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Background: Pulmonary artery sling is commonly associated with tracheal stenosis and intracardiac anomalies. While surgical repair is standardized, coexistent anomalies often determine outcomes. With the paucity of risk stratification, this study aimed to review our experience and stratify risk factors for the surgical outcome of complex pulmonary artery sling repair in the presence of airway or intracardiac lesions., Methods: Seventy-nine consecutive children with pulmonary artery sling were evaluated retrospectively following surgical repair. Median age at surgery was 5 months (interquartile range 3-9). Surgical approaches included pulmonary artery sling alone ( n  = 10), pulmonary artery sling with tracheoplasty ( n  = 41), and pulmonary artery sling with both intracardiac and tracheal surgery ( n  = 28)., Results: There were 7 early (8.8%) deaths. Two patients after left pulmonary artery reimplantation needed revision of the anastomosis. The median intensive care and hospital stay were 11 (interquartile range 9.2-24.8) and 17.9 (interquartile range 4.3-19.8) days, and considerably longer when associated tracheal surgery ( p  = 0.002). Follow-up was complete in 66/69 and 3 (3.8%) children died late: 2.7, 10.2, and 17 months after surgery. Univariate analysis showed abnormal lung and coexisting structural heart disease as risk factors. Multivariate analysis revealed total cardiopulmonary bypass time as an independent predictor of overall mortality., Conclusion: Complex pulmonary artery sling repair can be performed with a good surgical outcomes even when associated with airway malformations or structural heart diseases. Lung abnormality and longer cardiopulmonary bypass time as a surrogate marker of complex surgery, are possible risk factors.

Herein, the case of an infant initially diagnosed with left pulmonary artery sling, ventricular septal defect and a small atrial septal defect is described. Further diagnostic work-up revealed tracheobronchial anomalies and the presence of an anomalous origin of the left coronary artery from the pulmonary artery. The staged surgical management is described.

A fistula between the right pulmonary artery and the left atrium is a very rare cause of central cyanosis. A clinical diagnosis is often made by first excluding other common causes and then performing diagnostic catheterization. Surgical closure offers a definitive cure for this rare congenital anomaly., (© The Author 2016. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

We report three cases of an abnormal finding of duplicated left pulmonary artery: two of these occurring in children with Kabuki syndrome and configuring the setting of a pseudo-pulmonary sling without any clinical or cardiac cross-sectional evidence of tracheal compression. The other case instead represents duplicated left pulmonary artery with pulmonary sling caused by the retro-tracheal course of the lower left pulmonary artery associated with "Christmas Tree" arrangement of the tracheo-bronchial system. In both patients with pseudo-pulmonary sling and Kabuki syndrome, the abnormal finding was incidental during echocardiographic examination and neither of the patients required surgical repair for the condition. To the best of our knowledge, they represent the third and fourth cases in which such an anomaly of the pulmonary artery branches not forming a sling is seen in association with Kabuki syndrome. Another case represents our second experience and the second case reported in literature with duplicated left pulmonary artery in the setting of a complex tracheal anatomy. In this symptomatic patient, surgical repair of atrial septal defect and relief of the vascular ring were indicated, and the surgical repair was performed successfully at the age of 3 years.

Pulmonary artery slings are rare congenital anomalies seen only on the left side. The presence of right pulmonary artery (RPA) sling is extremely rare. We report a case of a RPA sling with a single lung associated with tracheal stenosis and its management., (© 2013 Wiley Periodicals, Inc.)

Combined tracheobronchial and thoracic vascular injury in children following blunt trauma to the chest is potentially life-threatening and almost certain to be fatal unless managed promptly. We report one such incident where prompt identification and early aggressive surgical management prevented an almost certain fatal outcome in a 5-year-old girl with complete disruption of the right main bronchus just distal to the carina, and a tear in the right pulmonary artery.

Idiopathic pulmonary artery aneurysms are rare and need a multidisciplinary approach to diagnosis and treatment. Surgery is the treatment of choice, especially when the aneurysms are large and when they are associated with pulmonary regurgitation. This report highlights a case in which successful surgical repair preserved the native pulmonary valve after pulmonary artery reconstruction., (Copyright © 2010 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)