Clinical practice: pulmonary hypertension in children

Pulmonary arterial hypertension is a rare disorder in childhood, the two most common types being idiopathic pulmonary arterial hypertension and pulmonary hypertension associated with congenital left-to-right shunt lesions, together accounting for almost 90% of cases.

The clinical presentation of idiopathic pulmonary arterial hypertension (familial and non familial) is essentially non-specific and varies with age. Pulmonary vasoreactivity testing identifies responders and non-responders. Responders are treated with calcium channel blockers and have a better prognosis. Non-responders have a very limited survival beyond diagnosis if not treated with more selective pulmonary arterial vasodilators. Prostacyclin, endothelin receptor antagonists and phosphodiesterase-5 inhibitors improve haemodynamics, functional class and exercise tolerance and delay deterioration. Patients with congenital left-to-right shunts and irreversible pulmonary arterial hypertension leading to Eisenmenger’s syndrome have multiple organ disease. Despite a very pronounced exercise intolerance, their clinical course is rather stable with survival up to 40–60 years, depending on the complexity of their underlying cardiac defect. Treatment is based on general measures along with the same three types of selective pulmonary vasodilators as in idiopathic pulmonary arterial disease. Improvement in haemodynamics, functional class and exercise tolerance are comparable for both patient groups.

Pulmonary hypertension in children is idiopathic or associated with congenital heart disease in the majority of patients. Treatment with new selective pulmonary vasodilators offers haemodynamic and functional improvement.