Sildenafil as a selective pulmonary vasodilator in childhood primary pulmonary hypertension

• Published in: Heart (British Cardiac Society) Vol. 84; no. 2; pp. e4 - E4
• Main Authors: Abrams, D, Schulze-Neick, I, Magee, A G
• Format: Journal Article
• Language: English
• Published: England BMJ Publishing Group Ltd and British Cardiovascular Society 01.08.2000; BMJ Publishing Group LTD
• Subjects: Antihypertensive Agents - therapeutic use; Blood pressure; Child, Preschool; Electronic pages: Paper; Epoprostenol - therapeutic use; Female; Humans; Hypertension, Pulmonary - drug therapy; Nitric oxide; Phosphodiesterase Inhibitors - therapeutic use; Piperazines - therapeutic use; primary pulmonary hypertension; prostacyclin; Pulmonary arteries; Pulmonary hypertension; Purines; Rodents; sildenafil; Sildenafil Citrate; Sulfones; Treatment Outcome; Vasodilator Agents - therapeutic use; Veins & arteries
• ISSN: 1355-6037; 1468-201X
• DOI: 10.1136/heart.84.2.e4

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.