The following is a summary of “Pulmonary Hypertension in Children with Down Syndrome: Results from the Pediatric Pulmonary Hypertension Network Registry,” published in the JANUARY 2023 issue of Pediatrics by Hopper, et al.

For a study, researchers sought to analyze and compare the characteristics, comorbidities, treatment patterns, and outcomes of children with Down syndrome and pulmonary hypertension in an extensive, multicenter pediatric pulmonary hypertension registry. 

The researchers analyzed data from the Pediatric Pulmonary Hypertension Network (PPHNet) Registry and examined the factors associated with pulmonary hypertension resolution and a composite outcome of pulmonary hypertension severity in the cohort with Down syndrome. 

Of the registry’s 1,475 pediatric patients with pulmonary hypertension, 158 (11%) had Down syndrome. The median age at diagnosis of pulmonary hypertension in patients with Down syndrome was 0.49 years (IQR, 0.21-1.77 years), similar to that in patients without Down syndrome. There was no significant difference in rates of cardiac catheterization and prescribed pulmonary hypertension medications between children with Down syndrome and those without. Comorbidities in Down syndrome included congenital heart disease (95%; repaired in 68%), sleep apnea (56%), prematurity (49%), recurrent respiratory exacerbations (35%), gastroesophageal reflux (38%), and aspiration (31%). Pulmonary hypertension resolved in 43% of children with Down syndrome after 3 years, and it was associated with an early diagnosis of pulmonary hypertension (at age <6 months) and a pretricuspid shunt (65% vs 38%; P = .02). The five-year transplantation-free survival was 88% (95% CI, 80%-97%). Tracheostomy (hazard ratio [HR], 3.29; 95% CI, 1.61-6.69) and reflux medication (HR, 2.08; 95% CI, 1.11-3.90) use were independently associated with a composite outcome of severe pulmonary hypertension. 

Children with pulmonary hypertension and Down syndrome generally had a similar survival rate to children without pulmonary hypertension associated with Down syndrome, despite the high prevalence of comorbidities that affected the severity of pulmonary hypertension. However, among children with complex respiratory comorbidities, the remission of pulmonary hypertension was less common.