A
Yes! Certain congenital airway anomalies can increase the risk of pediatric pulmonary hypertension (PH) due to chronic hypoxia, ventilation-perfusion mismatch, and increased pulmonary vascular resistance. Key airway anomalies associated with PH risk include:
1. Tracheomalacia & Bronchomalacia
- Dynamic airway collapse leads to air trapping and hypoxia, increasing pulmonary vascular resistance.
- Common in conditions like TEF (tracheoesophageal fistula) repair, bronchopulmonary dysplasia (BPD), and congenital heart disease (CHD).
2. Laryngomalacia
- Chronic upper airway obstruction causes intermittent hypoxia and can contribute to pulmonary hypertension if severe.
- Seen in syndromes like Pierre Robin sequence and 22q11.2 deletion syndrome.
3. Congenital Subglottic or Tracheal Stenosis
- Causes chronic airway obstruction, increasing work of breathing and hypoxic episodes.
- Associated with pulmonary hypertension if untreated due to persistent hypoxia.
4. Tracheal Compression (Vascular Rings, Slings, & Mediastinal Masses)
- Anomalous aortic arch, double aortic arch, or pulmonary artery sling can compress the trachea, leading to airway obstruction and chronic hypoxia.
- These conditions are strongly linked to secondary pulmonary hypertension if not surgically corrected.
5. Severe Obstructive Sleep Apnea (OSA) in Children
- Often seen in craniofacial syndromes (e.g., Down syndrome, Pierre Robin sequence) and conditions like adenotonsillar hypertrophy.
- Leads to chronic nocturnal hypoxia and hypercapnia, increasing pulmonary artery pressures.
Clinical Implications:
- Early recognition of airway anomalies is critical to preventing chronic hypoxia-induced pulmonary hypertension.
- Consider pulmonary hypertension screening (echocardiography, BNP levels) in children with chronic airway obstruction.
- Multidisciplinary management (pulmonology, cardiology, ENT, surgery) is often required.
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