Pulmonary artery sling with bronchial bridge malformation in children: a report of 3 cases / 中国综合临床

Pulmonary artery sling (PAS) with bronchial bridge malformation is a very rare developmental malformation of vascular and trachea. In the past 2 years, we treated 3 children with pulmonary artery sling complicated with bronchial bridge, all of whom were clinically characterized by recurrent cough, asthma and dyspnea, which were confirmed by cardiac color ultrasound and chest CT three-dimensional reconstruction. All of the 3 children underwent surgical treatment, and no recurrent wheezing or respiratory tract infection occurred after surgery.

Anillos vasculares: Serie de 28 pacientes atendidos en un Servicio de Endoscopía Respiratoria Pediátrica / Vascular rings: Series of 28 patients of a Pediatrie Respiratory Endoscopy Department

Los anillos vasculares tienen una incidencia del 1 % entre las enfermedades cardiovasculares congénitas. Constituyen una malformación embriológica en la que el arco aórtico, sus ramas o las arterias pulmonares provocan presión sobre la tráquea y/o el esófago. Anatómicamente, se dividen en dos grupos, dependiendo de cómo rodean la tráquea y el esófago: completo o incompleto. La sintomatología suele iniciarse en los primeros meses de vida con estridor bifásico o espiratorio que aumenta con el llanto y la alimentación, tos traqueal de tonalidad metálica, infecciones respiratorias recurrentes, episodios de apnea refleja y cianosis, sibilancias, hiperextensión cervical, retracción esternal e intercostal, y dificultad en la alimentación. Se realizó un análisis retrospectivo de 28 pacientes con diagnóstico de anillo vascular que concurrieron a la consulta en el Servicio de Endoscopía Respiratoria del Hospital Garrahan entre enero de 2015 y septiembre de 2017.

Vascular rings account for 1 % of the congenital cardiovascular diseases. They constitute an embryological malformation in which the aortic arch, its branches, or the pulmonary arteries cause pressure on the trachea and/or oesophagus. Anatomically, they are divided into two groups -complete or incomplete- depending on how they surround the trachea and/or the oesophagus. Symptom onset is usually in the first months of life with biphasic or expiratory stridor that increases with crying and feeding, a metallic tracheal cough, recurrent respiratory infections, episodes of apnea and cyanosis, wheezing, cervical hyperextension, sternal and intercostal retraction, and feeding difficulties. A retrospective analysis of 28 patients with vascular rings seen at the Department of Respiratory Endoscopy at Garrahan Paediatric Hospital between January 2015 and September 2017 is presented.

Research progress of infant pulmonary artery sling / 中华实用儿科临床杂志

The pulmonary artery sling (PAS)is a rare congenital cardiovascular disease and usually associated with airway stenosis and cardiovascular anomalies. Abnormal left pulmonary artery oppress trachea and esophagus can cause respiratory tract obstruction or esophageal compression symptoms,such as recurrent wheezing,cough,lung infec-tions,difficulty of breathing or swallowing. Once diagnosed,the patients has surgical indications,but due to the lack of specific clinical manifestations,missed diagnosis or misdiagnosis for other respiratory diseases may occur. The surgical method of PAS is mainly the left pulmonary transplantation,whether perform tracheoplasty remains controversial.

Simultaneous Repair of Pulmonary Artery Sling and Tracheal Stenosis in Neonate / 日本心臓血管外科学会雑誌

A 17-days-old-girl with pulmonary artery (PA) sling, patent ductus arteriosus (PDA), and persistent left superior vena cava was admitted to our hospital. Despite good respiratory status just after birth, the respiratory status gradually worsened, and tracheal intubation was performed on 13th day after birth. Emergency division of the PDA was conducted on day 16. Although preoperative computed-tomography did not demonstrate tracheal stenosis, bronchoscopy after tracheal intubation revealed progression of tracheal stenosis with difficulty for stable anesthetic management. Therefore, operation was postponed to re-evaluate tracheal stenosis. Bronchoscopy and computed-tomography revealed the worsening of tracheal stenosis. Eventually, we performed total correction of the PA sling and tracheal stenosis on day 17. Cardiopulmonary bypass was established after median sternotomy. After transection of the PDA, the left PA originating from the right PA was also transected, and transplanted to the main PA. Then, sliding tracheoplasty was performed following the division of the tracheal stenotic region. Her respiratory condition improved after operation, and postoperative computed-tomography showed successful correction of tracheal stenosis. Although few such cases were reported for neonatal PA sling requiring concomitant tracheoplasty, this case suggests that total correction of PA sling and tracheal stenosis is feasible and useful surgical procedure for such cases.

