Introduction
Pulmonary artery sling with complete tracheal rings is a rare but severe congenital anomaly that often presents significant surgical challenges, particularly in premature infants. Reference Sezer, Acar, Ekiz, Kaya, Bornaun and Aslan1,Reference K. Rahmath and Durward2 In this anomaly, the left pulmonary artery originates abnormally from the right pulmonary artery and courses between the trachea and oesophagus, causing compression of the distal trachea. When combined with complete tracheal rings, which cause intrinsic tracheal stenosis, the airway compromise can be severe. Reference Speggiorin, Torre, Roebuck, Mclaren and Elliott3 The combination of pulmonary artery sling, complete tracheal rings, ventricular septal defect, and patent ductus arteriosus in a low birth weight premature infant presents an exceptionally complex case requiring careful surgical planning and execution. Historically, management approaches have often advocated for staged repairs or waiting for weight gain, particularly in premature infants. Reference Kanavitoon, Kou and Rutter4,Reference Backer, Mavroudis, Gerber and Holinger5 However, this approach may prolong exposure to abnormal physiology and increase cumulative surgical risk. We present a case of successful single-stage complete repair of pulmonary artery sling with complete tracheal rings, ventricular septal defect, and patent ductus arteriosus in a premature infant weighing only 2.7 kg, demonstrating that body weight alone should not be an absolute contraindication for surgical intervention.
Case presentation
A male premature infant born at 34 weeks gestation with a birth weight of 1820 g was diagnosed prenatally with cardiac abnormalities and required continuous hospitalisation from birth. Initial echocardiography and cardiac computed tomography angiography revealed pulmonary artery sling, perimembranous ventricular septal defect (5.5 mm), patent ductus arteriosus, and patent foramen ovale (Figure 1A–C). After a multidisciplinary discussion, cardiac surgery was postponed until the infant achieved adequate weight gain. At one month of age, however, the patient required endotracheal intubation and mechanical ventilation due to respiratory distress. Bronchoscopy revealed severe tracheal stenosis 3 cm from the subglottic area, with complete tracheal rings and a 3.1 mm external diameter, preventing distal examination. Although quickly weaned from invasive ventilation within days, he remained dependent on non-invasive ventilation support with recurring episodes of desaturation, making hospital discharge impossible while awaiting growth and development.
Preoperative imaging and postoperative bronchoscopy findings in a premature infant with pulmonary artery sling and complete tracheal rings. ( a , b ) CT images demonstrating compression of the trachea (black arrows) by the anomalous left pulmonary artery. ( c ) Three-dimensional CT reconstruction showing the pulmonary artery sling configuration with the anomalous left pulmonary artery (red arrow) originating from the right pulmonary artery. ( d ) Postoperative bronchoscopic examination after slide tracheoplasty showing patent trachea and bilateral bronchi with adequate airway calibre.
Despite the high surgical risk due to low body weight and complex anatomy, we decided to proceed with surgical correction at 2.7 kg body weight. Two surgical strategies were considered. The first option was single-stage complete repair, including ventricular septal defect closure, slide tracheoplasty, and patent ductus arteriosus ligation. The second option was a staged approach, performing only slide tracheoplasty and patent ductus arteriosus ligation initially, while deferring ventricular septal defect closure until adequate weight gain was achieved. After multidisciplinary discussion and careful preoperative preparation, we ultimately decided to proceed with single-stage complete repair. Through a median sternotomy approach, cardiopulmonary bypass was established. The surgical procedure included pulmonary artery sling repair, slide tracheoplasty, ventricular septal defect closure, and patent ductus arteriosus ligation. The left pulmonary artery was detached from its anomalous origin, and the posterior wall was anastomosed to the main pulmonary artery, with autologous pericardium used for anterior wall augmentation. The stenotic tracheal segment was addressed with slide tracheoplasty using interrupted everted 6-0 PDS sutures throughout the anastomosis, while minimising dissection around the trachea to prevent ischemic complications. Adequate patency was confirmed by intraoperative bronchoscopy, showing a well-aligned anastomosis with properly everted mucosal edges, without evidence of narrowing or distortion (Figure 1d).
