Pulmonary arterial hypertension in children: a medical update.

PURPOSE OF REVIEW

With rapid advances in the understanding and treatment of pulmonary arterial hypertension, navigating the pediatric literature becomes challenging. A comprehensive review of the most recent literature over the past year on available and emerging novel therapies as well as an approach to target pediatric populations will provide insight into the current management of pediatric pulmonary hypertension patients.

RECENT FINDINGS

Recent therapeutic advances have significantly improved the prognosis for children with pulmonary arterial hypertension. Pediatric pulmonary arterial hypertension continues to be a serious condition, however, which is extremely challenging to manage. There are also new target groups, such as those with sickle cell disease, congenital diaphragmatic hernia and Eisenmenger syndrome who may be candidates for treatments previously used for idiopathic pulmonary arterial hypertension patients.

SUMMARY

The data in children are often limited to case reports as many of those described here. Thus, the reader needs to be cautious about the interpretation of such small uncontrolled studies. While many of these data support the rationale for using novel agents for children with pulmonary arterial hypertension, further controlled and well designed studies are necessary to assess the true impact of these agents on various subgroups of children with pulmonary arterial hypertension.