Pulmonary function, exercise performance, and growth in survivors of congenital diaphragmatic hernia

Abstract

A cohort of survivors of congenital diaphragmatic hernia (CDH), with matched controls, was studied to assess growth, respiratory function, and exercise performance. Nineteen of 24 survivors from an 11 year period (79%) were compared with 19 matched controls. Subjects had detailed auxology, performed spirometry and cycle ergometry, and completed questionnaires about respiratory symptoms and exercise. There were no significant diVerences between the groups for height, weight, sitting height, head circumference, or body mass index expressed as SD scores. The mean (95% confidence interval) percentage predicted forced vital capacity (FVC) was 84.7% (79.1 to 90.3) in index cases and 96.5% (91.4 to 101.6) in controls (p < 0.01). There was no significant diVerence in total lung capacity. Expiratory flow rates corrected for FVC were also similar between groups, suggesting normal airway function relative to lung size. Mean maximum oxygen consumption in ml/kg/min was 40.1 (36.8 to 43.4) and 42.2 (38.5 to 45.8) in index and control cases. These diVerences were not significant. Index cases achieved a similar minute ventilation to controls by more rapid and shallower breathing. Index cases had lower perception of their own fitness and lower enjoyment of exercise, although habitual activity levels were similar. Survivors of CDH repair have reduced functional lung volumes, but normal airway function compared with matched controls. They have no growth impairment nor significant impairment of exercise performance, although they have more negative perceptions of their own fitness. They should be encouraged and expected to participate fully in sport and exercise. (Arch Dis Child 1998;78:137-142)

The long-term follow-up of patients with a congenital diaphragmatic hernia: a broad spectrum of morbidity

“…Some longterm follow-up studies indicate a higher incidence of restrictive lung function impairment among CDH patients compared to the healthy population [63,65] especially when there was a large diaphragmatic defect [65], while others cannot confirm this finding [59,66]. Overall CDH patients appear not to have an important reduction of total lung capacity in their school-and adolescence years [66,68,69].…”

“…All participating patients considered themselves healthy and they did not have any particular symptom. In the study from Marven et al [66], CDH patients perceived their own fitness to be worse than the healthy control group. Other studies demonstrate that around 20% of interviewed CDH patients consider themselves less fit than their healthy peers [59,65,73].…”

“…Spirometric testing of CHD patients revealed evidence of obstructive airway disease [64] when compared with healthy controls [48,62,64,[66][67][68][69]. Some authors describe that one-third of the CDH patients have reduced forced expiratory volume in 1 s (FEV 1 ) and forced vital capacity (FVC) [62,69].…”

“…Furthermore, there is evidence for more peripheral airway obstruction in CDH patients [69] possibly due to structural abnormalities in distal airways [69]. It has been speculated that there must be some degree of emphysema because of the finding of a high residual volume total lung capacity (RV/TLC) ratio, despite the lack of airway dysfunction [66]. Others suggest that the high RV/TLC ratio (index for the amount of trapped air after full exhalation) is associated with chest wall deformity [68].…”

“…Sport practice and cardiopulmonary exercise testing CDH patients achieve normal aerobic capacity (V 0 O 2 max) (an index for maximal oxygen uptake during maximal exercise; it can determine an athlete's capacity and is linked to aerobic endurance) when performing a maximal exercise test [66], which is compatible with the normal diffusion capacity that has been described [69,72]. Nonetheless, Italian investigators report significantly lower V 0 O 2 max for CDH patients representing a decreased degree of physical fitness [67].…”