Electronic pages: Paper
Sildenafil as a selective pulmonary vasodilator in childhood
primary pulmonary hypertension
D Abrams, I Schulze-Neick, A G Magee
Department of
Paediatric Cardiology, Royal Brompton & Harefield NHS Trust, Sydney
Street, London SW3 6NP, UK
Correspondence to: Dr Magee email: a.magee{at}rbh.nthames.nhs.uk
Accepted 19 April
2000
Primary pulmonary hypertension is a rare disease of childhood,
which carries a poor prognosis. Patients often present with severe
exercise limitation, and untreated life expectancy is less than 1 year.
Pharmacological intervention is directed towards reduction of the
raised pulmonary artery pressure with vasodilator treatment, initially
with calcium antagonists, although more recently long term prostacyclin
treatment has shown benefit in some patients. Heart-lung
transplantation remains an option for children with severe disease
refractory to therapeutic treatment. A 4 year old Bangladeshi
girl with dyspnoea, cyanosis, and signs of a low cardiac output, is
described. Initial treatment with prostacyclin was gradually reduced,
and maintenance treatment with oral sildenafil (Viagra; Pfizer)
instituted. At follow up 3 months later, her exercise capacity was
greatly improved and she continues to enjoy a good quality of life
without obvious side effects. In view of the encouraging initial
results, this may become an acceptable adjunct in treating this patient group.
Keywords: primary pulmonary hypertension; sildenafil; prostacyclin
© 2000 by Heart
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