Pulmonary Hypertension in Children

A heterogeneous group of PH types and subtypes comprise the different forms of PH found in children. Children with PH present at any age with a variety of comorbid conditions and disease severity. The diagnostic approach to PH is fairly uniform regardless of age at presentation, and should at a minimum determine the PH classification and hemodynamic profile. While not uniformly employed in all cases, strong justification to omit cardiac catheterization should be provided for any pediatric PH case. Fortunately, largely based upon experience and application of adult trial data, short-term and long-term outcomes have improved over the past 30 years since the advent of modern PH-specific medications. Despite progress, however, well-funded clinical studies of pediatric PH remain of high need to determine best therapeutic approaches, features of risk and resilience to clinical decline, and nuanced treatment choices according to PH classification.