General Approach to the Care of the Child With Pulmonary Hypertension

Abstract

Pulmonary hypertension (PH) is a complex pulmonary vascular disease of multiple etiologies, characterized by inappropriate elevation of pulmonary vascular pressure and resistance that can present at any age, including during childhood. The pharmacologic care of patients with PH has seen tremendous advances in the past 20 years, improving survival and overall quality of life for patients of all ages, although mortality remains high.1 While the care of children with PH involves the same PH-specific medicines used for adults, PH-specific drugs are employed off-label due to a lack of Federal Drug Administration approval, which adds to the complexity of administration.2 Furthermore, in addition to pharmacologic therapy, multiple other factors are involved in the care of the pediatric PH patient that require careful consideration, such as differences relating to growth and developmental stages and according to PH etiology. This review will discuss some of the highly relevant pediatric-related issues outside of traditional pharmacologic factors, with a focus on the importance of a collaborative approach to care, genetic testing, and palliative care.