Pediatric Pulmonary Hypertension.

Abstract

Pulmonary hypertension (PH) is a rare disease in newborns, infants, and children that is associated with significant morbidity and mortality. In the majority of pediatric patients, PH is idiopathic or associated with congenital heart disease and rarely is associated with other conditions such as connective tissue or thromboembolic disease. Incidence data from the Netherlands has revealed an annual incidence and point prevalence of 0.7 and 4.4 for idiopathic pulmonary arterial hypertension and 2.2 and 15.6 for pulmonary arterial hypertension, respectively, associated with congenital heart disease (CHD) cases per million children. The updated Nice classification for PH has been enhanced to include a greater depth of CHD and emphasizes persistent PH of the newborn and developmental lung diseases, such as bronchopulmonary dysplasia and congenital diaphragmatic hernia. The management of pediatric PH remains challenging because treatment decisions continue to depend largely on results from evidence-based adult studies and the clinical experience of pediatric experts. (J Am Coll Cardiol 2013;62:D117–26) a 2013 by the American College of Cardiology Foundation Pulmonary hypertension (PH) can present at any age from infancy to adulthood. The distribution of etiologies in children is quite different than that of adults, with a predominance of idiopathic pulmonary arterial hypertension (IPAH) and PAH associated with congenital heart disease (APAH-CHD) (1–5). In pediatric populations, IPAH is usually diagnosed in its later stages due to nonspecific symptoms. Without appropriate treatments, median survival rate after diagnosis of children with IPAH appears worse when compared with that of adults (6). Therapeutic strategies for adult PAH have not been sufficiently studied in children, especially regarding potential toxicities, formulation, or optimal dosing, and appropriate treatment targets for goal-oriented therapy in children are lacking. Nevertheless, children with PAH are currently treated with targeted PAH drugs and may benefit from these new therapies. This review provides an overview of recent information regarding the current approach and diagnostic classification of PAH in children as based on discussions and recommendations from the Pediatric Task Force of the 5th World Symposium on Pulmonary Hypertension (WSPH) in Nice, France (2013).