P-19: Impaired Pulmonary Function and Ventilatory Limitation in Children With Successfully Repaired Oesophageal Atresia

Abstract

Significant respiratory morbidity, such as aspiration and recurrent pneumonia occur commonly in young children with oesophageal atresia/tracheo-oesophageal fistula (OA/TOF). Despite clinical improvement with age, lung function may be irreversibly impaired. The role of cardiopulmonary exercise testing (CPET) in the assessment of children with OA/TOF is unknown. To assess lung function, exercise capacity and ventilatory reserve in OA/TOF children. Prospective evaluation of children ≥ 7 years attending a multidisciplinary OA/TOF clinic. Clinical assessment, spirometry and CPET (Bruce Treadmill Protocol) was performed when the child was clinically well. Eighteen children, aged 7.3–17.9 years, performed spirometry according to ATS guidelines. Fifteen children also performed a maximal CPET. BMI z-score range was −4.3–1.25. Thirty-nine percent experienced chronic cough/wheeze during the last year; 33% had a respiratory admission in the previous year; 11% asthma, 61% had a “TOF” cough, 50% experienced exercise limitation and 33% had undergone an aortopexy. Abnormal spirometry (FVC or FEV1 < 80%) was noted in 73% and an additional 3 children had distal airway obstruction (FEF25–75% < 80%). Normal exercise capacity was found in 14 children (93%). However ventilation limitation (defined as Ventilator Reserve <20% normal) was observed in 11 (73%). Perceived limitation had a sensitivity of 73%, PPV of 89% and NPV of 57%. Spirometry had a sensitivity of 81%, PPV of 100% and NPV of 67%. Our preliminary results suggest that perceived exercise intolerance and spirometry are insensitive assessments of ventilatory limitation. A negative history or spirometric evaluation does not exclude diminished breathing reserve.