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Association between upper airway obstruction and pulmonary hypertension in children with Down syndrome

Kevin Guy1, Amal Isaiah1,2,3,4

  • 1Department of Otorhinolaryngology-Head and Neck Surgery, University of Maryland School of Medicine.

Current Opinion in Pulmonary Medicine
|May 27, 2026
PubMed

Insights

Pulmonary hypertension (PH) in children with Down syndrome (DS) is often linked to upper airway obstruction and obstructive sleep apnea (OSA). Further research is needed to improve PH diagnosis and treatment in this population.

Area of Science:

  • Pediatric Cardiology
  • Pulmonology
  • Genetics

Background:

  • Down syndrome (DS) is a common genetic disorder associated with significant health complications.
  • Pulmonary hypertension (PH) affects approximately 25% of children with DS, contributing to mortality.
  • Obstructive sleep apnea (OSA) is highly prevalent in DS, but its role in PH development is unclear.

Purpose of the Study:

  • To review current evidence on the contribution of upper airway obstruction to PH in children with DS.
  • To identify gaps in knowledge regarding PH diagnosis and management in this population.

Main Methods:

  • Systematic review of existing literature.
  • Analysis of studies investigating upper airway obstruction, OSA, and PH in Down syndrome.
  • Evaluation of diagnostic tools and treatment efficacy.

Main Results:

  • Children with DS experience multilevel upper airway obstruction contributing to OSA and hypoxemia.
  • OSA-related hypoxemia promotes pulmonary vascular remodeling, often classifying PH as WHO Group III.
  • Adenotonsillectomy may improve OSA but lacks proven impact on PH in DS. Endothelin receptor antagonists show promise, while sildenafil efficacy is questionable.

Conclusions:

  • A DS-specific PH staging system is critically needed.
  • Future research should focus on airway intervention impact on pulmonary hemodynamics, DS-tailored biomarkers, and optimized pharmacologic therapies.
Abstract

Keywords:
adenotonsillectomyobstructive sleep apneapulmonary hypertensionupper airway obstruction

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