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The utility of sildenafil in pulmonary hypertension: a focus on bronchopulmonary dysplasia
  1. Andrew James Wardle1,
  2. Richard Wardle2,
  3. Karen Luyt3,
  4. Robert Tulloh4
  1. 1University of Bristol, Bristol, UK
  2. 2University of Leicester, Leicester, UK
  3. 3Department of Neonatology, University of Bristol, Bristol, UK
  4. 4Department of Paediatric Cardiology, University of Bristol, University Hospitals Bristol NHS Foundation Trust, Bristol, UK
  1. Correspondence to Dr Robert Tulloh, Department of Paediatric Cardiology, University of Bristol, University Hospitals Bristol NHS Foundation Trust, Upper Maudlin Street, Bristol BS2 8BJ, UK; roberttulloh{at}btinternet.com

Abstract

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.

  • Paediatric
  • Respiratory
  • pulmonary hypertension
  • sildenafil
  • bronchopulmonary dysplasia

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