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Baczynski M, Kelly E, McNamara PJ, Shah PS, Jain A. Short and Long-term Outcomes of Chronic Pulmonary Hypertension in Preterm Infants Managed using a Standardized Algorithm. Pediatr Pulmonol. 2020 Dec 3. doi: 10.1002/ppul.25200. Epub ahead of print. PMID: 33270376.

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. Inter-group 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 (95% CI) 1.02 (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. This article is protected by copyright. All rights reserved.

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