Left Pulmonary Artery Sling Research Articles

Prenatal sonographic diagnosis of partial left pulmonary artery sling: A rare case report

Pulmonary artery sling is a rare congenital vascular anomaly. Partial anomalous left pulmonary artery is even rarer and no in utero observation has yet been reported. Here, we present the ultrasonographic findings of a 38-year-old woman at 32 weeks of gestation whose fetus showed a normal bifurcation of the pulmonary trunk into the right and left pulmonary arteries, but an anomalous origin of the left lower lobe pulmonary artery from the right pulmonary artery. These findings were confirmed by postnatal echocardiography and thoracic computed tomography.

Pulmonary Sling: verification of prenatal diagnosis after 5 years

Results of postnatal verification of the case of prenatal diagnosis of the left pulmonary artery sling and literature review are presented.

Clinical and structural aspects of tracheal stenosis and a novel embryological hypothesis of left pulmonary artery sling.

To identify the imaging features peculiar to congenital tracheal stenosis (CTS) complicated with left pulmonary artery sling (LPAS) with the aim of presenting a hypothesis of tracheal stenosis embryology in LPAS. We retrospectively reviewed CTS patients (with complete cartilaginous rings) admitted between April 2010 and July 2018. All the patients were classified into the LPAS or non-LPAS group, and their clinical characteristics and qualitative variables on computed tomography (CT) imaging were compared. Of the 72 patients enrolled, 61 had bilateral lungs. Among the bilateral lung patients, 26 (43%) had LPAS. The tracheal bifurcation was significantly deeper, the stenotic region was longer, and the bronchial angle (especially in the right) was wider, in the LPAS group. The cut-off values for the thoracic vertebral level at the tracheal bifurcation (>4.8), subcarinal angle (>118.1), and right bronchial angle (>61.9) were useful for diagnosing suspected cases of LPAS. In the time-dependent course, LPAS complicated with a congenital heart defect was a statistically significant risk factor of respiratory symptoms (hazard ratio, 3.01; 95% confidence interval, 1.23-7.37; P = .02). The CT findings described here should immediately raise suspicion of LPAS on chest X-ray and also suggest tracheal "squeezing and milking" by the surrounding vessels in the embryo. Patients with LPAS complicated with a heart defect should be followed carefully to determine the optimal timing of intervention.

Tracheal Stenosis Successfully Treated without Surgery in Preterm Infant

Congenital tracheal stenosis is a rare disease that can be life threatening, especially when early identification is missed. A premature twin showed progressive respiratory symptoms at birth and tracheal stenosis, with difficulty intubating. Chest computed tomography scan revealed diffuse tracheal stenosis and left pulmonary artery (LPA) sling. Tracheal ballooning under fluoroscopic guide was successfully performed at 5 days old and maintained with endotracheal intubation until 7 months old. The patient underwent surgery for LPA sling and is doing well without any respiratory symptoms following extubation. Herein, we report the first case of congenital tracheal stenosis in a preterm infant who was successfully treated with tracheal ballooning and conservative management in Korea.

The bridging bronchus: A comprehensive review of a rare, potentially life‐threatening congenital airway anomaly associated with cardiovascular defects

The bridging bronchus is a rare congenital airway anomaly in which the right upper lobe of the lung is supplied by the right main bronchus while the right lower lobe, and often the right middle lobe is supplied by an aberrant bronchus arising from the left main bronchus. The aberrant bronchus crosses (bridges) the lower part of the mediastinum, hence the term bridging bronchus (BB). This potentially life-threatening condition, usually accompanied by diffuse or focal airway stenosis, commonly presents with signs and symptoms related to large airway obstruction, such as respiratory distress, apnea, wheezing, stridor, and recurrent respiratory tract infections. Diagnosis of the BB is often challenging because the associated signs and symptoms mimic those of common conditions such as bacterial and viral bronchiolitis, bronchial asthma, cystic fibrosis, and foreign body aspiration. The BB is also often accompanied by congenital cardiovascular anomalies, including left pulmonary artery sling, atrial, and ventricular septal defects, tetralogy of Fallot, patent ductus arteriosus, and coarctation of the aorta. Patients presenting with the above signs and symptoms who are not responsive to standard treatment modalities, and have accompanying cardiovascular congenital anomalies should, therefore, be investigated for the BB. Herein, we review the anatomy, embryology, clinical presentation, differential diagnosis, imaging techniques and surgical management of the BB.

