Oral sildenafil greatly improved exercise capacity and quality of life at 3 months in a 4-year-old girl with primary pulmonary hypertension.
Case Report (n=1)
Does oral sildenafil improve exercise capacity and quality of life in a child with primary pulmonary hypertension?
Oral sildenafil showed encouraging initial results in improving exercise capacity and quality of life in a child with primary pulmonary hypertension.
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.
Abrams et al. (Tue,) conducted a case report in Primary pulmonary hypertension (n=1). Oral sildenafil was evaluated on Exercise capacity and quality of life. Oral sildenafil greatly improved exercise capacity and quality of life at 3 months in a 4-year-old girl with primary pulmonary hypertension.