Left Pulmonary Artery Sling Research Articles

Fetal diagnosis and management of pulmonary artery sling: Acase series.

Pulmonary artery sling is a rare congenital anomaly accounting for 2% of all patients with vascular anomalies that cause airway obstruction. In the normal heart, the left (LPA) and right (RPA) pulmonary arteries arise in the intrapericardial space. However, in the pulmonary artery sling, the LPA trunk arises in the extrapericardial space from the posterior aspect of the mid RPA and courses posterior to the trachea causing tracheal compression and, at times, bronchial compression. While a full spectrum of congenital cardiac pathology can be identified before birth, only a few case reports document the prenatal diagnosis of an Left pulmonary artery sling (LPAS). We retrospectively identified all cases of prenatal LPAS from three Canadian fetal cardiology centers (2015-2022). Using the 3-vessel-tracheal view via fetal echocardiography (FE), four fetuses from three pregnancies demonstrated abnormal origin of the LPA from RPA and echogenic trachea. In one of two affected monochorionic twins coronal imaging demonstrated a significant narrowing of the large airways consistent with significant airway obstruction. Prenatal detection of LPAS by FE is possible and should prompt an evaluation for airway obstruction in the coronal view. Investigating associated lesions and genetic testing are recommended for informed shared decision making.

Left pulmonary artery sling with right upper lobe pulmonary artery arising from left pulmonary artery with tetralogy of Fallot.

Left pulmonary artery sling with right upper lobe pulmonary artery arising from left pulmonary artery with tetralogy of Fallot.

Prenatal diagnosis of right lung agenesis combined with left pulmonary artery sling

Prenatal diagnosis of right lung agenesis combined with left pulmonary artery sling

Anesthetic challenges during repairing left pulmonary artery sling without cardiopulmonary bypass: a case report

Left pulmonary artery sling (LPAS) is a rare vascular anomaly. The general surgical technique to correct it is through median sternotomy under cardiopulmonary bypass (CPB). In this case, we discuss the anesthetic management of left pulmonary artery (LPA) reimplantation without CPB to improve the understanding and provide an overview for practitioners in managing patients with this rare vascular anomaly. Patient was a 10 months old baby, with main complaints of shortness of breath since he was 4 months old, and diagnosed with LPAS from cardiac multi-slice CT scan (MSCT) examination. Intraoperatively, when the LPA was clamped, the blood pressure decreased due to decreased preload, and the end-tidal CO2 increased to 70 mmHg due to increased dead space, which was successfully managed. Postoperatively, the patient suffered from ventilator acquired pneumonia (VAP). Evaluating and assessing the potential complications that can occur during the perioperative period will help prepare for management and improve the success of anesthetic management.
 Abbreviations: CPB- Cardiopulmonary Bypass; ETT- Endotracheal Tube; LPA- Left Pulmonary Artery; LPAS- Left Pulmonary Artery Sling; MSCT- Multi-Slice CT Scan; RPA- Right Pulmonary Artery; VAP- Ventilator Acquired Pneumonia
 Key words: Left Pulmonary Artery; Anesthesia Management; Pulmonary Sling; Cardiopulmonary Bypass
 Citation: Arifin A, Perdana A. Anesthetic challenges during repairing left pulmonary artery sling without cardiopulmonary bypass: a case report. Anaesth. pain intensive care 2023;27(6):776−779; DOI: 10.35975/apic.v27i6.2352
 Received: August 10, 2023; Reviewed: October 16, 2023; Accepted: October 30, 2023

Left pulmonary artery sling: why is virtual CT bronchoscopy important? Case report

BackgroundMany causes of vascular rings may present as stridor among children. Co-occurrence of tracheal anomaly in left pulmonary artery sling (LPAS) is very common and needs special attention.Case presentationWe report a case of an infant who presented with expiratory stridor and wheezing. A preliminary chest x-ray revealed no significant findings. CT and virtual bronchoscopy confirmed the diagnosis of LPAS with associated distal tracheal stenosis. He underwent surgical correction with good results in the follow-up period.ConclusionsVirtual bronchoscopy may help to decide the type of operation and to avoid a potentially invasive bronchoscopy in LPAS.

Incidence of congenital tracheal stenosis in left pulmonary artery sling diagnosed by bronchoscopy.

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. 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. 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). CTS might be universally associated with LPAS. The circular tracheal cross-section on CT might imply the existence of a complete tracheal ring.