Prenatal three-dimensional ultrasound detection of left pulmonary artery sling

Left pulmonary artery (LPA) sling is a very rare congenital anomaly. This anomaly is often associated with tracheal narrowing caused by either intrinsic stenosis or malformation of tracheal rings. To the present authors’ knowledge, prenatal diagnosis of LPA sling has not been reported frequently, and three-dimensional ultrasound was not used in any of the previous cases. The aim of this article is to report one case of LPA sling, which was diagnosed in a fetus at 24 weeks gestation by routine echocardiography and four-dimensional spatio-temporal image correlation (STIC), with focus on the particularity of this rare anomaly prenatally diagnosed.

Combined Surgical and Endoscopic Approach for Ring-Sling Complex.

Left pulmonary artery (LPA) sling (PAS) is a vascular ring, which is frequently associated with long-segment tracheal stenosis (TS). Mortality rate in operated children is still high, especially in cases of severe tracheal hypoplasia and/or associated congenital heart defects (CHDs). We report our experience of treatment and follow-up in a pediatric cohort of patients affected by PAS with severe tracheobronchial involvement. From 2005 to 2017, we enrolled 11 children diagnosed with PAS and congenital TS requiring surgical intervention. Echocardiography, computed tomography, and bronchoscopy were performed in all patients. Associated CHD were present in 5 (45%) patients. Tracheal reconstruction techniques included slide tracheoplasty (7/11; 63%), slide tracheoplasty and costal cartilage graft (2/11; 18%), and Hazekamp technique (2/11; 18%).Nine patients underwent LPA direct reimplantation and concomitant tracheoplasty; concomitant surgical repair for CHD was performed in three children. Over a mean follow-up of 30 months (range: 3-75 months), a late mortality of 18% was registered; no early death occurred. Good flow through LPA could be documented in all patients. Ten children required operative bronchoscopies (mean: 16/patients) aimed at stent positioning/removal, treatment of granulomas, and tracheobronchial dilatation. Severe tracheobronchial stenosis and associated CHD were the main determinants for hospitalization time, intensive assistance, and repeated endoscopic procedures.Patients affected by PAS/TS complex require a careful management at high-specialized centers providing multidisciplinary team.Respiratory endoscopy may play a central role both in preoperatory assessment and in postoperative management of patients showing severe tracheobronchial involvement.

Left pulmonary artery sling with tracheal stenosis

We present the case of a 4-year-old girl, bronchofiberscopy (scope diameter 2.8mm): one third below oftrachea, which was divided into two orifices. The orifice on the right side was larger and divided into twosuborifices. The left orifice was stenosis and could not pass the bronchoscope. Whole trachea mucosa was redand edema, and could not observe the cartilage ring of trachea. Chest X-ray: infiltration of right lower lobe.Chest CT multiple probes with contrast injection confirmed that: At the sixth to seventh thoracic vertebral level,the left pulmonary artery was arisen from the right pulmonary artery then passed in between the behindtrachea and anterior oesophagus to reach the left lung hilar. And at the fourth to fifth thoracic vertebral level,the right upper bronchus was arisen from trachea and then the trachea was shrunk diameter to reach the leftlung hilar, at the end, the trachea divided 2 bronchi: left main bronchus and “bridging bronchus”. Thatbronchus from left lung hilar returned to the right lung.This patient was diagnosed birth defect: Left pulmonary artery sling with trachea stenosis, type IIA.

Scimitar Syndrome with Partial Left Pulmonary Artery Sling in a Neonate.

