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Journal of the American Society of... Apr 2021Fetal aortic arch anomalies and pulmonary slings can be difficult to accurately diagnose but have important clinical implications related to vascular rings, congenital... (Review)
Review
Fetal aortic arch anomalies and pulmonary slings can be difficult to accurately diagnose but have important clinical implications related to vascular rings, congenital heart disease, and chromosomal anomalies. In this article, the authors briefly review the embryology and development of the fetal arch to facilitate understanding of its diverse variants. Two-dimensional echocardiographic characteristics are summarized for each type of these malformations to propose a strategy for fetal diagnosis. The added benefits of three-dimensional echocardiography with spatiotemporal image correlation are also shown. Finally, the authors propose a strategy for volume acquisition and postanalysis to spur postanalysis clinical use of this technology.
Topics: Aorta, Thoracic; Echocardiography; Echocardiography, Three-Dimensional; Female; Heart Defects, Congenital; Humans; Pregnancy; Ultrasonography, Prenatal; Vascular Ring
PubMed: 33166631
DOI: 10.1016/j.echo.2020.10.013 -
Journal of Pediatric Surgery Jul 2017Vascular rings are traditionally treated via an open thoracotomy. In recent years the use of thoracoscopy has increased. Herein we report our experience with...
BACKGROUND/PURPOSE
Vascular rings are traditionally treated via an open thoracotomy. In recent years the use of thoracoscopy has increased. Herein we report our experience with thoracoscopic division of vascular rings in pediatric patients.
METHODS
We reviewed all patients who underwent thoracoscopic or open division of a vascular ring at our institution between 2007 and 2015. We analyzed patient demographics, presenting symptoms, diagnostic imaging modality, ring anatomy, operative details, complications, and symptom resolution.
RESULTS
Thirty-one patients underwent thoracoscopic division of a vascular ring while sixteen had open operations. Median age was 24months in the thoracoscopic group and 13months in the open group. Operative time averaged 74min (thoracoscopic) and 95min (open). There were no mortalities at 30days. There was complete symptom resolution in 71% of thoracoscopic patients and 63% of open. Patients in the thoracoscopic group had decreased ICU admissions (10% vs. 94%), chest tube use (62% vs. 100%), chylothorax (6% vs. 38%) and overall length of stay (1.7days vs. 5days).
CONCLUSIONS
Thoracoscopic division of vascular rings in pediatric patients is a feasible alternative to open division and is associated with comparable rates of symptom resolution and decreased length of hospital stay and chylothorax.
LEVEL OF EVIDENCE
III.
Topics: Adolescent; Child; Child, Preschool; Female; Follow-Up Studies; Humans; Infant; Infant, Newborn; Length of Stay; Male; Retrospective Studies; Thoracoscopy; Thoracotomy; Treatment Outcome; Vascular Ring
PubMed: 28213995
DOI: 10.1016/j.jpedsurg.2017.01.064 -
Italian Journal of Pediatrics Feb 2023Abnormalities in position and/or branching of the aortic arch can lead to vascular rings that may cause narrowing of the tracheal lumen due to external compression, or... (Review)
Review
Abnormalities in position and/or branching of the aortic arch can lead to vascular rings that may cause narrowing of the tracheal lumen due to external compression, or constriction of the oesophagus, causing symptoms that vary in relation to the anatomical vascular pattern and the relationship between these structures. Respiratory morbidity related to external airways compression is a major concern in children affected by vascular rings. Clinical presentation depends on the severity of the tracheal lumen reduction and the presence of associated tracheomalacia. Recurrent respiratory infections, wheezing, atelectasis, and hyperinflation are mostly reported. As they are nonspecific and therefore difficult to recognize, attention should be given to all children with history of respiratory distress, extubation failure, noisy breathing, and recurrent respiratory infections. Early diagnosis and referral to specialized centres can prevent the long-term complications and improve the respiratory outcomes of these patients.
