The prevalence and clinical impact of pulmonary artery sling on school-aged children: a large-scale screening study.

摘要

BACKGROUND: Airway obstruction is a leading cause of mortality and morbidity in patients with pulmonary artery sling (PAS). Early identification of PAS is imperative for prompt initiation of appropriate treatments. OBJECTIVE: Our aim was to investigate the prevalence and clinical impact of PAS on school-aged children through a large-scale pre-sports participation cardiovascular screening (PPCVS) study. METHODS: A total of 186,213 school-aged children underwent portable two-dimensional echocardiography (2DE) study in a PPCVS study between 2001 and 2004 in Taichung City, Taiwan. We prospectively collected data on the prevalence of PSA, demographics, clinical features, tracheo-bronchial anomalies (TBA), on computed tomography (CT), pulmonary function test (PFT), follow-up data in 1 year, and clinical outcomes. RESULTS: PAS was identified in 11 children (8 males/3 females) with a median age of 13 years (range: 7.2-13.7 years). The prevalence of PAS was 1 in 17,000 school-aged children. The majority of children with PAS had recurrent broncho-pulmonary infections (90.9%) and asthmatic cough (81.8%), which had a low positive predictive value of PAS (<1%). Seven patients had diffused complete cartilaginous rings. The other four patients were found to have prominent localized external compression of the trachea or bronchus. No coexisting intracardiac anomalies were found. Two patients had mental retardation. Seven patients underwent surgical correction without mortality or major complications. After surgery, significant improvement in PFT was shown in forced expiratory volume in 1 s (FEV1) and functional vital capacity (FVC). CONCLUSIONS: This is the first study to determine the prevalence of PAS as 59 per million school-aged children by a large-scale screening using 2DE. Although the majority of children with PAS have associated TBA and impaired pulmonary function, all patients with PAS were unrecognized due to non-specific symptoms. Early identification of PAS and surgery result in significant improvement in pulmonary function.