A New Era in Medical Management of Severe Pediatric Pulmonary Arterial Hypertension

Pulmonary arterial hypertension (PAH) is a life-threatening disease whose prognosis has changed dramatically over the past decade since the introduction of new therapeutic agents as well as the off-label application of adult pulmonary hypertension specific therapies to children. Nevertheless, PAH still has no cure and the aim of treatment is to prolong survival by improving quality of life, symptoms, exercise capacity and hemodynamics. The selection of appropriate therapies for PH is complex and must be carefully chosen according to the etiology and pulmonary vasoreactivity. As insight advances into mechanisms responsible for the development of PAH, the introduction of novel therapeutic agents will hopefully further improve the outcome of this incurable disease. [Introduction] Pulmonary hypertension (PH) is an important determinant of morbidity and mortality in pediatric patients. Advances in understanding the pathobiology of pulmonary arterial hypertension (PAH) have led to novel therapies; however, there remains no cure for some forms of PH, as in idiopathic pulmonary arterial hypertension (IPAH). [Definition] PAH is defined as a mean pulmonary artery pressure (PAP) ≧25mmHg at rest, with a normal pulmonary capillary wedge pressure (≦15mmHg) and increased pulmonary vascular resistance index (≧3 Woods units・m2)1).