Abstract
Background
Respiratory morbidity in children with esophageal atresia/tracheoesophageal fistula (OA/TOF), due to impaired mucociliary clearance, recurrent infections, and aspiration, may impair pulmonary function during childhood and this may persist into adult life. Early recognition of reduced lung function could optimize respiratory management and improve long-term outcomes. However, static lung function assessments, using spirometry and body plethysmography, may not sensitively identify impaired lung function. Cardiopulmonary exercise testing (CPET), which involves a dynamic assessment of respiratory function and determines aerobic capacity, degree of fitness, and ventilatory (breathing) reserve during maximal exertion, may detect poor lung function earlier.
Aim
This study determines the clinical utility of cardiopulmonary exercise testing in children with OA/TOF.
Methods
Retrospective chart review of children attending a multidisciplinary OA/TOF clinic who underwent spirometry, plethysmography, and a maximal CPET (Bruce Treadmill Protocol). Plethysmography and CPET were performed on the same day in 16 children; 4 children within 2 days and 6 within 2 months. Studies exceeding 2 months were not analyzed (n = 2).
Results
Thirty-nine children, aged ≥ 7 years, underwent CPET when clinically well. Thirty-two children, 7–18 years, achieved a maximal CPET (82.1%). There was no significant exercise-induced drop in PPFEV1 in any child. One child experienced a reduction in oxygen saturation to 92%. Exercise capacity (VO2MAX, peak oxygen consumption) was normal in 30 children (93.8%). However reduced ventilatory or breathing reserve (BR), defined as <20% predicted, was identified in 18 children (56.3%). Four of these children had no ventilatory reserve (BR = 0). In contrast, spirometry was normal in 20 children (62.5%). Nine (28%) had a mild obstructive or restrictive pattern. One child had moderate restriction and 2 had a mixed pattern. Plethysmography (n = 26) revealed mild restrictive lung disease in 9 (34.6%). An additional 3 children had air-trapping.
Conclusion
Children with OA/TOF have significant lung function impairment. Spirometry and plethysmography revealed normal or mildly abnormal airway function/lung volumes in the majority of children. CPET identified significant ventilation limitation in over half of all children. CPET assessment is a feasible and sensitive assessment of cardiorespiratory function in children with OA/TOF. Further evaluation of risk factors and longitudinal CPET assessments may inform future management guidelines.
Comorbidities and course of lung function in patients with congenital esophageal atresia
