What is pulmonary hypertension?

The blood pressure in the arteries that supply the lungs and the rest of the body are at similar pressure in foetal life. After birth, as the ductus arteriosus closes, after a short while the pressure in the lung circuit falls and for the rest of life this pulmonary circuit works at a lower pressure compared to the rest of the circulation (systemic circulation)

The pulmonary artery transfers blood from the right side of the heart to the lungs. In some situations the pulmonary pressure remains or becomes high, when it is termed pulmonary hypertension. This situation can lead to several health problems, depending on whether it occurs suddenly (acute) or persists over a much longer period (chronic).

Who is affected and what are the risk factors?

The disease is very rare in babies. About 1-2 of 1000 newborn babies may be affected, and it arises mainly during periods of intensive care. It sometimes accompanies asphyxia or bronachopulmonary dysplasia.

What is the cause?

Pulmonary hypertension can occur as part of severe acute illness, such as during mechanical ventilation for a range of conditions, or be chronic due to the effect of severe lung disease or a PDA. Extremely rarely it occurs on its own, when it may be termed idiopathic pulmonary hypertension.

How is it diagnosed?

The main observation is a need for high oxygen concentrations in excess of what would be expected from any lung disease. Usually it is diagnosed following an ultrasound examination (echocardiography).

How is it managed?

Pulmonary hypertension may resolve with better ventilation, e.g. using high frequency oscillatory ventilation (HFOV). It also sometimes resolves with the use of inhaled nitric oxide gas.  Very rarely in term babies, usually after aspiraton of meconium, it is reistant to ordinary treatment and babies may be referred for a complex treatment termed ECMO.  The healthcare tea would discuss this in more detail if it is required and it is not used for small preterm babies.

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