Objective: To study the clinical presentation, treatment, and outcome of pulmonary artery hypertension (PAH) that develops in children with chronic airway obstruction.
Design: Case study.
Setting: Academic tertiary care children's hospital.
Patients: A 3-year (October 1, 1992, to September 30, 1995) review of the medical records of all children with chronic airway obstruction in whom PAH developed.
Main outcome measure: The clinical course, including objective laboratory data to measure PAH (cardiac catheterization, echocardiography, electrocardiography) both before and after treatment.
Results: Pulmonary artery hypertension developed in 18 patients. It was diagnosed using cardiac catheterization in 13 patients and echocardiography in 5 patients. Obstructive problems include chronic lung disease (9 patients), tracheobronchomalacia (6 patients), adenotonsillar hypertrophy (5 patients), laryngomalacia (4 patients), macroglossia (5 patients), subglottic stenosis (2 patients), and pharyngeal collapse (2 patients). Nine patients were born prematurely and 7 had Down syndrome. Treatments included tracheotomy (7), adenotonsillectomy (5), adenoidectomy (3), laser epiglottoplasty (1), and supplemental oxygen (12). Fourteen patients had documented improvement of PAH as seen from the cardiac catheterization, echocardiography, or electrocardiography findings; in 4 patients, PAH worsened (3 deaths).
Conclusions: Chronic airway obstruction may lead to PAH. In this study, PAH was more likely to develop in premature infants or children with Down syndrome and cardiac anomalies. Surgery or supplemental oxygen will usually improve PAH, but fixed and irreversible PAH developed in patients with the most severe airway disease.