Topics: Child; Humans; Infant; Vascular Ring; Aorta, Thoracic; Respiratory Sounds; Respiratory Tract Infections; Morbidity
PubMed: 36797770
DOI: 10.1186/s13052-023-01430-x -
Maedica Dec 2021Double aortic arch represents a congenital vascular malformation that is characterized by the development of a complete vascular ring around the esophagus and trachea...
Double aortic arch represents a congenital vascular malformation that is characterized by the development of a complete vascular ring around the esophagus and trachea due to an anomaly in the development of branchial arteries. We present the case of a 31-year-old gravida that was referred for fetal ultrasound anomalies screening at 22 weeks and six days of gestation. Routine ultrasound scanning of the fetus revealed a structural aortic arch anomaly consistent with a double aortic arch, with no other cardiac and diextracardiac congenital structural malformations. Knowledge of embryology and imaging spectrum of aortic arch anomalies that are able to form vascular rings around the trachea and esophagus are essential for an accurate antenatal diagnosis and therefore, for a correct clinical management.
PubMed: 35261677
DOI: 10.26574/maedica.2020.16.4.717 -
Translational Pediatrics Apr 2017
PubMed: 28503412
DOI: 10.21037/tp.2017.03.05 -
Journal of Paediatrics and Child Health May 2017
Topics: Child, Preschool; Humans; Male; Treatment Outcome; Vascular Ring
PubMed: 28470807
DOI: 10.1111/jpc.13539 -
European Journal of Cardio-thoracic... Nov 2021This review aims at presenting and summarizing the current state of literature on the presentation and surgical management of a right-sided aortic arch with a left-sided... (Review)
Review
OBJECTIVES
This review aims at presenting and summarizing the current state of literature on the presentation and surgical management of a right-sided aortic arch with a left-sided ligamentum forming a complete vascular ring around the oesophagus and trachea.
METHODS
A systematic database search for appropriate literature was conducted on PubMed/MEDLINE. Articles were considered relevant when providing details on the presentation, diagnosis and surgical treatment of this specific congenital arch anomaly in human beings.
RESULTS
Affected patients present with respiratory and/or oesophageal difficulties due to tracheoesophageal compression. Conservative treatment might be reasonable in asymptomatic or mildly symptomatic cases; however, once moderate-to-severe symptoms develop, surgical intervention is definitely indicated. Surgery is commonly performed through a left thoracotomy or median sternotomy and includes the division of the left ductal ligamentum; if a Kommerell's diverticulum is present that is >1.5 times the diameter of the subclavian artery, then concomitant resection of the large diverticulum and translocation of the aberrant left subclavian artery is also conducted. Postoperative morbidity and mortality are low and are rather related to concomitant intracardiac and extracardiac anomalies than to the procedure itself. In a majority of patients, full resolution of symptoms is seen within months to years from the surgery. Nevertheless, there is also a subset of patients who remain with some tracheobronchial narrowing, sometimes even requiring reintervention during follow-up due to persisting or recurring symptoms.
CONCLUSIONS
Overall, the surgical management of a right aortic arch forming a true vascular ring in infancy, childhood and adulthood seems relatively safe and effective in providing symptomatic relief despite some persistent tracheobronchial and/or oesophageal narrowing in some cases.
Topics: Adult; Aorta, Thoracic; Child; Diverticulum; Heart Defects, Congenital; Humans; Subclavian Artery; Vascular Ring
PubMed: 33970211
DOI: 10.1093/ejcts/ezab225 -
Thoracic Surgery Clinics Feb 2018Vascular rings and slings may represent life-threatening compression of the esophagus and trachea. Such anatomic variants, although rare, are encountered by all thoracic... (Review)
Review
Vascular rings and slings may represent life-threatening compression of the esophagus and trachea. Such anatomic variants, although rare, are encountered by all thoracic surgeons in the scope of their practice at some time. The thoracic surgeon, whether treating such patients in the practice of congenital heart surgery, or in the practice of adult cardiac or thoracic surgery must have a requisite understanding of such anatomic variants, their diagnostic workup and radiologic interpretation, as well as their surgical management. Primary tracheobronchial disorders are also frequently encountered in the scope of a thoracic surgical practice and also are discussed.
