Severe stenosis of a long tracheal segment, with agenesis of the right lung and left pulmonary arterial sling

2006 ◽  
Vol 16 (1) ◽  
pp. 89-91 ◽  
Author(s):  
Hamish M. Munro ◽  
Andrea M. C. Sorbello ◽  
David G. Nykanen
Keyword(s):  
Pulmonary Artery ◽  
Surgical Repair ◽  
Main Bronchus ◽  
Left Lung ◽  
Proximal Part ◽  
Pulmonary Vasculature ◽  
Severe Stenosis ◽  
Right Pulmonary Artery ◽  
Pulmonary Arterial ◽  
The Right

A baby presented at term with respiratory distress was managed with extracorporeal membrane oxygenation. Bronchoscopy revealed tracheal hypoplasia, complete tracheal rings, and agenesis of the right main bronchus. Echocardiography showed a left pulmonary arterial sling arising from the proximal part of the right pulmonary artery. Cardiac catheterization demonstrated abnormal pulmonary vasculature in the left lung which would have prevented survival, even after surgical repair. Diagnostic catheterization was important in delineating the anatomy, and aided in the decision not to proceed with surgical repair.

Incidental finding of pulmonary arterial sling during patent ductus arteriosus surgery in a patient with Mowat–Wilson syndrome

2018 ◽  
Vol 28 (8) ◽  
pp. 1074-1076 ◽  
Author(s):  
Juan D. Cano Sierra ◽  
Camilo F. Mestra ◽  
Miguel A. Ronderos Dumit
Keyword(s):  
Pulmonary Artery ◽  
Incidental Finding ◽  
Ductus Arteriosus ◽  
Left Lung ◽  
Posterior Wall ◽  
Left Pulmonary Artery ◽  
Genetic Condition ◽  
Right Pulmonary Artery ◽  
Pulmonary Arterial ◽  
The Right

AbstractMowat–Wilson syndrome is a genetic condition due to a mutation in the ZEB2 gene; it affects many systems including the cardiovascular system. The pulmonary arterial sling originates from a failure of development of the proximal portion of the left sixth aortic arch, resulting in an anomalous left pulmonary artery origin from the posterior wall of the right pulmonary artery and the left pulmonary artery crossing to the left lung between the trachea and the oesophagus. We present a 4-month-old infant with Mowat–Wilson syndrome and left pulmonary arterial sling, and discuss the association of these two rare conditions. Pulmonary arterial sling is significantly more frequent in patients with Mowat–Wilson syndrome than in the general population.

scholarly journals Left pulmonary artery sling with tracheal stenosis

2018 ◽  
Vol 2 (5) ◽  
Author(s):  
Nguyen Chi Lang ◽  
Nguyen Thi Ngoan ◽  
Nguyen Duc Khoi
Keyword(s):  
Pulmonary Artery ◽  
Main Bronchus ◽  
Left Lung ◽  
Left Pulmonary Artery ◽  
Vertebral Level ◽  
Pulmonary Artery Sling ◽  
Right Pulmonary Artery ◽  
Bridging Bronchus ◽  
Left Pulmonary Artery Sling ◽  
The Right

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.

Cardiovascular-associated tracheobronchial obstruction in children

2012 ◽  
Vol 23 (2) ◽  
pp. 233-238 ◽  
Author(s):  
Shen-Hao Lai ◽  
Sui-Ling Liao ◽  
Kin-Sun Wong
Keyword(s):  
Pulmonary Artery ◽  
Airway Obstruction ◽  
Main Bronchus ◽  
Main Pulmonary Artery ◽  
Diagnostic Tools ◽  
Radiographic Findings ◽  
Tracheal Compression ◽  
Airway Obstructions ◽  
Right Pulmonary Artery ◽  
The Right