Surgical management of congenital tracheal stenosis associated with complex cardiovascular anomalies.

Although surgical outcomes have improved in patients with congenital tracheal stenosis (CTS), the management of such patients with complex cardiovascular anomalies (CVAs) remains a challenge. This study aimed to clarify the clinical features of this combination. Medical records of 14 patients with complex CVAs who had undergone slide tracheoplasty for CTS between May 2016 and February 2022 were retrospectively reviewed. Complex CVAs were defined as CVAs without left pulmonary artery sling and simple cardiac shunts. Data collected included age and body weight at tracheal reconstruction, preoperative respiratory support, surgery details, and mortality. The median age and body weight at tracheal reconstruction were 3.5 (range, 1-17)months and 4.7 (range, 2.3-8.2)kg, respectively. Simultaneous repair of CTS and CVAs was performed in eight patients and staged repair in six patients. Patients who underwent simultaneous repair required preoperative respiratory support more frequently than those who underwent staged repair (8/8 [100%] vs 2/6 [33.3%]; P = 0.015). No mortality occurred. Patients with complex CVAs often require tracheal reconstruction in early infancy. Surgical management strategy for this combination was generally decided on the basis of the severity of respiratory symptoms due to CTS.

Successful surgical treatment of congenital tracheal stenosis combined with tracheal bronchus and left pulmonary artery sling: a 10-year single-institution experience.

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. 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. The mean age at the operation and body weight were 8.0 ± 4.4months old and 6.5 ± 0.8kg, respectively. The mean tracheal diameter and length of the stenotic lesion were 3.2 ± 1.0mm (mean stenosis rate 46.2%) and 25.4 ± 4.9mm, 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 ± 32.1days. There were no major intraoperative or postoperative complications, including hypoxic-ischemic encephalopathy. The mean hospital stay was 92.2 ± 73.4days. All patients were discharged home without tracheostomy or oxygen support. Our slide tracheoplasty technique for CTS with TB and PA sling achieved excellent outcomes. Level IV.

Computed tomography and magnetic resonance imaging of vascular rings and other things: a pictorial review.

Compression of the airway, esophagus or both by aortic and pulmonary vessels can be caused by a variety of anatomical situations. Vascular rings are the most commonly encountered entity; however, compression can also occur from less common anomalies such as a left pulmonary artery sling or innominate artery compression. Vascular rings and other vascular compression abnormalities can be challenging to visualize and image and often require advanced imaging by CT or MRI to better understand the cause and extent of compression. Atretic vascular structures, such as the ligamentum arteriosum or atretic arch, play a key role in creating a vascular ring and do not enhance with contrast agent in a typical fashion. Despite these imaging challenges, classic and useful signs can indicate the presence or absence of a vascular ring or compression.

Left pulmonary artery sling in an adult

Left pulmonary artery sling in an adult

Left Pulmonary Artery Sling: Report of Five Cases on Multidetector Computed Tomography From Vietnamese Children

Left Pulmonary Artery Sling: Report of Five Cases on Multidetector Computed Tomography From Vietnamese Children

Left Pulmonary Artery Sling: Postoperative Outcomes for Patients at a Single Center.

Left pulmonary artery (LPA) sling is a rare anomaly characterized by the origin of the LPA from the right pulmonary artery with a course between the trachea and esophagus. It is often associated with airway and cardiac anomalies. This is a retrospective case series of consecutive patients who underwent LPA sling repair (LPASR) at a tertiary care center over a 35-year period with a focus on tracheal and/or LPA reinterventions and survival. Between June 1983 and July 2018, 42 patients were identified: isolated LPASR was performed in 16 (38%), LPASR/intracardiac repair in 10 (24%), and LPASR/tracheal repair in 16 (38%). There were 5 (12%) in-hospital and 4 (10%) late deaths. Survival rates (15 years) were as follows: 100% (isolated LPASR), 65% (concomitant intracardiac repair), and 52% (concomitant tracheal surgery). Preoperative intensive care unit (ICU) hospitalization was associated with future intervention on the LPA/trachea (61%, 11/18). The median distribution of blood flow to the left lung post-index surgery was 38%. Freedom from isolated LPA intervention was 100% after isolated LPASR, 93% after LPASR/tracheal surgery, and 69% after LPASR/intracardiac repair. Freedom from isolated tracheal intervention was 92% after isolated LPASR, 73% after LPASR/tracheal surgery, and 78% after LPASR/intracardiac repair. ICU hospitalization prior to index surgery may indicate the severity of associated cardiac/tracheal abnormalities as this is associated with increased morbidity and mortality. Patients who underwent LPASR/intracardiac surgery were more likely to undergo isolated LPA intervention and those who underwent LPASR/tracheal surgery were more likely to undergo isolated tracheal intervention.

