One page summary: Persistent pulmonary hypertension of the newborn (PPHN)

In two sentences

Persistent pulmonary hypertension of the newborn means the blood vessels in a baby's lungs stay tight after birth instead of opening up. Blood takes the shortcuts it used before birth and skips the lungs, so oxygen levels stay low. ¹

That is why a baby with PPHN can be on high oxygen and still be blue. The oxygen is in the lungs; the blood is not going there. Treatment is about opening the lung blood vessels, not about adding more oxygen. ¹

The numbers, with their sources

• How often it happens. A multicenter study reported persistent pulmonary hypertension in about 1.9 per 1,000 live births ¹
• What is going wrong. The lung blood vessels stay constricted after birth, so blood passes through fetal shortcuts instead of through the lungs ¹
• The bedside sign. A difference in oxygen saturation between the right hand and a foot, because blood bypassing the lungs enters the circulation after the right arm branches off ¹
• The specific treatment. Inhaled nitric oxide, a gas that relaxes the blood vessels in the lungs ¹
• The rescue treatment. ECMO, in which a machine oxygenates the blood outside the body while the lungs recover ¹
• Why the room is quiet. Handling and stress can cause sharp drops in oxygen, so care is clustered and stimulation is minimized ¹

What to do this week

1. Ask whether an echocardiogram has been done and whether heart disease has been ruled out.
2. Ask what the right hand and foot saturations are.
3. Ask whether inhaled nitric oxide is being used and whether it is helping.
4. Ask what the plan is if things get worse, and whether ECMO would mean transfer.
5. Ask when you can hold your baby and what handling is safe now.
6. Ask for hearing assessment before discharge and a repeat later.
7. Ask for developmental follow-up to be arranged.

Questions to take with you

Take these with you. Write the answers down in the moment, because you will not remember them later.

For the neonatologist

• What is causing the PPHN?
• What did the echocardiogram show, and has heart disease been excluded?
• Is nitric oxide working, and what is the next step if it is not?
• Would ECMO mean transfer, and at what point would you refer?
• Was there a period of low oxygen that affects the brain?

For the nurse

• When is the next handling window?
• What can I do that helps rather than disturbs?
• What are the numbers you are watching, and what do they mean?

You can also build a printable list with the question generator.

Where to read more

The full page on this condition covers how it happens, causes split into what happens even with perfect care and what is associated with gaps in care, signs by age, diagnosis, treatment, outlook, daily life and lifetime cost.

Read the full page on Persistent pulmonary hypertension of the newborn (PPHN)

Related pages are listed at the bottom of it, including the events that can lead to this condition and the guides that cover therapy, school and money.