Pulmonary Hypertension in Infants, Children, and Young Adults

Abstract

Pulmonary hypertension (PH) in neonates, infants, children, adolescents, and young adults is a complex condition that can be associated with several cardiac, pulmonary, and systemic diseases contributing to morbidity and mortality. The underlying pulmonary hypertensive vascular disease (PHVD) is characterized by inflammation, pulmonary vascular remodeling, and angio-obliteration leading to elevated pulmonary arterial pressure and resistance, right ventricular dysfunction, left ventricular compression, and subsequent heart failure. Recent advancements in PH-targeted therapies and interventional-surgical procedures have contributed to the improvement in quality of life and survival in PH/PHVD. This paper gives an update on recent developments in the diagnosis and treatment of children and young adults with PH. The focus is on the heterogeneous etiology/pathophysiology of PH in the young, and particularly on PHVD associated with congenital heart disease. Moreover, new pharmacological, surgical, and interventional therapies and their practical application in progressive/severe pulmonary arterial hypertension with inadequate response to conventional pharmacotherapy are discussed.

Keywords: congenital heart disease; developmental lung disease; pulmonary vascular disease; right heart failure; risk factors; treatment algorithm.