Long Term Respiratory Outcomes And Lung Function Following Vascular Ring Repair

Abstract

M. E. Soto-Martinez1, A. F. Gibson2, A. D. Cochrane3, P. Robinson11Royal Children's Hospital, Melbourne, Australia, 2Murdoch Children's Research Institute, Melbourne, Australia, 3Monash Medical Centre,Clayton, AustraliaCorresponding author's email: manuel.soto@rch.org.auBACKGROUND: Vascular rings (VR) are a group of congenital vascular malformations that result in extrinsic compression of theoesophagus and large airways. In symptomatic patients, the treatment of choice is surgical repair. Long-term respiratory outcomespost-surgery are unknown. AIM: To evaluate the respiratory function post-surgery in patients who had VR surgery early in life. METHODS:Patients who underwent VR surgery between 1977 to 2002 were invited to complete a symptom questionnaire detailing respiratorysymptoms in the past 12 months, and also to perform lung function testing at rest and after exercise. RESULTS: 16 patients (9 males) wererecruited: 12 had double aortic arch and 4 patients had a right aortic arch with left ligamentum arteriosum. Median age at diagnosis was9.5 (0.55 – 60.6) months. Surgery was performed at 10.3 (0.64 – 62.7) months of age. The mean age at review was 12.1 (6.94 – 31.8) years,mean time after surgery was 12 (5.5 – 30.5) years. Eleven (68.7%) patients described persistent respiratory symptoms following surgicalrepair, mostly barking cough, noisy breathing during exercise, and restriction in exercise. Eleven (68.7%) children had a history of coughfor more than 3 weeks following colds. In the preceding 12 months, seven (43.7%) patients had a diagnosis of asthma, however fromhistory just two responded clinically to bronchodilators. Seven (43.7%) missed school in the last 12 months because of respiratorysymptoms (mean = 17.5 school days). Mean baseline FEV1 (% predicted) was 90% (84 – 95), FVC 93% (86 – 101) and MMEF 78% (71 – 86).Mean post-exercise FEV1 (% predicted) was 86% (80 - 91), FVC 92% (86 - 97) and MMEF 72% (64 - 80). Fifteen patients (93.8%) had anabnormal flow volume loop at rest (either abnormal inspiratory or expiratory curve), 7(43.7%) having an abnormality during both phases.All patients had an abnormal flow volume loop post-exercise, 11 (73.3%) showing an abnormality in both inspiratory and expiratory loops.No patient had a significant reduction in FEV1 post-exercise. CONCLUSIONS: Tracheomalacia years after VR repair seems to be morefrequent than previously reported. Resting lung function in surgically corrected patients is normal although complete flow volume loopsshows widespread residual tracheomalacia. All patients showed abnormalities of lung function during and immediately postexercise.Long term respiratory follow up should be included in the review programme for children following VR surgery, especially those patientsthat remain with respiratory symptoms.This abstract is funded by: None