ReviewCurrent opinion in pulmonary medicine2026
Association between upper airway obstruction and pulmonary hypertension in children with Down syndrome.
Review in Current opinion in pulmonary medicine, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
purpose of reviewDown syndrome (DS) is the most common nonlethal chromosomal aneuploidy, affecting 1 in 700 live births. Pulmonary hypertension (PH) occurs in approximately 25% of children with DS and contributes to a 10% mortality rate within 3 years of diagnosis. Despite obstructive sleep apnea (OSA) affecting up to 80% of children with DS, the specific contribution of upper airway obstruction to PH in this population remains poorly characterized. This review synthesizes current evidence to address that gap. RECENT
findingsChildren with DS develop multilevel upper airway obstruction due to craniofacial dysmorphology, relative macroglossia, hypotonia, and reduced peripheral chemosensitivity. OSA-driven chronic hypoxemia promotes pulmonary vascular remodeling, with 87% of recurrent PH cases classified as WHO Group III. Echocardiographic diagnosis is limited by chronic lung disease. NT-proBNP is the most reliable biomarker in DS. Adenotonsillectomy reduces apnea severity, though evidence of a reduction in mean pulmonary arterial pressure in DS is lacking. Endothelin receptor antagonists show functional benefit, while sildenafil appears less effective in DS-specific analyses. SUMMARY: No DS-specific PH staging system exists, representing a critical gap. Future research should quantify the impact of airway interventions on pulmonary hemodynamics, develop DS-tailored biomarkers, and optimize pharmacologic regimens in this molecularly distinct population.
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