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Persistent Pulmonary Hypertension (Pediatric)

What is persistent pulmonary hypertension (PPHN)?

In PPHN the baby’s lungs have difficulty providing enough oxygen to the body. This is because the blood vessels within the lungs are narrowed and tight. As a result, the blood traveling to the lungs is under higher pressure, and some of it is diverted, so it never gets the necessary oxygen, before moving on to other areas of the body.

PPHN can be a serious, life-threatening condition. It may affect babies immediately after birth, in the hours or days following delivery, or even many weeks or months later.

Both full- and pre-term infants can suffer from this condition. Other problems that affect the lung, like infection, long-term lung injury, or very premature lungs can also lead to PPHN.

What are the symptoms of PPHN?

In a newborn, symptoms may include:

  • Rapid breathing
  • Rapid heart rate
  • Respiratory distress
  • Cyanosis (the baby's skin has a bluish tint even when receiving extra oxygen)
  • Heart murmur
  • Low oxygen levels (even while receiving treatment)

How is PPHN diagnosed?

Diagnosis entails:

  • Chest X-rays will show if the baby has lung disease or an enlarged heart.
  • Echocardiogram—an ultrasound of the heart—shows if the baby has heart or lung disease and also evaluates blood flow in those organs.
  • Arterial Blood Gas (ABG) help determine how well oxygen is being delivered throughout the body.
  • Complete Blood Count (CBC) to check the number of oxygen-carrying red blood cells.
  • Blood tests to find out if the baby has an infection.
  • Pulse oximetry to measure oxygen levels in the blood, and keep track of how much oxygen is going to the baby's tissues.

How is PPHN treated?

Treatment may include oxygen therapy with:

  • An oxygen hood.
  • CPAP (Continuous Positive Air Pressure), oxygen given through tiny tubes placed in the nose.
  • Ventilator, a machine that helps the baby to breathe.
  • High frequency ventilation, a machine that delivers rapid, short bursts of oxygen.

Medications may include:

  • Blood pressure medicine
  • Antibiotics
  • Sedation
  • Artificial Surfactant placed into baby's lungs with a breathing tube

Treatment for severe, life-threatening PPHN may involve treating with nitric oxide or an advanced procedure called ECMO (Extracorporeal Membrane Oxygenation), that acts as an artificial heart and lung and delivers oxygen to the child's blood. Our physicians pioneered the use of ECMO and now have the most advanced ECMO program in the nation.