AbstractTracheobronchial compression of cardiovascular origin is an uncommon and frequently unrecognised cause of respiratory distress in children. The compression may be due to encircling vessels or dilated neighbouring cardiovascular structures. Bronchoscopy and detailed radiography, especially computed tomography and magnetic resonance imaging, are among the most powerful diagnostic tools. Few previous reports have addressed the relationship between bronchoscopic findings and underlying cardiovascular anomalies. The objective of this study was to correlate bronchoscopic and radiographic findings in children with cardiovascular-associated airway obstruction. A total of 41 patients were recruited for the study. Patients with airway obstruction were stratified on the basis of the aetiology of the cardiovascular structures and haemodynamics into an anatomy-associated group and a haemodynamics-associated group. In the anatomy-associated group, stenosis and malacia were found with comparable frequency on bronchoscopy, and the airway obstructions were mostly found in the trachea (71% of patients). In the haemodynamics-associated group, malacia was the most common bronchoscopic finding (85% of patients), and nearly all locations of airway involvement were in the airway below the carina (90% of patients). The tracheal compression was usually caused by aberrant systemic branching arteries in the anatomy-associated group. In the haemodynamics-associated group, the causal relationships varied. Tracheal compression was often caused by lesions of the main pulmonary artery and aorta, whereas obstruction of the right main bronchus was caused by lesions of the main pulmonary artery and right pulmonary artery. The causes of left main bronchus compression were more diverse. In summary, the bronchoscopic presentations and locations are quite different between these two groups.

Systemic arterial supply of the right lung with venous drainage to the pulmonary arterial circuit

2011 ◽  
Vol 21 (6) ◽  
pp. 710-712 ◽  
Author(s):  
Peter Fritsch ◽  
Freyja-Maria Smolle-Juettner ◽  
Andreas Gamillscheg
Keyword(s):  
Pulmonary Artery ◽  
Venous Drainage ◽  
Left Pulmonary Artery ◽  
Scimitar Syndrome ◽  
Arterial Supply ◽  
Respiratory Problems ◽  
Right Pulmonary Artery ◽  
Pulmonary Arterial ◽  
The Right ◽  
Systemic Arterial Supply

AbstractIn a girl suffering from “Scimitar syndrome”, a rerouting of the scimitar vein was performed at the age of 6 years, but no embolisation of the aberrant systemic vessel was done. She presented with recurring respiratory problems 13 years later. An angiography revealed an invert flow from the aberrant systemic vessel via the right pulmonary artery into the left pulmonary artery. After pneumonectomy, she recovered well.

Non-confluent pulmonary arteries in a patient with pulmonary atresia and intact ventricular septum: ? a 5th aortic arch with a systemic-to-pulmonary arterial connection

2000 ◽  
Vol 10 (4) ◽  
pp. 419-422 ◽  
Author(s):  
Astolfo Serra ◽  
Francisco Chamie ◽  
R.M. Freedom
Keyword(s):  
Pulmonary Artery ◽  
Aortic Arch ◽  
Pulmonary Arteries ◽  
Pulmonary Atresia ◽  
Left Pulmonary Artery ◽  
Ventricular Septum ◽  
Intact Ventricular Septum ◽  
Right Pulmonary Artery ◽  
Pulmonary Arterial ◽  
The Right

AbstractMajor abnormalities of pulmonary circulation are uncommon in the patient with pulmonary atresia and intact ventricular septum. Non-confluent pulmonary arteries have only rarely been described in this setting. In this case report, we describe a patient in whom the pulmonary arteries are non-confluent, with the right pulmonary artery supplied through a right-sided arterial duct, and the left pulmonary artery most likely through a fifth aortic arch, thus providing a systemic-to-pulmonary arterial connection. We discuss the various forms of non-confluent pulmonary arteries in the setting of pulmonary atresia and intact ventricular septum.

scholarly journals Pulmonary Artery Intimal Sarcoma: A Case Report

2016 ◽  
Vol 9 (1) ◽  
pp. 267-272 ◽  
Author(s):  
Joseph P. Kriz ◽  
Nabil A. Munfakh ◽  
Gregory S. King ◽  
Juan O. Carden
Keyword(s):  
Pulmonary Artery ◽  
Malignant Tumors ◽  
Pulmonary Arteries ◽  
Vena Cava ◽  
Filling Defect ◽  
Intimal Sarcoma ◽  
Right Pulmonary Artery ◽  
Pulmonary Arterial ◽  
Left And Right ◽  
The Right

Pulmonary artery intimal sarcomas are rare and lethal malignant tumors that typically affect larger vessels: the aorta, inferior vena cava, and pulmonary arteries. Since symptoms and imaging of pulmonary arterial intimal sarcomas mimic pulmonary thromboembolism, the differential diagnosis of a patient presenting with chest pain, dyspnea, and filling defect within the pulmonary arteries should include intimal sarcoma. Often right ventricular failure is observed due to pulmonary hypertension caused by the obstructive effect of the tumor and concomitant chronic thromboembolism. We report the case of a 72-year-old African-American male with arterial intimal sarcoma of the left and right pulmonary artery with extension through the right artery into the bronchus and right lung.

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