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Logo of archdischArchives of Disease in ChildhoodVisit this articleSubmit a manuscriptReceive email alertsContact usBMJ
Arch Dis Child. 2007 August; 92(8): 689.
PMCID: PMC2083864

Epoprostenol for severe pulmonary hypertension

Average survival time with untreated idiopathic pulmonary hypertension is 2.8 years in adults and 10 months in children. Successful treatment with epoprostenol (prostacyclin) was reported for adults in 1996 and for children in 1999. Now a report from the Great Ormond Street Hospital for Children, London (Heart, published online 25 Oct 2006; doi 10.1136/hrt.2006.096412) has provided details of all 39 children treated there with continuous intravenous epoprostenol between 1997 and 2005.

The children (22 girls, 17 boys; age range 4–17 years, median 5.4 years) were all in WHO functional classes III and IV. Twenty five had idiopathic pulmonary arterial hypertension and 14 pulmonary hypertension associated with congenital heart disease (6), cardiomyopathy (3), connective tissue disease (2), chronic interstitial lung disease (2) and HIV infection (1). Epoprostenol was started if oral therapies had failed or there were severe symptoms at presentation. Parents were trained to prepare and administer the drug at home and training was given to community health staff.

Over a mean follow‐up of 27 months, seven children died and eight underwent transplantation (four double lung, three heart and lung, one heart only). Cumulative survival at 1, 2, and 3 years was 94%, 90% and 84%, respectively. WHO functional class improved and the mean increase in 6‐min walking distance was 77 m. Fourteen children needed additional drug therapy (bosentan in eight, sildenafil in five, and both in one) and 17 with syncope or pre‐syncope had atrial septostomy. Epoprostenol therapy is effective in children.

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