Prognostic analysis of patients with pulmonary artery sling combined with tracheal stenosis without tracheal intervention / 中华实用儿科临床杂志

Objective To analyze the prognosis of patients with pulmonary artery sling (PAS) combined with tracheal stenosis(TS) without tracheal intervention,and to discuss the method for improving the effect of treatment.Methods The clinical data of 17 children with PAS/TS (moderate or severe) who received treatment at Beijing Bayi Children's Hospital Affiliated to General Hospital of Bejing Military Command from October 2011 to July 2015 were retrospectively analyzed,and the relationship between the effect and prognosis of re-implantation of the left pulmonary artery (LPA) without tracheal intervention was analyzed.Results Seventeen patients received re-implantation of the left pulmonary artery without tracheal intervention.Extubation was successfully performed in 12 of the 17 patients and they healed.The remaining 5 patients received tracheal intervention after the first operation but they all died.Of those 5 patients,3 received tracheal stent implantation (1 died from necrotizing enterocolitis,2 died from infection and multiple organ failure),and 2 received traheoplasty (both of them died from infection and tracheal fistula).Respiratory symptoms were reduced or resolved in all survivors.Diameter/length (％) in survivors without tracheal intervention was significantly higher than those who received tracheal intervention [(10.14 ± 1.58) ％ vs.(5.72 ± 1.17) ％,t =3.600,P ＜ 0.001].Patients with PAS undergoing LPA re-implantation achieved a good outcome if the diameter/length(％) of the trachea was up to (10.14 ± 1.58) ％.Patients with PAS undergoing LPA re-implantation achieved a bad outcome if the diameter/length(％) of the trachea was below to (5.72 ± 1.17) ％.Conclusions Most of patients with PAS/TS undergoing LPA re-implantation without tracheal intervention have a good outcome.It is feasible for them to avoid tracheal intervention.Diameter/length (％) may be a reliable indicator for determining tracheal intervention in surgical management of PAS.

Treatment of 21 pediatric patients with pulmonary artery sling combined with tracheal stenosis / 中国小儿急救医学

Objective Total 21 pulmonary artery sling( PA sling)combined with tracheal stenosis children who received treatment in our hospital were reviewed. The feasibility of treatment strategy including left pulmonary artery( LPA)re-implantation without tracheoplasty was discussed in this study. Methods From April 2009 to November 2015,a total of 21 pediatric patients received surgical treatment due to PA sling with tracheal stenosis. Six patients received LPA re-implantation and trachea intervention simultaneously. The other 15 patients received LPA re-implantation alone to relieve the trachea compression without tracheoplasty. The postoperative strategy including early extubation and CPAP ventilation was employed in PICU. Results A total of 21 PA sling with tracheal stenosis children who underwent surgical treatment in our hospital were recruited. There were 9 females and 12 males. Ages of these children were from 1 months to 10 years old,and body weights were from 2. 9 kg to 25. 0 kg. Five patients needed mechanical ventilation for severe respiratory symptoms preoperatively. Six patients received LPA re-implantation and tracheal interven-tion simultaneously. Among them,3 patients received slide tracheoplasty,and one was discharged after recov-ery. The remaining 3 patients received tracheal stent implantation,but finally died. The survival rate was 16. 7% in these patients. Fifteen patients received LPA re-implantation alone,and slide tracheoplasty was per-formed in 2 patients for extubation failure who finally died of air leakage. The survival rate of 15 patients who received LPA re-implantation alone was 86. 7%. Conclusion The strategy in LPA re-implantation alone to relieve the trachea compression without tracheoplasty and early extubation and CPAP ventilation postoperative may be an ideal treatment for the pediatric patients with PLA sling combined with tracheal stenosis.

