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Sildenafil in neonatal pulmonary hypertension due to impaired alveolarisation and plexiform pulmonary arteriopathy
  1. M Chaudhari1,
  2. M Vogel1,
  3. C Wright2,
  4. J Smith3,
  5. S G Haworth4
  1. 1Department of Paediatric Cardiology, Freeman Hospital, Newcastle upon Tyne, UK
  2. 2Department of Pathology, Royal Victoria Infirmary, Newcastle upon Tyne, UK
  3. 3Paediatric Anaesthesiology, Freeman Hospital, Newcastle upon Tyne, UK
  4. 4Institute of Child Health, Great Ormond Street Hospital, London, UK
  1. Correspondence to:
    Milind Chaudhari
    Department of Paediatric Cardiology, Freeman Hospital, Newcastle upon Tyne, NE7 7DN, UK; milind.chaudharinuth.northy.nhs.uk

Abstract

We report a case of severe pulmonary hypertension in a neonate associated with impaired alveolarisation and plexiform pulmonary arteriopathy. Treatment with oral sildenafil in addition to inhaled nitric oxide (NO) resulted in recovery from the pulmonary hypertensive crisis. Long term sildenafil therapy was associated with complete resolution of the pulmonary hypertension.

  • BSA, body surface area
  • cGMP, cyclic-guanosine 5 monophosphate
  • ECG, electrocardiogram
  • ECMO, extra-corporeal membrane oxygenation
  • NO, nitric oxide
  • PPHN, persistent pulmonary hypertension of the newborn
  • RV, right ventricular
  • RVH, right ventricular hypertrophy
  • TR, tricuspid valve regurgitation
  • impaired alveolarisation
  • nitric oxide
  • persistent pulmonary hypertension of neonate
  • sildenafil

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Footnotes

  • Competing interests: none declared

  • Consent was obtained for the publication of these patient details