This is an updated, comprehensive and practical guideline for healthcare providers providing care to children and young adults with pulmonary hypertension (PH) and pulmonary vascular disease (PVD). Methodology included an executive writing group consisting of 22 pediatricians, 7 adult congenital heart disease physicians, 1 adult pulmonologist and 1 thoracic surgeon. Specific features of this guideline include new patient groups such as PH associated with congenital heart disease (PAH-CHD), persistent PH of the newborn period (PPHN) as well as PH associated with bronchopulmonary dysplasia (BPD) or chronic lung disease (CLD). The guidelines also include new recommendations for the treatment of acute PH in the intensive care unit (ICU). This is also the first time the challenges of addressing PH in middle to low income regions are addressed. The following class of recommendation (COR) and level of evidence (LOE) grading were used in this guideline.