Patient education: Persistent pulmonary hypertension of the newborn (The Basics)

What is persistent pulmonary hypertension of the newborn? — This is a rare condition that causes serious breathing problems in newborn babies. It is also called "PPHN."

PPHN happens when the blood vessels that carry blood to the lungs do not widen like they should after birth. This can cause problems if it is not treated. In severe cases, it can even lead to death, although this is not very common.

When PPHN happens, the cause is not always known. Certain health conditions seem to increase a baby's risk. For example, it might be more likely in babies who:

●Have something called "congenital diaphragmatic hernia" – This is when there is a hole in the diaphragm. The diaphragm is the muscle that separates the chest from the belly.

●Pass a bowel movement before birth – This is called "meconium." If this happens, the baby can breathe it in, which can lead to problems.

What are the symptoms of PPHN? — Symptoms appear soon after birth, usually while the baby is still in the hospital. They might include:

●Fast breathing

●In babies with light skin, the skin or lips looking blue

●In babies with dark skin, the lips looking blue or darker than usual

Will my baby need tests? — Yes. The main test is an "echocardiogram." This is an imaging test that uses sound waves to create pictures of the heart as it beats.

Your baby might also need other tests, including:

●Chest X-ray

●Blood test

●Electrocardiogram ("ECG") – This test measures the electrical activity of the heart.

How is PPHN treated? — Treatment happens in the hospital. This might include:

●Oxygen – Oxygen can be given different ways, including through:

•A plastic hood put over the baby's head

•2 plastic tubes put in the baby's nostrils

•A mask over the baby's mouth and nose – This is called "continuous positive airway pressure" ("CPAP").

●Breathing support – The doctor puts a tube in the baby's throat. The tube connects to a machine that helps the baby breathe. This machine is called a "ventilator."

●Inhaled nitric oxide – This is a gas that is given through the ventilator. It helps widen the baby's blood vessels.

●ECMO – ECMO is a special treatment done only for very serious cases that do not get better with other treatments. During ECMO, the baby is connected to a machine. The machine takes the baby's blood out little by little, adds oxygen to it, and then returns it to the baby.

What will my baby's life be like? — Many babies with PPHN have normal lives. But babies with very serious forms of the condition or those who need ECMO might have hearing problems or "developmental delays." This means that they take longer than average to learn how to do things like walking and talking.

What problems should I watch for? — After your baby comes home from the hospital, call the doctor or nurse for advice if they have:

●Very fast or very slow breathing

●Trouble breathing − Your baby's skin might pull in under the ribs when they breathe.

●Skin that looks blue, especially around the mouth, eyes, or fingers

●A very fast heart rate

●Fever of 100.4°F (38°C) or higher

●Trouble eating, like taking much longer than usual to eat or coughing a lot while eating

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