Persistent pulmonary hypertension (PPHN)
Persistent pulmonary hypertension, PPHN, is a condition in which the blood vessels in the lungs contract, either because the blood vessels never really relaxed after birth or because they contract in response to something in the environment.
Factors that can trigger PPHN include congenital infections, oxygen deficiency at birth and meconium aspiration (see section on MAS), but also by the baby becoming too cold or being in pain. When the blood vessels in the lungs contract, the baby's blood circulation reverts to how it was in the womb, that is, very little blood passes through the lungs. As a result, oxygen uptake is too low, the baby's oxygen supply drops and the baby's skin becomes bluish in colour. The diagnosis is based on the clinical presentation and is confirmed by ultrasound of the heart and blood vessels.
In mild PPHN, it may be sufficient for the baby to be given additional oxygen and sometimes respiratory support using CPAP. Severe PPHN is an emergency that requires intensive care. The baby then needs a ventilator, high levels of oxygen and sometimes nitric oxide (NO) as well. Both oxygen and NO help the pulmonary blood vessels to relax. There are also medications that can be given intravenously and orally, which can help. In severe cases if the baby cannot be properly oxygenated, a life support machine, called ECMO, may be needed, and it is always important to resolve any triggers, as well as to ensure that the baby is not fretting, as this will worsen the situation. Therefore, it is often necessary to administer sedatives, e.g. morphine.
With proper treatment, the blood pressure in the lungs will drop and most babies will make a full recovery.
Text: Kajsa Bohlin Blennow