Meconium aspiration syndrome is trouble breathing (respiratory distress) in a newborn who has breathed (aspirated) a dark green, sterile fecal material called meconium into the lungs before... read more )
Any other circumstance that causes a low level of oxygen in the fetus before, during, or after delivery, such as infection in the mother or the fetus (sepsis in the newborn Sepsis in Newborns Sepsis is a serious bodywide reaction to infection spread through the blood. Newborns with sepsis appear generally ill—they are listless, do not feed well, often have a gray color, and may have... read more ), problems with the placenta, diaphragmatic hernia Diaphragmatic Hernia A diaphragmatic hernia is a birth defect in which a hole or weakening in the diaphragm allows some of the abdominal organs to protrude into the chest. A diaphragmatic hernia causes severe difficulty... read more , collapsed lung Pneumothorax in the Newborn Pneumothorax is a collection of air between the lung and the chest wall that develops when air leaks out of the lung. This disorder may develop in newborns who have lung disorders such as respiratory... read more , underdeveloped lungs, or pneumonia in the newborn Overview of Pneumonia Pneumonia is an infection of the small air sacs of the lungs (alveoli) and the tissues around them. Pneumonia is one of the most common causes of death worldwide. The most common symptom of... read more
Certain changes in the newborn's circulation, including those resulting from taking certain medications during pregnancy (such as large doses of aspirin or other nonsteroidal anti-inflammatory drugs such as ibuprofen)
Persistent pulmonary hypertension of the newborn is more common among newborns who are full term (delivered between 37 weeks and 42 weeks of gestation Gestational Age Problems in newborns may develop Before birth while the fetus is growing During labor and delivery After birth About 10% of newborns need some special care after birth due to prematurity, problems... read more ) or postterm Postterm Newborns A postterm newborn is a baby delivered at or after 42 weeks of gestation. Near the end of a term pregnancy, the function of the placenta decreases, providing fewer nutrients and less oxygen... read more (delivered after 42 weeks of gestation).
Sometimes persistent pulmonary hypertension is present from birth. Other times, it develops over the first day or two.
Breathing is usually rapid, and the newborn may have severe trouble breathing if it has an underlying lung disorder (such as respiratory distress syndrome).
The skin and/or lips may have a bluish discoloration (cyanosis Cyanosis Cyanosis is a bluish or grayish discoloration of the skin resulting from an inadequate amount of oxygen in the blood. Cyanosis occurs when oxygen-depleted (deoxygenated) blood, which is bluish... read more ) due to low blood oxygen levels. Sometimes newborns with persistent pulmonary hypertension have low blood pressure Low Blood Pressure Low blood pressure is blood pressure low enough to cause symptoms such as dizziness and fainting. Very low blood pressure can cause damage to organs, a process called shock. Various drugs and... read more (hypotension) that causes weak pulses and a pale, grayish hue to the skin.
In newborns of color, the skin may change to colors such as yellow-gray, gray, or white. Newborns of color who have low blood pressure may also have a pale, grayish hue to the skin. These changes may be more easily seen in the mucous membranes lining the inside of the mouth, nose, and eyelids.
Bluish or gray discoloration even though the newborn is receiving oxygen
Doctors may suspect persistent pulmonary hypertension if the mother took high doses of aspirin or ibuprofen during pregnancy or had a stressful delivery. They also suspect persistent pulmonary hypertension if the newborn has severe trouble breathing, bluish or gray discoloration that does not go away when high concentrations of supplemental oxygen are given, and unexpectedly low oxygen levels in the blood. Doctors may also suspect persistent pulmonary hypertension in newborns who have meconium aspiration syndrome, who may have an infection, or who need more oxygen or breathing support than expected.
To confirm the diagnosis of persistent pulmonary hypertension of the newborn, doctors do an echocardiogram Echocardiography and Other Ultrasound Procedures Ultrasonography is a type of medical imaging that uses high-frequency (ultrasound) waves to produce a moving image of internal organs and other tissues. Echocardiography is ultrasonography of... read more to see how blood flows through the heart and to the lungs.
A chest x-ray may be normal or may show changes caused by an underlying disorder (such as diaphragmatic hernia or meconium aspiration syndrome).
Cultures of the blood may be done to look for certain kinds of bacteria.
Sometimes a ventilator
Sometimes nitric oxide gas
Sometimes extracorporeal membrane oxygenation
Treatment of persistent pulmonary hypertension of the newborn involves placing newborns in an environment with 100% oxygen. In severe cases, a ventilator Mechanical Ventilation Mechanical ventilation is use of a machine to aid the movement of air into and out of the lungs. Some people with respiratory failure need a mechanical ventilator (a machine that helps air get... read more (a machine that helps air get in and out of the lungs) may be needed and may be used to provide 100% oxygen. A high percentage of oxygen in the blood helps widen the arteries going to the lungs.
A very small concentration of the gas nitric oxide may be added to the oxygen that the newborn is breathing. Inhaled nitric oxide widens the arteries in the newborn’s lungs and reduces pulmonary hypertension. This treatment may be needed for several days.
Rarely, if all other treatments do not work, extracorporeal membrane oxygenation (ECMO) can be used. In this procedure, blood from the newborn is circulated through a machine that adds oxygen and removes carbon dioxide and then returns the blood to the newborn. The machine acts as an artificial set of lungs for the newborn. As the machine does the work of getting oxygen into the newborn's body, the newborn's lungs have time to rest and the blood vessels slowly widen. ECMO has been lifesaving, allowing some newborns with pulmonary hypertension who do not respond to other treatments to survive until the pulmonary hypertension resolves.
Fluids and other treatments, such as antibiotics for an infection, are given as needed.
About 10 to 60% of affected newborns die depending on the cause of the persistent pulmonary hypertension.
About 25% of survivors have developmental delays, hearing problems, functional disabilities (meaning a decreased ability to do physical activities), or a combination.