Stridor as a Harbinger of Congenital Cardiovascular Anomaly.

There is an intimate spatial relationship between the cardiovascular and airway structures. Central airway compression related to congenital cardiovascular anomalies should be considered in neonates, infants, and young children presenting with stridor. From July 31, 1990 to December 31, 2018, 24 pediatric patients, including 18 males and 6 females, aged 1 day to 11.3 years old, presenting with stridor and/or lip cyanosis were enrolled in this study. At presentation, none of the patients had a known history of congenital heart disease. Patients with congenital bronchopulmonary vascular/foregut malformations, congenital pulmonary venolobar syndrome, congenital pulmonary malinosculations, Eisenmenger syndrome, secondary pulmonary hypertension, and idiopathic pulmonary arterial hypertension were excluded from this study. Available profiles of 24 patients were reviewed and the underlying congenital cardiovascular anomalies contributing to the clinical manifestation of stridor were analyzed, including chart recordings, chest radiograms, echocardiograms, computerized tomography, electrocardiograms, esophagograms, cardiac catheterization, magnetic resonance imaging, and bronchography. Stridor was an early sign of congenital cardiovascular anomalies, including double aortic arch, right aortic arch (RAA) with Kommerell diverticulum, mirror-image right aortic arch with aortic diverticulum, anomalous right innominate artery, left pulmonary artery sling, RAA with tetralogy of Fallot and persistent fifth aortic arch, a vertical patent ductus arteriosus from a transverse left aortic arch, and absent pulmonary valve syndrome. Notably, chest radiography provided the first clue of RAA in 18 of the 24 patients. Stridor can be a harbinger of congenital cardiovascular anomalies causing central airway compression in pediatric patients.

A modified technique for single-stage repair of complete tracheal rings and pulmonary artery sling in a symptomatic newborn.

Left pulmonary artery sling (PAS) is a rare condition characterized by the left pulmonary artery (LPA) originating from the right pulmonary artery and coursing behind the trachea to the lung. PAS is associated with complete tracheal rings, a pair of findings termed “ring-sling complex.”1 We describe our modified technique for complete one-stage repair of this entity in an infant with associated tracheal diverticulum (Video 1). Institutional review board approval was not required for this single case report, given that the parents provided informed written consent for the publication of the study data.

Left pulmonary artery sling and COVID-19: David against Goliath

Left pulmonary artery sling and COVID-19: David against Goliath

Relationship between a Tracheal and Left Pulmonary Artery Stenosis Index and the Prognosis of Pulmonary Artery Sling with Tracheal Stenosis.

Pulmonary artery sling is a rare congenital pulmonary vascular malformation, often associated with tracheal or bronchial stenosis. Surgical treatment of pulmonary artery sling with tracheal stenosis (T) has a high risk of death and a relatively poor prognosis. This study explored the relationship between the T and left pulmonary artery stenosis (P) ratio and the effects of surgery for pulmonary artery sling with tracheal stenosis. Patients undergoing surgery for pulmonary artery sling in our center from January 2010 to December 2018 were retrospectively analyzed. Routine cardiac-enhanced computed tomography (CT) was performed preoperatively, and the P and T diameters were measured on the CT image. The T/P ratio was then calculated and analyzed. Thirty cases of pulmonary artery sling combined with tracheal stenosis were operated under cardiopulmonary bypass. The mean age at operation was 13.8 ± 13.6months (1.2-57.1months, Q1-Q3: 5-17months), and the mean body weight was 8.8 ± 3.5kg (3.8-17.3kg, Q1-Q3: 5.8-11.5kg). Twenty-three patients survived, and seven died, with an overall survival rate of 76.7%. Twenty-four patients underwent left pulmonary artery re-implantation, six patients underwent simultaneous left pulmonary artery re-implantation and slide tracheoplasty, and patients with intracardiac malformations (ventricular septal defect (n = 6) and atrial septal defect (n = 4)) underwent concurrent repair of the intracardiac defect. All cases had different degrees of tracheal stenosis, and the most narrowed trachea occurred with compression by the left pulmonary artery sling. The T/P ratio in the tracheoplasty group was significantly higher than that in the non-tracheoplasty group. The mortality rate in the T/P ≤ 1.15 group was significantly lower than that in the T/P > 1.15 group. Pulmonary artery sling treatment has a high risk of death. Left pulmonary artery re-implantation is an effective and safe surgical method for treating pulmonary artery sling, and slide tracheoplasty is an effective surgical method to correct tracheal stenosis. Children with T > 78.4% should receive active intervention for the tracheal stenosis, and these children have a considerable postoperative survival rate. The T/P ratio can be used to compare the relative superiority of the two compressions. A T/P ratio > 1.15 can be used as a reference index for intervention in tracheal stenosis and is a risk factor for postoperative death.