The patient was successfully weaned from cardiopulmonary bypass and had an uneventful recovery in the intensive care unit. The head was maintained in a flexed position for 72 hours postoperatively to minimise tension on the tracheal anastomosis. Bronchoscopy was performed on postoperative days 2 and 3 to assess anastomotic healing, evaluate for any evidence of oedema or granulation tissue, and ensure adequate airway patency before proceeding with extubation. The patient was extubated on postoperative day 5 and discharged home on postoperative day 21. At the 6-month follow-up, he showed normal growth and development with no respiratory symptoms. Bronchoscopy demonstrated a patent, growing trachea without evidence of restenosis. Echocardiography revealed no left pulmonary artery stenosis. The patient is currently 10 months post-operation and continues to do well under ongoing surveillance.
Discussion
This case highlights several important points that merit further discussion. First and foremost, the successful complete repair in a low-weight premature infant demonstrates that body weight alone should not be an absolute contraindication for surgical intervention. Our case shows that earlier intervention may be necessary and feasible when patients demonstrate significant respiratory compromise due to tracheal stenosis, when conservative management fails to maintain adequate oxygenation, or when there is evidence of progressive cardiac dysfunction. The key to success in such challenging cases lies in the combination of meticulous surgical technique, precise cardiopulmonary bypass management, and careful perioperative care optimisation.
The timing of surgical intervention requires careful consideration of multiple interconnected factors. From a respiratory perspective, the severity and progression of tracheal stenosis, the degree of ventilator dependence, and the risk of prolonged intubation complications must be weighed carefully. Cardiac considerations include the presence and degree of pulmonary hypertension, ventricular function, and the significance of left-to-right shunting. Additionally, the patient’s growth trajectory, nutritional status, and overall developmental progress play crucial roles in decision-making. Reference Kanavitoon, Kou and Rutter4
The choice between single-stage complete repair versus staged approach warrants detailed discussion. While staged repair theoretically offers the advantages of reduced surgical trauma and shorter cardiopulmonary bypass time, several factors in our case favoured single-stage repair. First, because the patient had a large ventricular septal defect requiring surgical closure, cardioplegic arrest was mandatory regardless of the surgical approach chosen, as cardiopulmonary bypass alone without aortic cross-clamping would result in air entrainment into the left ventricle with catastrophic consequences. Given that cardioplegic arrest was necessary, adding ventricular septal defect closure to the procedure would not significantly prolong the overall operative time. Second, leaving a large residual ventricular septal defect would perpetuate haemodynamic abnormalities and maintain the heart failure state, potentially impeding weight gain and nutritional improvement that are essential for subsequent surgery. If staged repair were chosen, pulmonary artery banding would typically be required to manage the haemodynamics of the ventricular septal defect. Third, single-stage repair avoids repeat sternotomy, thereby reducing cumulative surgical trauma, anaesthetic exposure, and family burden. Finally, at 2.7 kg body weight, advances in myocardial protection and cardiopulmonary bypass techniques have made cardiac surgery relatively safe in this weight range. We acknowledge that single-stage repair carries potential disadvantages, including possibly longer cardiopulmonary bypass duration, increased risk of conduction system injury in small hearts, higher infection risk, potentially longer ICU stay, and greater technical demands on the surgical team. After weighing these considerations, we determined that the benefits of immediate complete correction outweighed the risks of prolonged exposure to abnormal cardiopulmonary physiology.
The value of a multidisciplinary approach proved instrumental in achieving a successful outcome in this complex case. The collaborative expertise of paediatric cardiac surgeons, otolaryngologists, anaesthesiologists, neonatal intensivists, respiratory therapists, and cardiologists enabled comprehensive preoperative evaluation, optimal intervention timing, coordinated intraoperative management, and seamless postoperative care. The otolaryngology team’s intraoperative bronchoscopy was particularly crucial for precise localisation of the first complete tracheal ring, guiding the extent of resection, and ensuring adequate airway clearance post-repair. This team approach was particularly crucial for early recognition and management of potential complications.