Scimitar syndrome is a rare condition including right lung hypoplasia, anomalous pulmonary venous drainage, dextroposition of the heart, and hypoplastic right pulmonary artery (RPA). The infantile form is sometimes associated with pulmonary hypertension, cardiac failure, and a poorer prognosis. We present a 1-day-old girl with a rare combination of partial left pulmonary artery (LPA) sling and scimitar syndrome, which was suspected in prenatal sonography and confirmed by echocardiography, computed tomography and cardiac catheterization. The patient received re-implantation of the aberrant left lower pulmonary artery and patch augmentation of the main-left upper pulmonary arteries at 4 months of age. Another operation was performed at 7 months of age with patch augmentation for stenosis of the left lower pulmonary artery and left pulmonary vein elevation to relieve its clenching of the left lower bronchus, with improvement of respiratory distress.

Pitfalls in the management of congenital tracheal stenosis: is conservative management feasible?

Congenital tracheal stenosis (CTS) is rare and challenging. Complete tracheal rings cause a wide spectrum of airway-obstructing lesions and varying degrees of respiratory distress. Although surgical reconstruction is the primary option for symptomatic CTS, sometimes an appropriate management strategy may be difficult due to other anomalies. We aimed to identify pitfalls in the management of CTS. We retrospectively reviewed the records of patients with CTS during the last 10years in our institution. Sixteen pediatric patients were diagnosed with CTS. Of the 16 patients, 12 (75.0%) had cardiovascular anomalies including seven left pulmonary artery sling. Six patients with dyspnoea caused by CTS and three patients with difficult intubations due to CTS underwent tracheoplasty. Four patients underwent only cardiovascular surgery without tracheoplasty. Three asymptomatic patients were followed up without undergoing any surgical procedure. We repeatedly discussed management of four patients with especially complex pathophysiology at multidisciplinary meetings. Right ventricular outflow tract obstruction, tracheobronchial malacia, increased pulmonary blood flow, and pulmonary aspiration due to gastroesophageal reflux presumably accounted for their severe respiratory distress, and we forewent their tracheal reconstruction. The management of CTS should be individualized, and conservative management is a feasible option in selected cases.

Prenatal diagnosis of anomalous origin of pulmonary artery.

Anomalous origin of the pulmonary arteries is a rare congenital pulmonary vascular malformation that includes unilateral absence of the pulmonary artery (UAPA), anomalous origin of unilateral pulmonary artery (AOPA), and left pulmonary artery sling (LPAS). We analyze 15 cases of fetal pulmonary artery abnormalities from 2011 to 2017, detected via prenatal ultrasound at our center. The 15 cases include UAPA (5), AOPA (6), and LPAS (4). Of the UAPA cases, 2 had pulmonary atresia and 3 had pulmonary artery stenosis. In 5 cases, the descending aorta issued collateral vessels to the left lung. Of the AOPA cases, 2 had left pulmonary artery abnormalities originating in the ascending aorta and 4 showed right pulmonary artery abnormalities originating in the ascending aorta. Of the LPAS cases, 2 were type IA, 1 was type IIA, and 1 was type IIB. Two of the LPAS were associated with right lung dysplasia. Abnormal origin of the pulmonary artery has a characteristic ultrasonographic appearance. The branch of the pulmonary artery cross section can provide important clues to the diagnosis of abnormal pulmonary artery origin. Pulmonary artery abnormalities are often associated with pulmonary atresia or stenosis.

Operative or non-operative treatment of congenital tracheal stenosis: is there something new?

In the 2015, 8th issue of the International Journal of Clinical and Experimental Medicine , HONG et al . (1) focus on the non-operative treatment of congenital tracheal stenosis (CTS) associated with left pulmonary artery sling (PAS).

