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Paper 4
Published in Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw, The Final FRCR, 2020
Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw
The question described typical findings in a pulmonary sling or aberrant left pulmonary artery. Neonates can present with respiratory distress due to narrowing of the trachea and narrowing of the right main bronchus or bronchus intermedius, which can lead to air trapping. The left pulmonary artery arises from the right pulmonary artery and passes between the trachea anteriorly and the oesophagus posteriorly, hence indenting the posterior wall of the trachea and the anterior wall of the oesophagus. It is the only vascular anomaly to pass between the trachea and oesophagus.
The cases
Published in Chris Schelvan, Annabel Copeman, Jacky Davis, Annmarie Jeanes, Jane Young, Paediatric Radiology for MRCPCH and FRCR, 2020
Chris Schelvan, Annabel Copeman, Jacky Davis, Annmarie Jeanes, Jane Young
Echocardiography usually confirms the aberrant position of the left pulmonary artery and may demonstrate other cardiac abnormalities (which are present in 50% of cases), most commonly atrial septal defects, patent ductus arteriosus and ventricular septal defects.
Paediatrics
Published in Vincent Helyar, Aidan Shaw, The Final FRCR, 2017
Commonly described as a pulmonary sling, the aberrant left pulmonary artery arises from the right pulmonary artery. It then passes around distal trachea and between the trachea and oesophagus to reach the left lung. It therefore causes an impression on the anterior oesophageal wall. It presents in infancy with stridor and respiratory distress due to compression of the right main bronchus.
Pediatric bronchoscopy: recent advances and clinical challenges
Published in Expert Review of Respiratory Medicine, 2021
P Goussard, P Pohunek, E Eber, F Midulla, G Di Mattia, M Merven, JT Janson
The role of bronchoscopy in preschool wheezing includes evaluating the airways for any structural and anatomical abnormalities. It is also important to always evaluate the upper airways with flexible nasoendoscopy and laryngoscopy and not just a flexible bronchoscope through a laryngeal mask airway. Large airways can be either structurally or functionally abnormal or can present with a combination of abnormalities. Tracheomalacia and bronchomalacia can be missed if the child is not breathing spontaneously. Vascular abnormalities are more commonly found than airway stenosis. These abnormalities can include double aortic arch, innominate artery compression, left pulmonary artery sling, or left main bronchus (LMB) compression by a patent ductus arteriosus remnant [39]. The pattern of compression seen on bronchoscopy can be a guide to the type of vascular compression present.
Type I right pulmonary artery to left atrium fistula: a rare differential of cyanosis in adults
Published in Acta Cardiologica, 2020
Mumun Sinha, Niraj Nirmal Pandey, Arun Sharma, Sanjeev Kumar
A pulmonary artery (PA) to LA fistula is extremely rare and is thought to develop secondary to incomplete degeneration of the septum between the arterial and venous plexus of the pulmonary vascular bed during embryological development. It is more commonly seen involving the RPA compared to the left pulmonary artery. They are classified into four types based on the pulmonary venous drainage pattern seen in these cases. In the most common Type I, pulmonary artery and veins are normal with a fistulous channel connecting the RPA to LA. Type II shows the absence of the right inferior pulmonary vein with the lower lobe RPA branch draining directly into the LA. In Type III, there is anomalous drainage of pulmonary veins into the RPA to LA fistula. In Type IV, the right-sided pulmonary veins are connected to the aneurysmal pouch.
An update on current and emerging treatments for pulmonary arterial hypertension in childhood and adolescence
Published in Expert Review of Respiratory Medicine, 2019
Julie Wacker, Robert Weintraub, Maurice Beghetti
Reverse Potts shunt or creation of a unidirectional left pulmonary artery to descending aorta connection in an effort to facilitate right-to-left shunting has been shown to provide effective palliation in carefully selected children with end-stage pulmonary vascular disease and suprasystemic PAH, improving functional capacity [76,77]. The Potts shunt can be performed purely by interventional catheter or require surgery. The Potts recreate the physiology of an Eisenmenger syndrome in the setting of a PDA, with a desaturation of the lower body only. The shunt has the advantages to work in the setting of a preserved RV function. In fact, severe RV dysfunction is a contra-indication to a Potts shunt, with the risk of decreasing the cardiac output. In the French series, of the 24 children who underwent a Potts shunt, there were three early deaths related to low cardiac output, but the functional class was improved in all the survivors, with an ability to wean epoprostenol as the third medication in a majority of the patients [77]. Other pediatric studies seem to corroborate those findings [78,79].