This article discusses diagnostic and therapeutic options of adolescents with primary and secondary pulmonary hypertension. Pulmonary hypertension is an important determinant of morbidity and mortality in many diseases, including congenital heart disease and respiratory disease. Previously, the diagnosis of pulmonary hypertension in children carried a poor prognosis. However, advances in the diagnosis and treatment of pulmonary hypertension over the last decade have markedly improved survival of many patients. A targeted approach includes treatment of the underlying disease, and therefore requires an extensive evaluation. Many of the treatment modalities are based on advanced understanding of basic pulmonary vascular biology.