Objective To investigate the characteristics of persistent pulmonary hypertension of the newborn (PPHN) in extremely preterm infants and its impact on neurodevelopmental outcomes at 3 years of age.

Design A retrospective multicentre cohort study.

Settings 202 tertiary perinatal centres registered in the Neonatal Research Network of Japan (NRNJ).

Patients Infants born at <28 weeks of gestational age (GA), between 2003 and 2012, were extracted from tertiary perinatal centres participating in NRNJ.

Main outcome measures Demographic characteristics, morbidity, interventions and mortality were compared for infants with and without PPHN. Multivariable logistic analysis was performed to evaluate the impact of PPHN on long-term neurodevelopmental outcomes (the prevalence rate of cerebral palsy, need for home oxygen therapy, and visual, hearing and cognitive impairment) at 3 years of age.

Results The prevalence of PPHN among the 12 954 extremely preterm infants enrolled was 8.1% (95% CI 7.7% to 8.6%), with the trend increasing annually, and a higher proportion as GA decreased: 18.5% (range, 15.2% to 22.4%) for infants born at 22 weeks compared with 4.4% (range, 3.8% to 5.2%) for those born at 27 weeks. Clinical chorioamnionitis and premature rupture of membranes were associated with PPHN. On multivariate analysis of the data from 5923 infants followed up for 3 years, PPHN was a significant independent risk factor for visual impairment (adjusted OR, 1.42, 95% CI 1.03 to 1.97).

Conclusions The prevalence of PPHN in extremely preterm infants has been increasing over the past decade in Japan. Clinicians should be aware of visual impairments as a neurodevelopmental abnormality among infants with PPHN.