PT  - JOURNAL ARTICLE
AU  - Andrew James Wardle
AU  - Richard Wardle
AU  - Karen Luyt
AU  - Robert Tulloh
TI  - The utility of sildenafil in pulmonary hypertension: a focus on bronchopulmonary dysplasia
AID  - 10.1136/archdischild-2012-303333
DP  - 2013 Aug 01
TA  - Archives of Disease in Childhood
PG  - 613--617
VI  - 98
IP  - 8
4099  - http://adc.bmj.com/content/98/8/613.short
4100  - http://adc.bmj.com/content/98/8/613.full
SO  - Arch Dis Child2013 Aug 01; 98
AB  - 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.