The introduction of new medicines to treat pulmonary vascular disease has renewed interest in the management of children with idiopathic pulmonary arterial hypertension. We now have a practical classification of pulmonary hypertension, improved diagnostic techniques, and internationally recognized diagnostic and management guidelines. In 2009 the UK Pulmonary Hypertension Service for Children reported survival figures for idiopathic pulmonary arterial hypertension of 85.6% at 1 year, 79.9% at 3 years, and 71.9% at 5 years. A good quality of life can be assured for many years.
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