Short and long-term outcomes of chronic pulmonary hypertension in preterm infants managed using a standardized algorithm

Background There is limited data on management strategies for chronic pulmonary hypertension (cPH) in chronic lung disease (CLD) of prematurity. Our objective was to evaluate clinical outcomes following a standardized policy, wherein only cPH with right-ventricular (RV) dilatation was treated and diuretics were employed as first-line therapy; cPH without RV-dilatation was managed expectantly. Method In this retrospective cohort study, all infants with CLD were categorized as "CLD-only" or "CLD-cPH," using echocardiography at >= 36 weeks postmenstrual age. Intergroup comparison was performed. Regression analysis examined the association between cPH and primary outcome of death or disability at 18-24 months. Results Of 128 CLD infants, 48 (38%) had cPH, of which 29 (60%) received diuretics. Symptomatic improvement within 1-week was recorded in 90%. Although CLD-cPH had worse in-hospital respiratory course than CLD-only, all post-discharge respiratory and neurodevelopmental outcomes were similar. cPH was not associated with death or disability (adjusted odds ratio, 1.02; 95% confidence interval, 0.32-3.27). Disease progression treated with sildenafil occurred in 2 (4%) cases. There was no death from respiratory or RV failure. Conclusion Primary treatment of CLD-cPH with diuretics using RV-dilatation as therapeutic threshold, may result in symptomatic improvement, disease stabilization and post-discharge outcomes comparable to infants without cPH.