Clinical diagnosis, treatment, and prognosis of pulmonary artery sling in 38 children / 临床儿科杂志

Objective To explore the clinical characteristics and prognosis of congenital pulmonary artery sling (PAS) in children. MethodsThe clinical data of 38 children diagnosed with PAS during June 2009 and February 2015 were retrospectively analyzed. ResultsIn 38 PAS children, 35 cases (89.47%) were hospitalized for varying degrees of respiratory manifestations with recurrent cough (89.47%) and wheezing (84.21%) being the most common. The remaining 3 cases were found abnormal in routine preoperative examination and the diagnosis was confirmed after further examination. All 38 children were performed computer tomography angiography (CTA). Thirty-seven cases were diagnosed of PAS and diagnostic rate was 97.37%. One case was suspected of pulmonary dysplasia and diagnosed of PAS after operation. Twenty-six children received surgical treatment, of whom 25 children had pulmonary artery reconstruction (LPA). Seven children died during/after operation and 18 survived. The remaining 12 children received non-surgical treatment, of whom 9 died and 3 survived.ConclusionCardiac uhrasonography may reveal PAS in the early stage, while CTA is the best method for conifrmed diagnosis. LPA reconstruction is an important means of relieving left pulmonary artery oppression.

The review of surgical treatment to pulmonary artery sling / 国际儿科学杂志

Pulmonary artery sling(PAS)is a rare congenital cardiovascular disease.Due to the compression of the trachea and esophagus,it often causes severe airway and/or esophageal compression symptoms.Once diagnosed,the patient should undergo surgical treatment.There are various treatments,including left pulmonary artery reconstruction,tracheal stenosis correction and tracheal balloon dilatation and stent technology.Whether or not to repair the tracheal stenosis usually based on the trachea diameter.But there has no ideal surgical procedure to the tracheal stenosis.The patient with complex introcardiac malformation and PAS should be corrected simultaneously with cardiopulrmonary bypass.

An adult asymptomatic pulmonary artery sling / 영남의대학술지

A pulmonary artery sling is a very rare congenital abnormality in which the left pulmonary artery rises from the posterior surface of the right pulmonary artery and then passes between the trachea and the esophagus, causing tracheal compression. It is associated with tracheo-bronchial abnormalities (50%) and cardiovascular abnormalities (30%). It may produce respiratory symptoms through the airway compression of the abnormal left pulmonary artery and congenital abnormalities associated with it. Because most (90%) pulmonary artery sling patients present symptoms during infancy, their condition is often diagnosed in the first year of life. However, a pulmonary artery sling is occasionally found in adults. It is usually asymptomatic and found incidentally. This is a very rare case of an asymptomatic pulmonary artery sling in an adult. A 38-year-old man presented symptoms of mild exertional dyspnea. His spiral computed tomography showed a pulmonary artery sling. He was discharged without specific treatment because his symptoms improved without specific treatment and might not have been associated with a pulmonary artery sling. We report an adult case of an asymptomatic pulmonary artery sling diagnosed via spiral computed tomography, accompanied by a literature review.

Clinical analysis of 21 cases of pediatric bridging bronchus / 临床儿科杂志

Objective To explore the clinical diagnosis and treatment of pediatric bridging bronchus deformity and brid-ging bronchus with pulmonary artery sling. Methods The clinical data of the 21 cases of diagnosed bronchial bridge deformity and bronchial bridge with pulmonary artery sling, the imaging findings of all the echocardiography, MSCT chest scan, enhanced CT and reconstructive image were retrospectively analyzed. Results In 21 cases, 10 were diagnosed as bronchial bridge only, among which three cases had other types of congenital heart disease;11 cases were diagnosed of pulmonary artery sling with pulmonary artery sling, among which 8 cases had other types of congenital heart disease;their average age of the 8 cases were all younger than 2 years old, except one case;most of them had the symptoms of recurrent respiratory tract infections and parox-ysmal dyspnea. According to Wells, bronchial bridge was divided into type I and typeⅡ;15 cases were type I, 6 cases were typeⅡ, 10 patients with bronchial bridge only were all type I, 5 cases of typeⅡwere associated with pulmonary artery sling. All of the 6 cases were diagnosed as typeⅡassociated with pulmonary artery sling. Conclusions Infants and young children with clini-cal recurrent respiratory tract infections should be considered the possibility of bronchus bridge deformity, and should be paid more attention to the possibility of pulmonary artery sling. MSCT plain chest scan, enhanced CT and airway reconstructive im-age are the important ways to diagnose the disease.

