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BACKGROUND: To examine the safety and effectiveness of proactive tracheoplasty for pediatric ring-sling complexes. METHODS: We retrospectively collected the data from 304 children who were diagnosed with a ring-sling complex and underwent surgery at three cardiac centers in China between January 2010 and June 2023. Children were categorized into three surgical groups: concurrent sling and tracheal surgery (Group A, n=258), staged sling and tracheal surgery (Group B, n=25), and sling-only surgery (Group C, n=21). We compared perioperative clinical characteristics, tracheal morphology changes, and outcomes across the groups. RESULTS: The median age of the children was 1.2 (IQR: 0.7-1.9) years. The anomalous tracheobronchial arborization rates were higher in Groups A (52.5%) and B (60.0%) than in Group C (15.0%). The preoperative narrow-wide ratio (NWR) was lower in Groups A and B than in Group C, with values of 0.44 (IQR: 0.35-0.52), 0.44 (0.33-0.59), and 0.68 (0.54-0.72), respectively (P<0.001). Preoperative subcarina angles were similar among the groups (P=0.54). After specific surgeries, the NWR and subcarina angle significantly improved in Groups A and B but not in Group C. There were seven in-hospital deaths and two post-discharge deaths. Respiratory symptoms improved in Groups A and B but seven children in Group C remained respiratory dysfunction. Six children presented with residual stenosis of the left pulmonary artery. CONCLUSION: Concurrent sling and tracheal surgeries for children with the ring-sling complex are safe and effective and are especially preferable for those with NWR ≤0.6, long-segment or diffuse tracheal stenosis, anomalous tracheobronchial arborization, and pronounced respiratory symptoms.

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Congenital vascular anomalies such as pulmonary artery sling and persistent left superior vena cava are rare vascular disorders. We describe a case of a patient who presented with pneumonia and was found to have a pulmonary artery sling as a potential cause of the pneumonia.

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INTRODUCTION AND IMPORTANCE: Left pulmonary artery sling is an uncommon condition observed in infants. The severity of the condition is determined by the compression of the broncho-tracheal tree induced by the ring sling compression. The main goal of the treatment is to adjust the left pulmonary artery and eventually relieving the compression through surgery. The long-term outcome associated with the complexity of the anomalies. CASE PRESENTATION: A nine-months old patient complained of worsening respiratory distress. The computed tomography scan revealed the potential presence of a left pulmonary artery sling and compression of the trachea, without any abnormalities in the trachea itself. Echocardiography study showed no intracardiac lesion. We successfully did left pulmonary artery transection and re-implantation to main pulmonary artery without cardiopulmonary bypass. CLINICAL DISCUSSION: Pulmonary artery sling commonly treated with reimplantation of the sling to its origin that usually required cardiopulmonary bypass machine. However, in our case we delivered it without the need of cardiopulmonary bypass. The outcome result turned excellent with echo post-operative showed confluent pulmonary arteries. CONCLUSION: The optimal approach to treating congenital pulmonary artery sling is through early surgical intervention in symptomatic patients. Following surgical repair devoid of tracheal lesion, the prognosis appears favorable, and routine follow-up is required to determine the long-term effects.

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During the fetal period, the pulmonary artery bifurcation revealed the absence of the left pulmonary artery. Instead, an anomalous artery originated from the right pulmonary artery, coursing posteriorly the trachea to the left lung. The diagnosis of PAS was established following prenatal ultrasound screening, which was subsequently confirmed by postnatal echocardiography and CT after delivery.

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PURPOSE: Congenital tracheal stenosis (CTS) has been reported to occur in 50-65% of cases of left pulmonary artery sling (LPAS), but the exact incidence rate is unknown. This study aimed to determine the actual rate using bronchoscopy and to elucidate morphological features in computed tomography (CT) diagnosis. METHODS: We performed a single institutional retrospective review of all patients with LPAS between January 2010 and March 2022. The percentage of complete tracheal rings in patients with LPAS was evaluated using bronchoscopy. The anteroposterior/lateral diameter ratios at the smallest and largest diameters of each CTS patient's trachea were measured on CT. The Wilcoxon signed-rank test was used to analyze the differences between the two parts. RESULTS: Fifty-two patients with LPAS were enrolled. All patients had complete tracheal rings on bronchoscopy. CT analysis of 32 patients with CTS was performed. The median anteroposterior/lateral diameter ratio at the smallest diameter was 1.05 (interquartile range [IQR] 0.95-1.15); the median ratio at the largest diameter was 0.94 (IQR 0.89-0.99). There was a significant difference between the two parts (p = 0.013). CONCLUSION: CTS might be universally associated with LPAS. The circular tracheal cross-section on CT might imply the existence of a complete tracheal ring.

