Pulmonary hypertension in infants and children

Abstract

Pulmonary hypertension (PHT) is a condition defined by a mean pulmonary arterial pressure (PAP) of 25 mmHg or greater at rest and 30 mmHg or greater during exercise. PHT can be idiopathic or associated with a wide spectrum of etiological factors and conditions in the pediatric population, often resulting in diagnostic and therapeutic challenges. Due to recent advances in treatment which have markedly decreased the mortality and morbidity in pediatric patients with PHT, early and correct diagnosis is particularly essential in optimally managing children with PHT. In this chapter, we address the clinical presentation and the methods of evaluation of PHT in the pediatric population. Unique underlying pathophysiological processes and characteristic diagnostic study findings in idiopathic pulmonary arterial hypertension (IPAH) and common conditions typically associated with PHT in infants and children are also highlighted.