Clinical Course of Vascular Rings and Risk Factors Associated With Mortality

BACKGROUND AND OBJECTIVES: Vascular rings refer to anomalies of the great arteries that cause respiratory or feeding problems. The purpose of this study was to analyze a series of patients with vascular rings and evaluate associated risk factors for mortality. SUBJECTS AND METHODS: A retrospective review of all patients identified with vascular rings between 1997 and 2010 in the Seoul National University Children's Hospital. RESULTS: Thirty-five patients were diagnosed with vascular rings (median age at diagnosis, 7 months). The vascular rings of 32 patients were confirmed by cardiac computed tomography or magnetic resonance imaging. The types of vascular rings were: a double aortic arch in ten patients, a right aortic arch with persistent left ligamentum arteriosum in seven, an aberrant subclavian artery in seven, a pulmonary sling in eight, and others types in three patients. Eleven patients were asymptomatic. Gastrointestinal and respiratory symptoms were seen in ten and sixteen patients, respectively. Associated cardiovascular anomalies were present in fifteen patients. Twenty patients with definite symptoms underwent surgical treatment. The median interval between diagnosis and operation was 6 days. Four patients eventually died; three deaths were associated with complex heart diseases, and one had pulmonary artery sling with severe tracheal stenosis. Only the presence of a complex heart disease significantly influenced mortality (p=0.002). CONCLUSION: Vascular rings include several types of anomalies, each with a different prognosis and symptoms. The presence of a complex heart disease was significantly associated with mortality. Early diagnosis and timely surgery in symptomatic patients are essential.

A Case of Arch Anomaly with Right Aortic Arch and Pulmonary Artery Sling in Conjunction with Persistent Left Fifth Aortic Arch / 日本心臓血管外科学会雑誌

Persistent fifth aortic arch has been thought to be rare but often relevant to systemic circulation, however when it connects to the pulmonary artery (PA) in pulmonary atresia, it may be the sole arterial supply to the lung. This report describes a case of rare arch anomaly including right aortic arch (RAA), PA sling in conjunction with persistent left fifth aortic arch (PLFAA) and left subclavian artery arising from the left fourth arch. The tetralogy of Fallot, pulmonary atresia, and total anomalous of pulmonary venous connection (cardiac) were also diagnosed. A neonate was referred to our hospital for surgical treatment of cardiac and extracardiac anomalies. Persistent fifth aortic arch connecting with pulmonary artery was initially thought to be patent ductus arteriosus (PDA), so prostaglandin E 1 administration was commenced. He underwent emergency colostomy for anal atresia. Subepiglottic tracheal stenosis was diagnosed at initiation of anesthesia. At age 1-month-old, he required systemic to pulmonary shunt and reimplantation of left pulmonary artery through a median sternotomy using extracorporeal circulation. At the operation the PDA was divided and oversewn, and the wall structure was the same as that of a normal artery. The left pulmonary artery behind trachea was dissected and we then cut away and reimplanted to pulmonary trunk. Tracheostomy was performed at the age of two months. With the technical development of diagnostic imaging, the morphological features of arch anomaly were clearly demonstrated, but some understanding of embryological aspects are still required for diagnosis.

A Case of Pulmonary Artery Sling Misdiagnosed as Bronchial Asthma / 소아과

Anomalous left pulmonary artery(pulmonary artery sling) is a congenital anomaly in which the vascular structure arises either from the posterior surface of the right pulmonary artery, or from the main pulmonary artery and courses to the left lung between the posterior surface of the trachea and the anterior surface of the esophagus. It may compress on the tracheobronchial tree causing significant symptoms on the part of the respiratory system such as dyspnea, stridor, or cyanosis. It is a rare condition leading to death in the first months of life, if it is not corrected. We present a case of pulmonary sling who was suffering from recurrent lower respiratory tract diseases, expiratory wheezing, and intermittent dyspnea since birth, thus she was treated for bronchial asthma. We confirmed the diagnosis with computerized tomography, especially with three dimensional reconstruction, which provided information regarding the spatial relationship between the anomalous pulmonary artery branch and the trachea and bronchi.

Anterior Tracheoplasty: Two Cases Report / 대한흉부외과학회지

We describe here two cases of anterior tracheoplasty utilizing an autologous pericardial patch. One patient was a 9 year-old female who had a congenital long tracheal stenosis associated with major vascular anomalies including pulmonary artery sling. One-stage correction was done under the support of an extracorporeal membrane oxygenation system. She required a prolonged ventilation support for 10 days postoperatively until the implanted pericardium was fixed to the mediastinal structures. The other patient was a 8 year-old male who had acquired tracheal stenosis following a complicated tracheostomy. By applying additional support over the pericardial patch with the costal cartilage, an endotracheal tube could be removed immediately after the operation. Both patients have been doing well in a postoperative follow-up of over a year, and there have been evidences of growth in the reconstructed trachea.