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Objectives: This study aims to investigate the efficacy of prenatal ultrasonography in diagnosing the anomalous origin of the fetal pulmonary artery (AOFPA). Methods: A total of 26 AOFPA cases were retrospectively analyzed from January 2014 to January 2023. The features of the AOFPA were characterized by comparing the prenatal ultrasonic data with the results of anatomical casting after pregnancy termination or postnatal imaging and surgical intervention. Missed diagnoses and misdiagnoses were expounded. Results: Of the 26 AOFPA cases, there were 13 cases of pulmonary artery sling, 8 cases of anomalous origin of the unilateral pulmonary artery, and five cases of unilateral absence of the pulmonary artery; 17 cases received pathological anatomy and casting after pregnancy termination, and nine cases were confirmed by postnatal imaging and surgery. Nineteen cases were accurately prenatally diagnosed (19/26, 73.1%), and seven cases were missed or misdiagnosed (7/26, 26.9%). Conclusions: Prenatal ultrasonography has a favorable diagnostic efficacy for anomalous origin of the fetal pulmonary artery. The absence of either the left or right pulmonary artery from the image of pulmonary artery bifurcation may indicate origin abnormalities of the pulmonary artery in fetuses, which signifies the necessity to detect the abnormal origin of the pulmonary artery on the affected side and other potential intracardiac malformation complications.

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BACKGROUND: Vascular rings, including right aortic arch with aberrant left subclavian artery (RAA-ALSCA), double aortic arch (DAA) and pulmonary artery sling (PAS), are congenital anomalies that may cause airway and oesophageal compression. As prenatal detection has improved, literature comparing clinical outcomes of antenatally versus postnatally diagnosed cases continues to emerge. The aim is to define a statewide tertiary paediatric institution's clinical profile and outcomes of prenatal versus postnatally diagnosed isolated vascular rings. METHOD: A retrospective single-centre review of isolated RAA-ALSCA, DAA and PAS between 1 January 1999 and 31 December 2020 was conducted. Clinical characteristics, surgical and follow-up information were collected. Antenatal and postnatally diagnosed groups were compared. RESULTS: Out of 123 cases diagnosed with isolated vascular rings, 98 (79.7%) cases had RAA-ALSCA, 21 (17.1%) with DAA, 4 (3.3%) with PAS. The antenatal detection rate was 73.6% in the past decade; 20.3% had a genetic disorder, of which 48% had 22q11.21 microdeletion. Of prenatally diagnosed cases, 31.3% developed symptoms, commonly stridor and dysphagia, at a median age of 2.0 months (IQR 0.0-3.0), compared to a median age of diagnosis for the postnatal cohort of 9 months (IQR 1.0-40.7). Postnatally diagnosed cases were more likely to present with symptoms, primarily respiratory distress, than prenatally diagnosed cases (p=0.006). Fifty-nine (59) cases (50% antenatally diagnosed) required vascular ring division; 6.8% had residual symptoms following surgery. DISCUSSION: Antenatal diagnosis has improved and leads to better parental awareness and more timely, appropriate intervention. Postnatally diagnosed patients were older, more likely to be symptomatic, underwent more investigations and were commenced on more medications for symptom management prior to diagnosis. One in five cases of isolated vascular ring anomalies carried a genetic diagnosis, which has important implications on prenatal counselling and genetic testing.

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Pulmonary artery sling is a rare childhood vascular tracheobronchial compression syndrome that is frequently associated with tracheal stenosis. Consequently, neonates or infants may present with critical airway obstruction if there is long segment airway narrowing and complete rings. Rapid diagnosis of this cardiac vascular malformation and evaluation of the extent and severity of airway involvement is essential to plan surgery, typically a slide tracheoplasty to relieve critical airway obstruction. Long term outcome can be excellent following surgical repair of the stenosed airway and reimplantation of the left pulmonary artery. In this review we focus on the embryology, diagnostic workup, airway investigations and management for this rare but challenging congenital condition.

