Pulmonary function and exercise capacity in survivors of congenital diaphragmatic hernia

¹ VU University Medical Centre, Amsterdam, The Netherlands, Dept of paediatrics
² Paediatric Surgical Centre of Amsterdam (VU University Medical Centre/Emma Children's Hospital/AMC), The Netherlands
³ Erasmus Medical Centre/Sophia Children's Hospital, Rotterdam, The Netherlands, Dept of paediatric Surgery
⁴ Erasmus Medical Centre/Sophia Children's Hospital, Rotterdam, The Netherlands, Dept of paediatrics

^* To whom correspondence should be addressed. E-mail: m.peetsold{at}vumc.nl.

	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 hours) and to explore the relation of these findings with CDH severity.

Of 159 patients born with CDH, 84 survived. Of the 69 eligible patients, 53 children (mean age 11.9±3.5 years) underwent spirometry, lung volume measurements and maximal Cardiopulmonary Exercise Testing (CPET). Results of the pulmonary function tests were compared to those from a healthy control group matched for gender, age and height.

CDH survivors have a significantly lower FEV₁, FVC, FEV₁/FVC, MMEF and PEF when compared to healthy controls. RV/TLC ratio was significantly higher. Linear regression analysis showed that gastro-esophageal reflux disease was an independent determinant of reduced FEV₁ and FVC. CPET results were normal in those tested.

High risk CDH survivors have mild to moderate pulmonary function abnormalities when compared to a healthy matched control group, which may be related to gastro-esophageal 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.