Atypical pulmonary artery sling with diffuse-type pulmonary arteriovenous fistula

The case of a cyanotic infant with a rare combination of atypical pulmonary artery sling, imperforate anus, absence of the left kidney, interruption of the inferior vena cava, left side hemihypertrophy and diffuse-type pulmonary arteriovenous fistula is described. The clinical features were confusing, because of compounding abnormalities involving the respiratory tract and pulmonary circulation. The diagnostic approach to the etiology of cyanosis is discussed and the embryonic origin of pulmonary artery sling is reviewed.

A Case of Pulmonary Artery Sling Associated with Left Bronchial Stenosis and Patent Ductus Arteriosus

Pulmonary artery sling is a rare and potentially lethal vascular anomaly wherein the anomalous left pulmonary artery arises from the posterior aspect of the right pulmonary artery and passing to the left lung between the trachea and the esophagus which causes respiratory distress in infants and children due to the extrinsic compression of the airway by the anomalous vessel. Associated anomalies are common, particularly those of the tracheobronchial tree. The most common lesions are hypoplasia of the distal trachea and right main stem bronchus. About 50 percent of patients have cardiovascular anomalies, such as in the left superior vena cava, atrial septal defect, ventricular septal defect, patent ductus arteriosus, aortic arch anomalies, tetralogy of Fallot 1 etc. We experienced a case of a 6-year-old boy who showed dyspnea and frequent pneumonia. He was diagnosed with pulmonary artery sling associated with left bronchial stenosis, patent ductus arteriosus, and left superior vena cava which was parven by echocardiographic examinations, spiral computerized tomography of the chest and cineangiography. We report this case with related literature.

A Case of Pulmonary Artery Sling

Pulmonary artery sling is a rare vascular anomaly in which the left pulmonary artery arises from the right pulmonary artery and then traverses between the esophagus and the trachea toward the hilum of the left lung. Despite the availability of a corrective operation, the mortality rate remains very high due to the high incidence of associated obstructive anomalies of tracheobronchial trees. We experienced a 8 month old female infant who was admitted because of recurrent pneumonia and expiratory wheezing. She was diagnosed as pulmonary sling by esophagography, echocardiography, computed tomography. We report this with a brief review and related literature.

Four Cases of Pulmonary Artery Sling with Bridging Bronchus

Pulmonary artery sling with bridging bronchus is not only rare but also difficult to diagnose unless specially sought. When young infant suffers from prolonged or recurrent wheezing, possibility of underlying anomalies of the tracheobronchial trees or great vessels should be considered, even though bronchiolitis and gastroesophageal reflux are the most common causes. We experienced four cases of pulmonary artery sling with bridging bronchus who presented with dyspnea and recurrent wheezing since infancy. Diagnosis was made using bronchoscopy, three dimensional computed tomography, echocardiography and angiography. Bridging bronchus of three patients (case 1, 2, 3) correspond to type IIA and one patient (case 4) to type IIB by Wells classification. Bronchoscopic examinations on 3 patients showed segmental bronchomalacia and near complete obstruction of bronchus by the posterior left pulmonary artery. These congenital bronchial anormalies should be included in differential diagnosis in pediatric patients with unexplained persistent and recurrent wheezing, or emphysema of unknown etiology on plain chest X-ray.

Air Way Instability due to Pulmonary Artery Sling combined with Diffuse Tracheal Stenosis / 대한마취과학회지

Pulmonary artery sling is a rare congenital condition in which the left pulmonary artery arises anomalously fmm the right pulmonary artery extrapericardially and encirdes the right main stem bronchus and passes between the trachea and esophaghus to reach the hilum of the left lung. Respiratory obstruction vesults from compression of the lower area of trachea and right main stem bronchus. It is rare cause of respiratory distress in the new bom and may be associated with significant morbidity and mortality. We experienced the case of pulmonary artery sling with diffuse trachea stenosis and congenital heart disease (pulmonary atresia and PDA) and confirmed the anomaly by bronchogram and MRI. The presumed cause of death was respiratory obsruction due to pulmonary artery sling.