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Sildenafil in neonatal pulmonary hypertension due to impaired alveolarisation and plexiform pulmonary arteriopathy

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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