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Pulmonary arterial hypertension in paediatric and adult patients with congenital heart disease
European Respiratory Review, 09/03/09
Gatzoulis MA et al. – Uncontrolled studies suggest that prostacyclin analogues and phosphodiesterase type–5 inhibitors may have benefits in advanced pulmonary vascular disease. In the only randomised controlled trial dedicated to end–stage PAH–CHD, bosentan significantly reduced pulmonary vascular resistance and significantly increased 6–minute walk distance without compromising peripheral oxygen saturation, in patients with Eisenmenger syndrome. These data suggest that targeted therapies are beneficial in the PAH–CHD population, and warrant further research.
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