The Management of Left Pulmonary Artery Sling in Senegal: Our Experience from One Case

Pulmonary Artery Sling (PAS) is a rare congenital heart disease due to abnormal origin of Left Pulmonary Artery (LPA) from the Right Pulmonary Artery (RPA), then encircling the distal part of the trachea. The treatment is a surgical repair consisting of reimplantation of the LPA on the main pulmonary artery over cardiopulmonary bypass. The authors report a clinical case of PAS in a 5.5 years old boy diagnosed in Senegal then successfully operated in France.Cardiovasc. j. 2017; 10(1): 94-96

Clinical and anatomic analysis of children with congenital bridging bronchus malformation

Objective To analyze the clinical and anatomic characteristics of bridging bronchus (BB) malformation in children, and to explore its diagnostic strategy, treatment and risk factors. Methods A retrospective study was performed in 23 pediatric patients with BB who were admitted to Guangzhou Women and Children′s Medical Center form May 2010 to October 2016.All their clinical features, imaging examination, treatment and prognosis were reviewed and analyzed. Results (1) Among the 23 patients with BB, 15 were males and 8 were females.The age range of onset was 0 to 4 years old with a median age of 3.17 months.(2)The main clinical manifestations were cough (23/23 cases, 100.0%), recurrent wheezing (20/23 cases, 87.0%), cyanosis (8/23 cases, 34.8%)and feeding difficulty/slow body weight growth (6/23 cases, 24.6%). (3) There were 17 cases of type Ⅰ and 4 cases of typeⅡaccording to Wells, and the other 2 cases were anterior BB.There were 2 cases with atypical anatomical morphology in type Ⅰ and typeⅡrespectively.(4) Twenty-one cases were associated with bronchial stenosis in which the origin of left main bronchus complicated with BB stenosis was the most common(5 cases). Bronchoscopy revealed bronchomalacia in 5 of the 16 patients.Sixteen patients associated with cardiac or vascular anomalies, 11 of whom were compound anomalies, and the most common type was sling left pulmonary artery (SLPA) (12 cases). (5) Nine of 12 patients with SLPA received surgical management, ages ranging from 1 month to 8 years old.The respiratory symptoms remitted gradually or disappeared from 4 to 17 months after surgical management.Two died and one has been lost to follow-up among the remaining 3 patients with SLPA. Conclusions BB is a rare tracheobronchial malformation that is often associated with stenosis or bronchomalacia, and cardiac or vascular anomalies.The severity of the disease mainly depends on whether there is airway stenosis or malacia and its extent.The definitive diagnosis of BB requires the combination of different imaging modalities.The combination with simple SLPA could get better curative effect. Key words: Bridging bronchus; Child; Cardiac or vascular anomalies; Bronchostenosis

Pulmonary artery sling—a case report

Vascular sling/pulmonary artery sling is a congenital anomaly in which the left pulmonary artery arises from the right pulmonary artery extrapericardially (anomalous left pulmonary artery), courses to the left behind the tracheal bifurcation and in front of the esophagus to reach the left lung hilum, and forms a sling around the trachea. The prevalence is 1 in every 17,000 school-aged children. We present a 1.5-year-old male child of consanguineous parentage with history of recurrent chest infection and stridor leading to repeated hospitalization. Chest X-ray showed right upper lobe collapse with hyper inflated right middle and lower lobes. Echocardiography revealed left pulmonary artery arising from the right pulmonary artery with no intracardiac shunt. Cardiac computed tomography diagnosed the pulmonary artery sling—left pulmonary artery arising from the right pulmonary artery and compromising the lumen of proximal portion of right bronchus leading to right upper lobe collapse. The surgery included complete mobilization of the left pulmonary artery from its origin and along its course behind the trachea. The left pulmonary artery was disconnected from the right pulmonary artery and was then brought out from behind the trachea and aorta and reimplanted to the main pulmonary artery in an end to side fashion. Child developed chest infection postoperatively, which was treated with intravenous antibiotics and was subsequently discharged on postop day 10. Child is on regular follow-up for the past 8 months and is doing well.

