High prevalence of minor neurologic deficits in a long-term neurodevelopmental follow-up of children with severe persistent pulmonary hypertension of the newborn: a cohort study

BACKGROUND: Persistent pulmonary hypertension of the newborn (PPHN) is a severe condition that determines a profound brain hypoxia. Inhaled nitric oxide was approved for the treatment of PPHN since the end of the 1990s. The debate upon the long term outcome of these children is still open. Our aim was to investigate the incidence of minor long-term neurodevelopmental problems in a cohort of children affected by severe PPHN. METHODS: All neonates with severe PPHN treated with inhaled nitric oxide in our facility between 01.01.02 and 31.12.07 were seen in a follow up visit and evaluated with a neurodevelopmental scale, according to their age at the time of observation. RESULTS: in the study period 31 children were diagnosed with severe PPHN. 29 survived. 27 accepted to come for follow-up. Mean age: 41 months (range 12 - 70 months). 26% of the evaluated children had some behavioural problems, while 22% had some language disturbances. CONCLUSIONS: This is the first neurodevelopmental follow-up of neonates with PPHN in which children older than 36 months have been evaluated. There is an unexpected high incidence of minor neurological deficits, mainly regarding the fields of language and behaviour. These deficits seem to be related to the severity of illness rather than to the treatment. Language and behaviour are considered "higher functions" in humans and their integrity can be better defined in older children.