左肺動脈右肺動脈起始症

PURPOSE: In patients with a left pulmonary artery sling (LPAS) and congenital tracheal stenosis, indications for simultaneous slide tracheoplasty remain controversial. This study quantified three-dimensional tracheal morphologic changes after LPAS repair with and without slide tracheoplasty to provide insights into establishing surgical indications. METHODS: Airway geometries were reconstructed from pre- and postoperative computed tomography scans in 13 patients (10 with tracheoplasty, three with LPAS repair alone), measuring equivalent diameter and geometry-based flow resistance in a 30-mm segment proximal to the carina. All results are expressed as median [range]. RESULTS: The tracheoplasty group showed significant pericarinal enlargement (3.3 [2.4-4.1] to 5.2 [4.2-6.7] mm; P = 0.002) and a marked decrease in resistance estimates (2.30 [0.83-9.6] to 0.39 [0.11-1.88] cmH2O/(L/s); P = 0.004). In the LPAS repair alone group, two patients exhibited low preoperative resistance estimates (0.92 and 1.52 cmH2O/(L/s)), comparable to postoperative values in the tracheoplasty group. CONCLUSION: Slide tracheoplasty provides substantial morphologic and functional improvements, but certain patients achieve acceptable airway resistance with LPAS repair alone. Computational flow resistance may represent a useful tool for visualizing or quantifying the effects of surgical intervention.

OBJECTIVE: The study objective was to investigate the anatomic and clinical factors associated with tracheoplasty in children with pulmonary artery sling and congenital tracheal stenosis. METHODS: This retrospective multicenter study included children with pulmonary artery sling who underwent surgical treatment at 3 cardiac centers in China between January 2010 and September 2025. Preoperative clinical characteristics, computed tomography measurements, and operative data were reviewed. Univariable and multivariable logistic regression analyses were performed to identify factors independently associated with tracheoplasty. Interaction analyses were also conducted. RESULTS: After excluding 24 patients with incomplete preoperative imaging data, 330 patients with pulmonary artery sling and congenital tracheal stenosis were included. Among them, 302 (91.5%) underwent slide tracheoplasty and 28 (8.5%) underwent pulmonary artery sling correction alone. Younger age, long-segment tracheal stenosis, and more severe airway narrowing were independently associated with a higher frequency of tracheoplasty. CONCLUSIONS: Younger age, long-segment tracheal stenosis, and more severe airway narrowing were independently associated with tracheoplasty in this multicenter cohort. Tracheoplasty performance appeared to be influenced by multiple factors rather than any single imaging parameter. Further prospective studies with long-term follow-up are required to evaluate clinical outcomes associated with different management strategies.

Pulmonary developmental disorders comprise a heterogeneous group of congenital abnormalities involving the airways, lung parenchyma, pulmonary vasculature, or overlapping developmental pathways. Although these conditions commonly present during infancy or childhood, many remain clinically silent until adulthood, when they are discovered incidentally or manifest with recurrent respiratory symptoms such as infection, hemoptysis, or progressive dyspnea. These abnormalities may be broadly categorized into airway, vascular, parenchymal, and mixed developmental anomalies. Airway abnormalities include congenital tracheobronchomegaly, branching anomalies of the tracheobronchial tree, bronchial atresia, bronchogenic cysts, congenital pulmonary airway malformations (CPAM), and bronchopulmonary sequestration (BPS). Vascular anomalies include pulmonary artery sling, proximal interruption of the pulmonary artery, Scimitar syndrome, and meandering pulmonary veins. Parenchymal disorders encompass congenital lobar overinflation and pulmonary hypoplasia. Increasing recognition of overlapping lesions, particularly combinations of CPAM, sequestration, and bronchial atresia, has challenged traditional compartmental classification systems and supports a developmental spectrum model. This review synthesizes contemporary concepts regarding embryologic mechanisms, pathogenesis, imaging findings, and management of these entities in adults. Particular emphasis is placed on imaging evaluation with CT and MRI, updated terminology, evolving molecular understanding of CPAMs, including lesions with malignant potential, and distinctions between congenital sequestration and pseudosequestration. Recognition of these disorders and their associated anomalies is essential for accurate diagnosis, appropriate management, and surgical planning in adult patients.

An infant presented with persistent stridor and recurrent respiratory distress since birth. Imaging revealed a left pulmonary artery sling causing significant tracheal compression. Surgical reimplantation of the left pulmonary artery to the main pulmonary artery with concurrent slide tracheoplasty resulted in full recovery. This case highlights the importance of early recognition of vascular anomalies in infants presenting with unexplained airway obstruction.