Abstract
Pulmonary arterial hypertension (PAH) in the pediatric population is associated with
a variety of underlying diseases and causes, significantly morbidity and mortality.
In the majority of patients, PAH in children is idiopathic or associated with congenital
heart disease (CHD), with pulmonary hypertension (PH) associated with connective tissue
disease, a rare cause in children. Classification of pediatric PH has generally followed
the WHO classification, but recognition of the importance of fetal origins of PH and
developmental abnormalities have led to the formation of a new pediatric-specific
classification. Incidence data from the Netherlands has revealed an annual incidence
and point prevalence of 0.7 and 4.4 for idiopathic PAH and 2.2 and 15.6 for associated
pulmonary arterial hypertension-CHD cases per million children. Although the treatment
with new selective pulmonary vasodilators offers hemodynamic and functional improvement
in pediatric populations, the treatments in children largely depend on results from
evidence-based adult studies and experience of clinicians treating children. A recent
randomized clinical trial of sildenafil and its long-term extension has led to disparate
recommendations in the United States and Europe.
Keywords
pulmonary arterial hypertension - congenital heart disease - children - pediatric
pulmonary vascular disease
