Pulmonary hypertension is relatively common in children and has many causes. The management of the condition has changed dramatically in the last five years with the introduction of new medicines.
Diagnosis, investigation and choice of therapy remain a challenge. In 2002 the United Kingdom Pulmonary Hypertension Service for Children was established and this has become the mainstay of management in this country. This Service, based at Great Ormond Street Hospital for Children, provides advice, expertise and infrastructure support for the most severely affected, particularly those with Idiopathic Pulmonary Arterial Hypertension for whom chronic intravenous prostacyclin remains the most effective medication.
New medicines are being developed which rather than focussing on dilating a diseased pulmonary vascular bed, aim to structurally remodel the pulmonary vasculature towards normal.