The use of interrupted everted sutures in tracheal anastomosis offers several significant advantages. The technique enables precise control of tension at each suture point, with everted suture placement ensuring optimal mucosal edge approximation while preventing inward folding that could compromise the airway lumen. This even distribution of anastomotic tension prevents localised areas of excessive stress that could lead to scarring and stenosis. This is particularly crucial in paediatric airways, especially in premature infants, where the potential for growth must be carefully considered. The interrupted everted suture pattern, combined with the gradual absorption profile of PDS material, provides the necessary flexibility for tracheal growth and development while preventing long-term foreign body reaction that could impair natural tissue expansion. Additionally, the controlled absorption characteristics and minimal tissue reactivity of PDS help minimise excessive granulation tissue formation at the anastomotic site, thereby reducing the risk of airway narrowing. Reference Mckeown, Tsuboi, Togo, Thomas, Tuck and Gordon6 The technical precision afforded by interrupted everted sutures also allows surgeons to immediately correct any alignment discrepancies during the procedure, facilitating optimal mucosa-to-mucosa approximation that is essential for primary healing and the prevention of stricture formation.
Postoperative airway management and extubation timing represent critical components of successful outcomes following slide tracheoplasty. Our institutional protocol emphasises a conservative approach to postoperative care, maintaining the head in a flexed position for 48 to 72 hours to minimise tension on the tracheal anastomosis. Bronchoscopy was performed on postoperative days 2 and 3 to assess anastomotic healing, evaluate for oedema or granulation tissue formation, and ensure adequate airway patency. The optimal timing of extubation after slide tracheoplasty remains a subject of ongoing debate among paediatric airway centres. Some institutions advocate for early extubation within 24–48 hours, citing potential benefits including reduced risk of ventilator-associated pneumonia, decreased sedation requirements, and earlier assessment of native airway function. Manning and colleagues reported that over 50% of their patients could be extubated within 48 hours following slide tracheoplasty, particularly those without preoperative mechanical ventilation or prolonged cardiopulmonary bypass times. Reference Manning, Rutter and Border7 However, early extubation carries inherent risks, including potential reintubation in an inflamed surgical field, possible disruption of the fresh anastomosis, and inadequate time for resolution of postoperative oedema. In contrast, a more conservative approach with delayed extubation allows sufficient time for anastomotic healing, resolution of surgical oedema, establishment of haemodynamic stability, and comprehensive bronchoscopic evaluation before removing ventilatory support. We do not advocate for early extubation in complex cases involving simultaneous cardiac repair and slide tracheoplasty, particularly in low-weight infants.
Our experience contributes to the evolving understanding of managing complex congenital cardiovascular and airway anomalies in premature infants. While historical approaches often advocated for staged repairs or waiting for weight gain, our case suggests that, in carefully selected patients, primary complete repair may offer distinct advantages. This approach avoids prolonged exposure to abnormal physiology, reduces cumulative surgical risk from multiple procedures, and potentially improves long-term outcomes through earlier normalisation of anatomy. Moreover, addressing both cardiac and airway issues simultaneously may facilitate better growth and development.
However, several important caveats must be considered when contemplating such an aggressive surgical approach in premature, low-weight infants. The surgical team must have extensive experience in neonatal cardiac surgery, and the institutional infrastructure must be capable of supporting complex postoperative care. Patient selection must be extremely careful, and long-term follow-up is essential to evaluate outcomes. The importance of continuous bronchoscopic evaluation and monitoring during the perioperative period cannot be overstated. In our case, the ability to perform intraoperative bronchoscopy proved crucial for confirming adequate tracheal repair, assessing mucosal integrity, guiding optimal positioning of the endotracheal tube, and planning postoperative airway management.
Conclusion
This case demonstrates that with appropriate expertise, infrastructure, and careful patient selection, successful complete repair of complex cardiac and airway anomalies is achievable even in premature, low-weight infants. The experience gained from this case adds to our understanding of managing these challenging patients and may help guide future decision-making in similar cases.
Data availability
No data were generated or analysed for or in support of this paper.
Acknowledgements
We thank our multidisciplinary team members for their contributions to patient care.
Financial support
This research received no specific grant from any funding agency, commercial, or not-for-profit sectors.
Competing interests
The authors have no conflict of interests.
Ethical standard
The patient’s guardians provided informed consent for the publication of this case report.