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Background: Reimplantation of the left pulmonary artery (LPA) and slide tracheoplasty has been our standard approach of care for patients with pulmonary artery sling (PAS) and tracheal stenosis. We present our experience, with emphasis on tracheal arborization and hypoplastic lungs; and their impact on long-term outcome of children with PAS and tracheal stenosis. Methods: It is a retrospective comparative study. Data were classified and analyzed based on the type of tracheobronchial arborization and normal versus hypoplastic lungs. Results: Seventy-five children operated between January 1994 and December 2019 (67 with normal lungs and 8 with lung hypoplasia/agenesis) were included. Patients with hypoplastic lungs had higher rates of preoperative ventilation (87.5%), postoperative ventilation (10 vs 8 days, P = .621), and mortality (50% vs 9%, P = .009) compared with those with normal lungs. Nineteen patients had tracheal bronchus (TB) variety and 30 patients had congenital long-segment tracheal stenosis (CLSTS) variety of tracheobronchial arborization. Endoscopic intervention was needed in 47.4% of patients with TB type and 60% with CLSTS type. CLSTS patients had higher rates of preoperative ventilation (60% vs 47.4%, P = .386), longer periods of postoperative ventilation (13 vs 6.5 days, P = .006), and ICU stay (15 vs 11 days, P = .714) compared with TB type. Conclusion: Surgical repair of PAS with tracheal stenosis has good long-term outcomes. All variations of tracheal anatomy can be managed with slide tracheoplasty. Persistence of airway problems requires intervention during follow-up as tracheal stenosis continues to be the Achilles heel.

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Generally, pulmonary artery sling operation involves the pulmonary artery transplantation to be cut off. Nursing care is focused on the postoperative pulmonary vascular anastomosis, respiratory tract, and blood pressure after surgery. We report the case of an infant who underwent pulmonary artery tracheal transposition combined with Slide keratoplasty, where the pulmonary artery transplantation was not cut off. We highlight that postoperative pulmonary artery blood flow to the unobstructed airway and airway reconstruction surgery should be focused on to help children recover and ensure successful surgery. This case study reports the postoperative nursing experience of one patient with pulmonary artery sling undergoing pulmonary tracheal transposition combined with Slide arthroplasty. This demonstrates that throughout the postoperative care, airway management should be focused on to maintain circulation stability in the early postoperative period, and corresponding measures such as posture management, atomization inhalation, and improved chest physical therapy should be applied according to the special surgical method of the case in order to reduce airway complications and to improve the surgical success rate of children with pulmonary artery sling undergoing pulmonary tracheal transposition combined with Slide arthroplasty. In similar cases, after pulmonary tracheal transposition and Slide angioplasty, the doctors and nurses should pay attention to early circulation stability and focus on airway management through careful treatment and nursing, so as to promote the child's recovery.

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Pulmonary artery sling (PAS) is a rare congenital vascular anomaly, and is usually diagnosed during the infantile or fetal period. Adult presentation of PAS is rare. We report a 55-year-old woman with left pulmonary artery sling and left lung bronchiectasis, performing as persistent shortness of breath, coronary computed tomography angiography (CTA) showed the aberrant left pulmonary artery emerging from the right pulmonary artery and crossing to the left between the trachea and oesophagus. We experienced a rare adult case with LPAS and left bronchiectasis, stressing the importance of the anatomic abnormalities in such cases.

