Pulmonary hypertension of the newborn is an important cause of hypoxaemia,
particularly in the at term or near term neonate. It can occur as a primary
condition or secondary to a variety of other diseases. Endogenous nitric o
xide is an important modulator of vascular tone in pulmonary circulation. I
nitial uncontrolled studies indicated that inhalation of nitric oxide resul
ted in a reduction in pulmonary hypertension, with improvement in oxygenati
on, but no change in the systemic vascular resistance. There have now been
a number of randomised trials performed exploring the efficacy of inhaled n
itric oxide. These trials have demonstrated that in at term or near term in
fants, inhaled nitric oxide reduces the combined end point of death or the
need for extracorporeal membrane oxygenation. The significant effect seems
due to the reduced extracorporeal membrane oxygenation requirement. No such
beneficial effect has been consistently reported in infants with congenita
l diaphragmatic hernia. Randomised trials have failed to highlight long-ter
m positive results in preterm infants. Inhaled nitric oxide has side effect
s, although those due to nitrogen dioxide and methaemoglobin formation can
be minimised by appropriate nitric oxide delivery. It is important to use t
he smallest effective nitric oxide dose, continuous nitric oxide and nitrog
en dioxide monitoring and frequent methaemoglobin analyses. Careful patient
selection should be undertaken, avoiding those at high risk of haemorrhagi
c complications. Longer term follow-up studies are required to determine th
e real risk:benefit ratio of inhaled nitric oxide treatment.