The utility of sildenafil in pulmonary hypertension: a focus on bronchopulmonary dysplasia

Karen Luyt, Andrew Wardle, Rob Tulloh

Research output: Contribution to journalArticle (Academic Journal)peer-review

45 Citations (Scopus)


The treatment of pulmonary hypertension (PH) secondary to bronchopulmonary dysplasia (BPD) in infants has evolved in recent years, improving both quality of life and survival for patients. One of the potential agents for this condition is sildenafil, a phosphodiesterase-V inhibitor with proven efficacy within the idiopathic PH population. However, only limited evidence exists for its use as either monotherapy or part of a combination approach towards the management of PH in BPD. This review summarises the evidence base for sildenafil alone and in combination with other recognised therapeutic agents for ameliorating paediatric PH in the presence of BPD. It also examines the suitability for current practice with the aim of clarifying regimens that produce improved patient outcomes. We conclude that sildenafil is both safe and effective in this utility. Doses should be started at 0.5 mg/kg every 8 h before titrating up towards 2 mg/kg every 6 h to effect reductions in pulmonary vascular resistance and arterial pressure. Evidence suggests that if continued until PH resolution, this improves survival from 61% to 81% at 12 months. Furthermore, there are also data suggesting that in treatment refractory PH cases, the addition of endothelin antagonists and prostacyclin analogues to sildenafil therapy can also be considered.

Original languageEnglish
Pages (from-to)613-617
Number of pages5
JournalArchives of Disease in Childhood
Issue number8
Early online date26 Apr 2013
Publication statusPublished - Aug 2013


  • Bronchopulmonary Dysplasia
  • Humans
  • Hypertension, Pulmonary
  • Infant, Newborn
  • Phosphodiesterase 5 Inhibitors
  • Piperazines
  • Purines
  • Sulfones
  • Treatment Outcome


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