Procedure

Pulmonary artery sling with tracheal stenosis

Viktor Hraka^*, Joachim Photiadis, Christoph Haun, Ehrenfried Schindler, Martin Schneider, Peter Murin and Boulos Asfour

Department of Pediatric Cardiac Surgery, German Pediatric Heart Centre, Asklepios Clinic Sankt Augustin, Arnold Janssen Str. 29, 53757 Sankt Augustin, Germany

^* Corresponding author: Tel.: +49 2241-249 603; fax: +49 2241-249 602 v.hraska{at}asklepios.com

Pulmonary artery sling (PAS) is a rare congenital heart disease in which the left pulmonary artery (LPA) originates from the right pulmonary artery (RPA) and encircles the distal trachea and right mainstem bronchus as it courses between the trachea and esophagus. Typically, patients with PAS have some respiratory symptoms, either due to external tracheal compression that can be corrected by relief of the sling mechanism, or due to severe diffuse tracheal stenosis with complete rings (ring-sling complex). The diagnosis of PAS is optimally made by echocardiography, while bronchoscopy is the key to the assessment of tracheal stenosis. Diagnosis is indication for surgery. Repair using a strategy of median sternotomy, cardiopulmonary bypass, division of the LPA and reimplantation into the main pulmonary artery (MPA), and simultaneous tracheal repair takes preference. Tracheal repair should be considered only in clinically symptomatic patients. The techniques of free tracheal autograft plasty or slide tracheoplasty offer promising results, and the choice of tracheal reconstruction should be guided by the clinical experience of the surgeon. Coexisting intracardiac pathologies are repaired at the same time. Postoperative care requires close multidisciplinary effort to achieve the best long-term result.

Key Words: Congenital heart disease • Pulmonary artery sling • Ring-sling complex • Tracheal stenosis • Vascular ring