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Pulmonary artery hypertension: new drug treatment in children

Correspondence to:
For correspondence:
Katrina Ford
Great Ormond Street Hospital NHS Trust, London, UK; fordk2@gosh.nhs.uk

Keywords: pulmonary artery hypertension; paediatrics

The first 150 words of the full text of this article appear below.

Pulmonary artery hypertension (PAH) is a rare progressive disease of the pulmonary vasculature. Although it affects fewer than two adults per million population, and probably fewer children, the clinical course can be one of rapid deterioration, with death occurring within three years of diagnosis for adults, and less than one year for children.^1,2 In the last decade, greater understanding of the disease has led to rethinking the diagnostic classification of pulmonary hypertension, and the identification of therapeutic targets for drug development. A number of new drugs have been studied in randomised controlled trials, and are licensed to treat PAH. A key message from the recent World Symposium on Pulmonary Arterial Hypertension was to refer patients to a centre with expertise in managing PAH; however, paediatricians may encounter patients already commenced on these novel drugs and delivery systems.³ This review will summarise the key trials of the new drugs recently marketed . . . [Full text of this article]