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OBJECTIVE: This study aimed to investigate surgical outcomes of pulmonary artery (PA) sling without tracheoplasty. METHODS: From 2001 through 2020, among 22 patients who underwent PA sling repair, all but 1 patient who underwent concomitant tracheal surgery were analyzed. The outcomes of interest were all-cause death, PA reintervention, tracheal intervention, and readmission for respiratory symptoms. Computed tomography was used to measure the narrowest tracheal diameter. RESULTS: The median age and weight at repair were 7.6 months and 7.7 kg, respectively. Most patients (20 out of 21, 95.2%) had preoperative respiratory symptoms. Associated airway anomalies included tracheal ring in 12 (57.1%), bridging bronchus in 8 (38.1%), and tracheal bronchus in 2 patients (9.5%). There was 1 in-hospital death (4.8%). The median ventilator time and intensive care unit stay were 23 hours and 3 days, respectively. There was neither late death nor tracheal intervention during follow-up. Five patients (25.0%) underwent reintervention for left PA stenosis. Hospital readmission for respiratory symptom was required in 7 patients and was associated with the narrowest preoperative tracheal diameter (P = .025) and cardiopulmonary bypass time (P = .040) in univariable analysis. The narrowest tracheal diameter of 3.4 mm was identified as a cutoff value for readmission for respiratory symptom. Freedom from readmission for respiratory symptom was 63.3% at 10 years. CONCLUSIONS: PA sling repair without tracheal surgery might be a reasonable surgical option with rare need for tracheal intervention. Hospital readmissions for respiratory symptoms are more frequently required in patients with smaller tracheal diameter and all readmissions were limited to within 2 years after repair.

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INTRODUCTION: Left pulmonary artery sling (LPAS) is a rare congenital vascular anomaly which can be isolated or associated with other malformations. Patients with LPAS usually have symptoms of airway obstruction. CASE PRESENTATION: We present a case of LPAS with double-outlet right ventricle diagnosed by fetal echocardiography at 33 weeks' gestation. Postnatal computed tomography echocardiography confirmed the diagnosis. Additional bronchial bridge and left main bronchial stenosis found by computed tomography led to a diagnosis of type II LPAS. CONCLUSION: It is crucial to trace pulmonary artery branches in the prenatal diagnosis. Prenatal diagnosis of LPAS will facilitate postnatal care and timely treatment.

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PURPOSE OF REVIEW: Vascular rings are congenital malformations resulting from abnormal development of the great vessels, with the consequent encircling and compression of the trachea, esophagus, or both. We conducted a review of the current literature to identify the different management strategies that can be implemented based on the prognosis of each of these anomalies. RECENT FINDINGS: Although most vascular rings occur in isolation, they can also be associated with other congenital cardiac and/or respiratory diseases; therefore, thorough investigation is necessary before definitive surgical repair. Clinical presentation varies from asymptomatic to severe, with both respiratory and digestive symptoms. Although early surgical results are acceptable, the long-term outcome is variable; therefore, there is still controversy regarding the appropriate timing of treatment. This is especially true with regard to the Kommerell diverticulum (KD) and in patients without symptoms at the time of initial surgical evaluation. As more sophisticated diagnostic tools have become available and more studies on adults affected by this condition have been published, understanding of this condition and its additional clinical implications has grown and appears to be tilting management toward earlier intervention.

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PURPOSE: Radical surgery for congenital tracheal stenosis (CTS) is technically demanding. CTS combined with tracheal bronchus (TB) and pulmonary artery (PA) sling is a particularly challenging condition. We herein report our successfully modified surgical techniques for CTS combined with TB and PA sling. METHODS: Nine patients treated at our institution from July 2010 to December 2020 for CTS with TB and PA sling were enrolled. The patients' characteristics, operative results, and clinical outcomes were reviewed and analyzed retrospectively. RESULTS: The mean age at the operation and body weight were 8.0 ± 4.4 months old and 6.5 ± 0.8 kg, respectively. The mean tracheal diameter and length of the stenotic lesion were 3.2 ± 1.0 mm (mean stenosis rate 46.2%) and 25.4 ± 4.9 mm, respectively. All cases were complicated with PA sling at bifurcation stenosis with tracheobronchomalacia. All patients underwent modified posterior-anterior slide tracheoplasty with an inverted Y-shaped incision at the bifurcation and repositioning of the PA. The mean postoperative intubation period was 25.0 ± 32.1 days. There were no major intraoperative or postoperative complications, including hypoxic-ischemic encephalopathy. The mean hospital stay was 92.2 ± 73.4 days. All patients were discharged home without tracheostomy or oxygen support. CONCLUSION: Our slide tracheoplasty technique for CTS with TB and PA sling achieved excellent outcomes. LEVEL OF EVIDENCE: Level IV.

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