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Pulmonary function and exercise capacity in survivors of congenital diaphragmatic hernia
M G Peetsold1, H A Heij, A F Nagelkerke
1Dept of Paediatrics, VU University Medical Centre, P.O. Box 7057, 1007 MB Amsterdam, The Netherlands. m.peetsold@vumc.nl
Insights
Survivors of high-risk congenital diaphragmatic hernia (CDH) often have mild lung function deficits. Gastro-oesophageal reflux disease may contribute to these pulmonary issues in children with CDH.
Area of Science:
- Pediatric Pulmonology
- Congenital Abnormalities
- Respiratory Medicine
Background:
- Congenital diaphragmatic hernia (CDH) is linked to underdeveloped lungs and high blood pressure in the lungs.
- Early respiratory distress defines high-risk CDH, necessitating long-term outcome assessment.
Purpose of the Study:
- To evaluate pulmonary function and exercise capacity in children and adolescents with high-risk CDH.
- To identify early factors influencing pulmonary outcomes in CDH survivors.
- To correlate findings with the initial severity of CDH.
Main Methods:
- Pulmonary function tests (spirometry, lung volumes) and maximal cardiopulmonary exercise testing (CPET) were performed on 53 high-risk CDH survivors (mean age 11.9 years).
- Results were compared to a matched healthy control group.
- Linear regression analyzed determinants of pulmonary function.
Main Results:
- CDH survivors exhibited significantly reduced FEV(1), FVC, FEV(1)/FVC, and peak expiratory flow compared to controls.
- A higher residual volume/total lung capacity ratio was observed in CDH survivors.
- Gastro-oesophageal reflux disease was identified as an independent factor for decreased FEV(1) and FVC.
Conclusions:
- High-risk CDH survivors present with mild to moderate pulmonary function abnormalities.
- Pulmonary issues may be associated with early-life gastro-oesophageal reflux disease.
- Exercise capacity and gas exchange were generally normal, suggesting minimal physical impairment for most survivors.
Abstract:
Congenital diaphragmatic hernia (CDH) is associated with pulmonary hypoplasia and pulmonary hypertension. The objective of this study was to assess pulmonary function and exercise capacity and its early determinants in children and adolescents born with high-risk CDH (CDH-associated respiratory distress within the first 24 h) and to explore the relationship of these findings with CDH severity. Of 159 patients born with high-risk CDH, 84 survived. Of the 69 eligible patients, 53 children (mean+/-SD age 11.9+/-3.5 yrs) underwent spirometry, lung volume measurements and maximal cardiopulmonary exercise testing (CPET). Results of the pulmonary function tests were compared with those from a healthy control group matched for sex, age and height. CDH survivors had a significantly lower forced expiratory volume in 1 s (FEV(1)), forced vital capacity (FVC), FEV(1)/FVC, maximum mid-expiratory flow and peak expiratory flow when compared with healthy controls. The residual volume/total lung capacity ratio was significantly higher. Linear regression analysis showed that gastro-oesophageal reflux disease was an independent determinant of reduced FEV(1) and FVC. CPET results were normal in those tested. High-risk CDH survivors have mild to moderate pulmonary function abnormalities when compared with a healthy matched control group, which may be related to gastro-oesophageal reflux disease in early life. Exercise capacity and gas exchange parameters were normal in those tested, indicating that the majority of patients do not have physical impairment.
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