Pulmonary Hypertension Is Different in Children Than in Adults

To better understand the clinical features of pulmonary hypertension in children, researchers analyzed prospective data from 362 consecutive patients (age, <18 years; 59% female) with pulmonary hypertension who were enrolled in the international, industry-sponsored Tracking Outcomes and Practice in Pediatric Pulmonary Hypertension registry. Pulmonary hypertension was diagnosed on the basis of right heart catheterization.

Most patients (88%) had pulmonary arterial hypertension (PAH); of these, 57% had idiopathic or familial PAH, and 43% had PAH associated with other disorders (mainly congenital heart disease). Twelve percent of patients had pulmonary hypertension associated with respiratory disorders or hypoxemic conditions (most often bronchopulmonary dysplasia); this condition was more frequent in children aged 3 to 24 months at diagnosis than in older children. Overall, 13% of patients had chromosomal abnormalities (primarily trisomy 21). Dyspnea on exertion and fatigue were the most common symptoms. Syncope was reported most often in idiopathic and familial PAH and did not occur in children with unrepaired or residual systemic-to-pulmonary shunts. Most (64%) of the cohort, including some patients with severe pulmonary hypertension, were in the best functional classes.