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Treatment and survival in children with pulmonary arterial hypertension: the UK Pulmonary Hypertension Service for Children 2001-2006.

Haworth, SG; Hislop, AA; (2009) Treatment and survival in children with pulmonary arterial hypertension: the UK Pulmonary Hypertension Service for Children 2001-2006. Heart , 95 (4) pp. 312-317. 10.1136/hrt.2008.150086.

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Abstract

OBJECTIVE: A retrospective study of the UK Pulmonary Hypertension Service for Children for the first 5-year period of its existence. DESIGN AND PATIENTS: Records of 216 children with idiopathic pulmonary arterial hypertension (IPAH) and associated pulmonary arterial hypertension (APAH) were reviewed. Kaplan-Meier survival curves were constructed for different diagnostic groups and for different therapies. RESULTS: At cardiac catheterisation only 7.4% of those with IPAH and 6% of those with APAH responded positively to vasodilator testing and so were treated with nifedipine. Others needing treatment were given continuous intravenous epoprostenol, bosentan or sildenafil singly or in combination. For IPAH survival rates were 85.6%, 79.9% and 71.9% at 1, 3 and 5 years, respectively, compared with a survival time of less than a year in historical untreated controls. A combination of intravenous epoprostenol with either bosentan or sildenafil, or both, appeared to achieve the best outcome. Six children underwent lung transplantation. In APAH survival rates were 92.3%, 83.8% and 56.9% at 1, 3 and 5 years, respectively, postoperative congenital heart disease with severe pulmonary hypertension having the worst outcome. CONCLUSION: New pulmonary hypertension-specific medicines have improved survival in children as in adults. Outcome in this series compares favourably with international outcome data.

Type: Article
Title: Treatment and survival in children with pulmonary arterial hypertension: the UK Pulmonary Hypertension Service for Children 2001-2006.
Location: England
DOI: 10.1136/hrt.2008.150086
Keywords: Adolescent, Case-Control Studies, Child, Child, Preschool, Epoprostenol, Female, Great Britain, Heart Defects, Congenital, Humans, Hypertension, Pulmonary, Infant, Kaplan-Meier Estimate, Lung Transplantation, Male, Nifedipine, Piperazines, Purines, Retrospective Studies, Sildenafil Citrate, Sulfonamides, Sulfones, Survival Rate, Vasodilator Agents
UCL classification: UCL > School of Life and Medical Sciences
UCL > School of Life and Medical Sciences > Faculty of Population Health Sciences
UCL > School of Life and Medical Sciences > Faculty of Population Health Sciences > Institute of Child Health
URI: http://discovery.ucl.ac.uk/id/eprint/174898
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