Pediatric Pulmonary Hypertension

Pediatric pulmonary hypertension (PH) is a hemodynamic condition characterized by an increased mean pulmonary arterial pressure more than 20 mmHg measured with right heart catheterization. Pulmonary hypertension can be caused by a large variety of diseases and is subdivided into five different classes. Pulmonary arterial hypertension (PAH) is a specific class of PH characterized by typical pulmonary vascular remodeling. Although survival in children with PAH has improved over the last decades, mortality and morbidity remain high. Presenting symptoms of children with PH are often nonspecific. The most common complaint is exercise intolerance and dyspnea with exertion. Syncope occurs more often in children compared to adults with PH. Also, PH in childhood often presents with concomitant genetic mutations or syndromic traits. In every child with suspicion of PH, an extensive diagnostic work-up is indicated, often requiring referral to an expert center. A specific form of pulmonary vascular disease presenting in infancy is PH due to developmental lung diseases. The most frequent example is PH associated with bronchopulmonary dysplasia (BPD) in preterm infants. In contrast to the progressive nature of PAH, BPD-associated PH may eventually resolve. Currently, medications are available that target three different pathways involved in the pathophysiology of PAH. The current pediatric treatment algorithm, recommended by the pediatric task force of the World Symposium for Pulmonary Hypertension, is based on risk stratification at time of diagnosis.