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Pulmonary vascular complications of chronic airway obstruction in children
I N Jacobs1, W G Teague, J W Bland
1Department of Otolaryngology, Emory University School of Medicine, Atlanta, Ga., USA.
Insights
Pulmonary artery hypertension (PAH) can develop in children with chronic airway obstruction. Treatments like surgery or oxygen often improve PAH, but severe airway disease may lead to irreversible conditions.
Area of Science:
- Pediatric Cardiology
- Pulmonology
- Critical Care Medicine
Background:
- Chronic airway obstruction is a significant concern in pediatric patients.
- Pulmonary artery hypertension (PAH) is a serious complication that can arise from chronic airway obstruction.
- Understanding the link between these conditions is crucial for effective management.
Purpose of the Study:
- To investigate the clinical presentation of pediatric patients with chronic airway obstruction who develop PAH.
- To evaluate the treatment strategies and outcomes for this patient group.
- To identify risk factors associated with the development and progression of PAH in children with airway issues.
Main Methods:
- A case study design was employed, reviewing medical records of children with chronic airway obstruction and PAH.
- Data collection spanned three years at an academic tertiary care children's hospital.
- Clinical courses were analyzed using objective measures like cardiac catheterization, echocardiography, and electrocardiography, both pre- and post-treatment.
Main Results:
- Eighteen pediatric patients were identified with PAH secondary to chronic airway obstruction.
- Common causes included chronic lung disease, tracheobronchomalacia, and adenotonsillar hypertrophy.
- Treatments such as tracheotomy, adenotonsillectomy, and supplemental oxygen led to improvement in 14 patients, while 4 experienced worsening PAH, including 3 deaths.
Conclusions:
- Chronic airway obstruction is a potential cause of pulmonary artery hypertension in children.
- Premature infants, children with Down syndrome, and those with cardiac anomalies are at higher risk.
- While interventions can improve PAH, severe airway disease may result in fixed, irreversible pulmonary artery hypertension.
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.