Sildenafil as a selective pulmonary vasodilator in childhood primary pulmonary hypertension.

摘要: Primary pulmonary hypertension is a rare disease of childhood, which carries poor prognosis. Patients often present with severe exercise limitation, and untreated life expectancy less than 1 year. Pharmacological intervention directed towards reduction the raised artery pressure vasodilator treatment, initially calcium antagonists, although more recently long term prostacyclin treatment has shown benefit in some patients. Heart–lung transplantation remains an option for children refractory to therapeutic treatment.  A 4 year old Bangladeshi girl dyspnoea, cyanosis, signs low cardiac output, described. Initial was gradually reduced, maintenance oral sildenafil (Viagra; Pfizer) instituted. At follow up 3 months later, her capacity greatly improved she continues enjoy good quality without obvious side effects. In view encouraging initial results, this may become acceptable adjunct treating patient group.