The association of congenital tracheobronchial stenosis and cardiovascular anomalies.

OBJECTIVES: Congenital tracheobronchial stenosis (CTBS) is a rare disorder characterized by the presence of focal or diffuse complete tracheal or bronchial cartilage rings resulting in a fixed lumen narrowing. The aim of this study was to expose the association of various cardiovascular (CV) anomalies with various types of CTBS.

METHODS: A retrospective review of 58 patients who had bronchoscopically proven CTBS between 1997 and 2011 was conducted. Totally, 48 cases who had received echocardiography, computed tomography and other examinations including cardiac catheterization were enrolled. There were 33 boys and 15 girls, and the median age of diagnosis was 4 months (0-44 months). The image findings of associated CV anomalies were reviewed and analyzed, along with the clinical manifestations and patients' outcome.

RESULTS: There were 14 generalized hypoplasia (29.2%), 10 funnel type (20.8%), 15 segmental type (31.3%), and 9 isolated bronchial stenosis cases (18.7%). Among 48 cases of CTBS, 32 patients (66.7%) had various CV anomalies including atrial septal defect (n=9), pulmonary artery sling (n=7), ventricular septal defect (n=7), patent ductus arteriosus (n=6), tetralogy of Fallot (n=3), peripheral pulmonary stenosis (n=3), etc. No significant difference existed in the incidence and types of comorbid CV anomalies among 4 types of CTBS, except that funnel type had significantly higher comorbidity of pulmonary artery sling (50%, p<0.01).

CONCLUSION: Patients with CTBS had high incidence of various associated CV anomalies. Isolated bronchial stenosis had similar high comorbidity of CV anomalies as did the 3 classical categories of congenital tracheal stenosis. The higher comorbidity of pulmonary artery sling in patients with funnel CTBS might be related to its gradual caudal narrowing of the trachea.