Topics: Adolescent; Bronchial Diseases; Child; Esophageal Diseases; Heart Defects, Congenital; Humans; Tracheal Diseases; Vascular Ring; Young Adult
PubMed: 29150036
DOI: 10.1016/j.thorsurg.2017.08.005 -
Digestive Diseases and Sciences Apr 2020Dysphagia lusoria is rare embryologic defect of the aortic arch vasculature characterized by an aberrant retro-esophageal course of the right subclavian artery (RSA),... (Review)
Review
Dysphagia lusoria is rare embryologic defect of the aortic arch vasculature characterized by an aberrant retro-esophageal course of the right subclavian artery (RSA), comprising a vascular sling. This may manifest clinically with symptoms of dysphagia or reflux. Given the rarity of this pathology, there remains uncertainty regarding the most effective treatment algorithm. Vascular decompression procedure surgery is often used to rectify the aberrant vascular morphology. However, a low percentage of aberrant arteries result in trachea-esophageal symptoms. As such, we suggest (1) one must exclude co-morbid esophageal motility disorders in patients with dysphagia; and (2) aberrant RSA may be a cause of dysphagia, so-called dysphagia lusoria, but represents an unlikely mechanism, since this morphological arrangement typically does not result in a highly constrictive, circumferential vascular ring. It follows, therefore, that directly addressing the esophageal motility issue should take precedence over complex and potentially noncurative vascular procedures directed to the aberrant RSA. However, the presence of marked aneursymal dilation of the aberrant RSA, and/or the presence of a bicarotid (socalled bovine) trunk, would favor vascular repair in the clinical decision-making due to the elevated risk of rupture and more severe esophageal compression in these specific situations.
Topics: Cardiovascular Abnormalities; Deglutition Disorders; Esophageal Motility Disorders; Humans; Manometry; Subclavian Artery
PubMed: 32095966
DOI: 10.1007/s10620-020-06152-2 -
Pediatrics International : Official... Feb 2017Vascular rings may cause pressure on the trachea and/or esophagus of varying degree, resulting in symptoms. This study assessed the presentation symptoms, diagnostic...
BACKGROUND
Vascular rings may cause pressure on the trachea and/or esophagus of varying degree, resulting in symptoms. This study assessed the presentation symptoms, diagnostic methods and treatment results after surgery in children with vascular ring.
METHODS
Symptomatic vascular ring patients undergoing surgery between January 2010 and August 2014 at Mehmet Akif Ersoy Cardiovascular Research and Training Hospital, Istanbul, were retrospectively assessed. The presentation symptoms, demographic characteristics, and diagnostic tests were evaluated. Operative data and postoperative follow up, complications and problems were also examined in detail.
RESULTS
Twenty-one patients underwent surgery, 13 (63%) of whom were male. Median age was 12 months (range, 1 month-8 years). Among these patients, 62% (n = 13) had double aortic arch, 24% (n = 5) had right-sided aortic arch + ligamentum arteriosum, 10% (n = 2) had left-sided aortic arch + aberrant right subclavian artery, and 4% (n = 1) had pulmonary sling abnormality. Computed tomography (CT) angiography was performed in 17/21 patients. All the patients were successfully operated on. In the early postoperative period, two patients had chylothorax and one had nerve paralysis of nervus laryngeus recurrens. One patient died in the early period, and two patients had ongoing stridor on follow up.
CONCLUSIONS
CT angiography is effective for the differential diagnosis and visualization of vascular ring abnormality. In such cases, full recovery can be assured with early diagnosis and surgery.
Topics: Aorta, Thoracic; Child; Child, Preschool; Computed Tomography Angiography; Diagnosis, Differential; Esophageal Stenosis; Female; Follow-Up Studies; Humans; Infant; Male; Postoperative Complications; Retrospective Studies; Tracheal Stenosis; Treatment Outcome; Vascular Malformations
PubMed: 27454661
DOI: 10.1111/ped.13101