Aortotracheal fistula after slide tracheoplasty in a patient with dextrocardia, left pulmonary artery sling and tracheal stenosis: a case report

BackgroundAortotracheal fistula (ATF) is an uncommon and fatal complication of tracheal or aortic surgery, especially among pediatric patients.Case presentationWe reported a case in a 1-year-old boy with dextrocardia, left pulmonary artery sling and long segment tracheal stenosis. He received slide tracheoplasty at 9 months of age and had post-operative refractory granulation at distal trachea status post repeated balloon dilatation and laser vaporization. Episodes of hemoptysis occurred on post-operative day 81. Bronchoscopy revealed a pulsating pseudoaneurysm at lower trachea which ruptured during the procedureUrgent surgical repair under cardiopulmonary bypass with deep hypothermic circulatory arrest was done. No recurrent bleeding or significant neurologic deficits noticed at a 4-month follow-up.ConclusionCongenital anomaly that changes the spatial relationship between trachea and aorta could have contributed to formation of ATF. This warrant future attention when managing tracheal granulation with this not uncommon anatomy.

A case of timely diagnosis and successful surgical correction of the left pulmonary artery sling in a newborn

The article describes a clinical observation of a newborn patient with a rare congenital cardiovascular disorder of the left pulmonary artery sling. There are few publications in the world’s literature on the clinical observations of the above pathology, which causes the key difficulty in diagnostics. Moreover, this case features a favorable outcome thanks to a timely diagnosis and a successful surgical correction that followed.

Influence of Fetal Diagnosis on Management of Vascular Rings

Fetal diagnoses of vascular rings have been increasing. We compared management strategies and outcomes of infants with fetal diagnosis with those with postnatal diagnosis to inform recommendations regarding optimal management. A retrospective review was performed of vascular ring operations from January 2000 to June 2019. Standard demographic data (preoperative clinical status, timing of diagnosis, cross-sectional imaging, operative and perioperative details, and clinical outcomes) were collected. Statistical analysis was performed to compare characteristics and outcomes of fetal versus postnatal diagnosis. Of 190 patients, 15% (n= 29) were diagnosed prenatally. Anatomic variants were double aortic arch (n= 66, 14 fetal diagnoses), right aortic arch, aberrant left subclavian artery (n= 94, 12 fetal diagnoses), circumflex aorta (n= 7, 1 fetal diagnosis), and pulmonary artery sling (n= 19, 2 fetal diagnoses). An increasing frequency of fetal diagnoses has been noted in the past 10 years. In 2012 1 of 9 patients (11%) had a fetal diagnosis, whereas in 2018 8 of 11 (72%) had a fetal diagnosis (P < .001). Patients with a fetal diagnosis were significantly younger at the time of surgery (13.1 months [interquartile range (IQR), 20.6] vs 24.0 months [IQR, 87.0], P= .029). There was no difference in postoperative complications or length of stay (3 days [IQR, 1] for fetal diagnoses vs 4 days [IQR, 3] for postnatal diagnoses, P= .50). Fetal diagnosis leads to the potential for expectant management of vascular ring patients. This has resulted in earlier time of intervention with no increase in postoperative morbidity. This may lead to improved long-term outcomes and potentially alter the natural history for these children.

Pulmonary sling in a patient with common arterial trunk

We report a rare association of common arterial trunk with left pulmonary artery sling and highlight the importance of cross-sectional imaging in complex congenital cardiac lesions. The patient was antenatally diagnosed with common arterial trunk and underwent surgical repair in the neonatal period. At the age of 20 months, the patient presented with respiratory symptoms and increased right ventricular pressure. Multislice computed tomography demonstrated a pulmonary sling with compression of the distal trachea. Surgical correction of the pulmonary sling and change of the right ventricular to pulmonary artery conduit to a bigger size was performed.