Evaluation of left pulmonary artery sling, associated cardiovascular anomalies, and surgical outcomes using cardiovascular computed tomography angiography

We evaluated the prevalence, image appearance, associated cardiovascular anomalies, and surgical outcomes of left pulmonary artery sling (LPAS) using cardiovascular computed tomography angiography (CCTA). A retrospective search of patients from our database between October 2007 and December 2014 identified 52,200 patients with congenital heart diseases (CHD) referred for CCTA, echocardiography, or magnetic resonance imaging. Clinical information, CCTA findings, associated cardiovascular anomalies, and surgical outcomes were analyzed. We showed a hospital-based prevalence of 71 patients with LPAS (0.14%, 71/52,200) among CHD patients. Of these, 47 patients with CCTA examinations were assessed further. Most patients (40/47, 85%) had associated cardiovascular anomalies, of which ventricular septal defects (22/47, 47%), atrial septal defects (20/47, 43%), patent ductus arteriosus (16/47, 34%), persistent left superior vena cava (14/47, 30%), and abnormal branching of the right pulmonary artery (ABRPA) (14/47, 30%) were most commonly identified. In total, 28 patients underwent LPA reanastomosis and/or tracheoplasty in our center, and 5 died. LPAS had a hospital-based prevalence of 0.14% among CHD patients. ABRPA is not uncommon and must be recognized. CCTA is a feasible method for demonstrating LPAS and its associated cardiovascular anomalies for an optimal pre-operative assessment of LPAS.

Right lung agenesis with left pulmonary artery sling: Computed tomography imaging

We report a case of an infant with respiratory distress and difficult intubation, who on multidetector computed tomography (MDCT) imaging was found to have an extremely rare combination of left pulmonary artery (LPA) sling and right lung agenesis (RLA). The complete right-sided mediastinal shift resulted in dextroposed, coronally placed, anteriorly crossing aortic arch causing posterior displacement and significant anterior compression of the trachea. The posterior tracheal displacement and stenosis made the intubation quite difficult and challenging in this patient. Cross-sectional imaging with computed tomography angiography has an edge over echocardiography and conventional pulmonary angiography in demonstrating the anomalous posterior course of an LPA in the presence of coexisting RLA. MDCT imaging plays a pivotal role in evaluating the structural anatomy and thus provides with pertinent information regarding the cause and the level of airway obstruction.

Left pulmonary artery sling without symptoms.

Left pulmonary artery sling without symptoms.

Congenital tracheal stenosis associated with left pulmonary artery sling accompanied by tracheal diverticula: A case report

Abstract We present the case of an eleven-year-old boy with history of stridor since birth with suspicion of tracheal stenosis due to a vascular ring. CT-evaluation revealed a pulmonary artery sling (PAS) and complete tracheal rings with a long segment funnel type tracheal stenosis. Histopathological evaluation additionally revealed a septum within the stenotic part dividing the trachea into a stenotic left part and a right part which turned out to only be an evagination like a small diverticulum. The long segment tracheal stenosis was opened in the middle and an anterior-posterior tracheal sliding plasty was performed after resecting the diseased part of the obstructive cartilage. The patient was extubated on the third postoperative day and the stridor disappeared immediately. To the best of our knowledge, this is the first report of tracheal stenosis with a pulmonary artery sling accompanied with tracheal diverticulum.

Virtual 3D Modeling of Airways in Congenital Heart Defects.

The involvement of the airway is not uncommon in the presence of complex cardiovascular malformations. In these cases, a careful inspection of the relationship between the airway and the vasculature is paramount to plan the surgical procedure. Three-dimensional printing enhanced the visualization of the cardiovascular structure. Unfortunately, IT does not allow to remove selected anatomy to improve the visualization of the surrounding ones. Computerized modeling has the potential to fill this gap by allowing a dynamic handling of different anatomies, increasing the exposure of vessels or bronchi to show their relationship. We started to use this technique to plan the surgical repair in these complex cases where the airway is affected. This technique is routinely used in our Institution as an additional tool in the presurgical assessment. We report four cases in which the airways were compressed by vascular structures – ascending aorta in one, left pulmonary artery sling in one, patent ductus arteriosus in one, and major aorto-pulmonary collateral artery in one. We believe this technique can enhance the understanding of the causes of airway involvement and facilitate the creation of an appropriate surgical plan.