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Archives of Disease in Childhood - Fetal and Neonatal Edition 2005;90:F527-F528; doi:10.1136/adc.2004.062885
Copyright © 2005 BMJ Publishing Group Ltd & Royal College of Paediatrics and Child Health
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SHORT REPORT

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

M Chaudhari1, M Vogel1, C Wright2, J Smith3, S G Haworth4

1 Department of Paediatric Cardiology, Freeman Hospital, Newcastle upon Tyne, UK
2 Department of Pathology, Royal Victoria Infirmary, Newcastle upon Tyne, UK
3 Paediatric Anaesthesiology, Freeman Hospital, Newcastle upon Tyne, UK
4 Institute of Child Health, Great Ormond Street Hospital, London, UK

Correspondence to:
Correspondence to:
Milind Chaudhari
Department of Paediatric Cardiology, Freeman Hospital, Newcastle upon Tyne, NE7 7DN, UK; milind.chaudhari{at}nuth.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.

Abbreviations: 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

Keywords: impaired alveolarisation; nitric oxide; persistent pulmonary hypertension of neonate; sildenafil




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