Pulmonary Hypertension: Transition Challenges in the Current Therapeutic Era

The management of pulmonary hypertension has evolved significantly over the past 30 years. The introduction of targeted pulmonary arterial hypertension pharmacotherapies revolutionized care for a once untreatable disease. With improved outcomes, an increasing number of pediatric patients are surviving into adulthood. However, patients face a significant morbidity burden, due to both the disease and the complex therapies used to treat it. This chapter reviews the epidemiology and pathophysiology of pulmonary hypertension, the impact the disease has on quality of life, and the aspects of care that are important to address during the transition process. Improved outcomes are seen when patients are well-educated about their disease, actively participate in their care, and are appropriately supported throughout their disease course.