BetaEntity Annotation Prototype
← Back to diseases

Annotated abstract

P04 A retrospective study identifying predictive factors for unresolved pulmonary hypertension with sildenafil

bmjpo · 2026-04-09 · canonical JSON source

14 visible annotations · policy: published · automated confidence ≥ 75.00%

Document resource

Paediatric pulmonary hypertension (PH) remains a challenging diagnosis due to limited prospective paediatric studies, prognostic markers and treatment options. Sildenafil is the preferred initial agent in our local setting, yet PH resolution rates remain low at eight to forty percent, 1–3 highlighting the need to identify factors influencing treatment outcomes. This retrospective case-control study aimed to characterise baseline clinical patterns of paediatric PH in our country’s largest paediatric hospital and identify predictive factors of unresolved PH at 1-year follow-up with sildenafil monotherapy. By doing so, we sought to refine treatment strategies and enhance patient selection for optimised therapeutic outcomes.Paediatric patients (≤18 years) diagnosed with PH and initiated on oral sildenafil in our institution between 1 January 2014 and 31 December 2023 were included. At 1-year follow-up, participants were categorised into 2 groups: those with PH resolution (Group R) and those without PH resolution (Group N). Baseline categorical data were analysed using the Pearson’s chi-square test or the Fisher’s exact test, as appropriate. Continuous variables were compared using the independent samples t-test for normally distributed data and the Mann-Whitney U test for non-normally distributed data. A multivariate regression model was used to identify predictive factors of unresolved PH.A total of 129 patients met eligibility criteria and were analysed in our study (Group R, N = 87; Group N, N = 42). Majority (79.8%) of the patients were aged >28 days to 1 year, and 76.4% were preterm. Analysis of the World Health Organisation (WHO) group distribution revealed no statistically significant differences between the two groups (p-value 0.946). WHO Group 3 PH, associated with lung disease and/or hypoxia, was the most prevalent diagnosis (56 [64.4%] in Group R and 29 (69.0%) in Group N). Maximum sildenafil doses were comparable (Group R: 5.56 mg/kg/day [Interquartile Range {IQR} 3.16 to 7.88 mg/kg/day]; Group N: 5.13 mg/kg/day [IQR 2.91 to 8.95 mg/kg/day]; p-value 0.890). Baseline tricuspid regurgitant maximum velocity showed no significant difference (Group R: 3.13 m/sec [IQR 2.98 to 3.58 m/sec]; Group N: 3.25 m/sec [IQR 2.98 to 3.71 m/sec]; p-value 0.186). The univariate logistic regression analysis identified need for home ventilatory support at discharge, maternal clinical chorioamnionitis or oligohydramnios and maternal pre-eclampsia as independent predictors of unresolved PH at 1-year follow-up with sildenafil monotherapy. In the multivariate analysis, after adjusting for maternal pre-eclampsia and the need for home ventilatory support at discharge, maternal clinical chorioamnionitis or oligohydramnios was identified as a predictive factor for unresolved PH at 1-year follow-up with sildenafil monotherapy, with an adjusted OR of 2.94 (95% CI 0.994 to 8.72; p-value 0.0514).To our knowledge, this is the first study exploring predictive factors for unresolved PH at 1-year follow-up with sildenafil monotherapy. Therefore, our PREDICT-PH study has provided valuable insights into the clinical profiles of paediatric PH in our country and highlighted key prognostic indicators that may guide future clinical decision-making. These findings underscore the potential utility of early identification of patients who may benefit from alternative therapies beyond sildenafil monotherapy.References Cohen JL, Nees SN, Valencia GA, et al. Sildenafil use in children with pulmonary hypertension. The Journal of Pediatrics 2019;205:29–34.e1.Mourani PM, Sontag MK, Ivy DD, et al. Effects of long-term sildenafil treatment for pulmonary hypertension in infants with chronic lung disease. The Journal of Pediatrics 2009;154(3):379–384.Humpl T, Reyes JT, Erickson S, et al. Sildenafil therapy for neonatal and childhood pulmonary hypertensive vascular disease. Cardiology in the Young 2011;21:187–193.