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Archives of Disease in Childhood.... Aug 2021A 12-year-old girl was referred with a 7-month history of episodes of dyspnoea, stridor and a sense of chest constriction during physical exercise. These were...
A 12-year-old girl was referred with a 7-month history of episodes of dyspnoea, stridor and a sense of chest constriction during physical exercise. These were self-limiting and never occurred at night. Physical examination was normal. An initial diagnosis of vocal cord dysfunction was made.Spirometry showed a plateau of forced expiratory flow, with a truncated aspect of the expiratory phase (figure 1). Six weeks later she described occasional dysphagia with solid foods. The barium swallow, performed only in anteroposterior projection, did not show oesophageal dilation. A bronchoscopy showed extrinsic compression of the middle lower third of the trachea and the right main bronchus. A chest CT scan was performed (figures 2 and 3).
Topics: Airway Obstruction; Child; Exercise; Female; Humans; Respiratory Sounds; Spirometry; Subclavian Artery
PubMed: 31653663
DOI: 10.1136/archdischild-2019-317625 -
The Journal of Thoracic and... Jul 2022
Topics: Humans; Mitral Valve Insufficiency; Treatment Outcome; Vascular Ring; Vascular Surgical Procedures
PubMed: 34548156
DOI: 10.1016/j.jtcvs.2021.08.070 -
Magnetic Resonance Imaging Clinics of... Feb 2015Vascular rings and pulmonary artery slings are rare congenital anomalies that often present with symptoms of tracheal and esophageal compression. These can involve the... (Review)
Review
Vascular rings and pulmonary artery slings are rare congenital anomalies that often present with symptoms of tracheal and esophageal compression. These can involve the aortic arch branches and pulmonary arteries, respectively. This review illustrates the current role of MR imaging, highlights its advantages, and provides insight into the diagnosis of these anomalies by describing the embryology and characteristic imaging features of these lesions.
Topics: Aorta, Thoracic; Humans; Magnetic Resonance Angiography; Pulmonary Artery; Vascular Malformations
PubMed: 25476681
DOI: 10.1016/j.mric.2014.09.011 -
The Journal of Thoracic and... Feb 2024Cross-sectional imaging allows identification of rare patients with a vascular ring and circumflex aorta. The key diagnostic feature is crossing of the transverse aortic... (Review)
Review
OBJECTIVE
Cross-sectional imaging allows identification of rare patients with a vascular ring and circumflex aorta. The key diagnostic feature is crossing of the transverse aortic arch from right to left posterior to the trachea and superior to the carina in a patient with a right aortic arch. We evaluated our patients who received an aortic uncrossing procedure.
METHODS
We reviewed all patients who underwent aortic uncrossing from 2002 to 2022. All patients received preoperative computed tomography imaging and bronchoscopy.
RESULTS
Eleven patients ranging in age from 1.5 to 10 years (median 4 years) underwent aortic uncrossing. Two patients had prior left ligamentum division, and 3 patients had prior left aortic arch division. All had significant clinical symptoms. Eight patients had deep hypothermic circulatory arrest (mean 34 minutes), and 3 patients had antegrade cerebral perfusion (median, 28 minutes). Patch material was not used for aortic augmentation, and no patient underwent a posterior tracheopexy or rotational esophagoplasty. Postoperative length of stay ranged from 4 to 31 days (median, 5 days). One patient required a temporary tracheostomy for bilateral recurrent laryngeal nerve paresis, which recovered. One patient required an aortic extension graft to alleviate esophageal compression from an unusual ectatic esophageal course. All patients had relief of airway symptoms and dysphagia.
CONCLUSIONS
In properly selected patients with a right aortic arch and circumflex aorta, aortic uncrossing is a safe and effective therapy to treat airway and esophageal compression. The procedure can be conducted with deep hypothermic circulatory arrest or antegrade cerebral perfusion. Careful attention to the location of the esophagus and recurrent laryngeal nerves is required.
Topics: Humans; Infant; Child, Preschool; Child; Aorta; Aorta, Thoracic; Bronchoscopy; Vascular Surgical Procedures; Perfusion
PubMed: 37406764
DOI: 10.1016/j.jtcvs.2023.06.013 -
Cardiovascular Diagnosis and Therapy Apr 2018Congenital anomalies of the aortic arch include diverse subgroups of malformations that may be clinically silent or may present with severe respiratory or esophageal... (Review)
Review
Congenital anomalies of the aortic arch include diverse subgroups of malformations that may be clinically silent or may present with severe respiratory or esophageal symptoms especially when associated with complete vascular rings. These anomalies may be isolated or may be associated with other congenital heart diseases. Volume rendered computed tomography (CT) and magnetic resonance angiography (MRA) help in preoperative surgical planning by providing information about the complex relationship of aortic arch and its branches to the trachea and esophagus. Three dimensional capabilities of both computed tomography angiography (CTA) and MRA are helpful in determining evidence of tracheal or esophageal compression or other high-risk features in patients with a complete vascular ring.
PubMed: 29850417
DOI: 10.21037/cdt.2017.10.15 -
Pediatric Cardiology Aug 2023We present the clinical course and echocardiographic and genetic findings of two fetuses with an unusual vascular ring, created by a left aortic arch with a right...
We present the clinical course and echocardiographic and genetic findings of two fetuses with an unusual vascular ring, created by a left aortic arch with a right arterial duct and an aberrant right subclavian artery. One fetus was diagnosed with 22q11.2 microdeletion and the other became symptomatic in infancy. It is important to consider the position of the arterial ductal ligament in patients who present with tracheoesophageal compressive symptoms in the presence of a left aortic arch. These cases also highlight that a vascular ring formed from a left arch may have similar associations to a vascular ring formed by a right aortic arch.
PubMed: 37566242
DOI: 10.1007/s00246-023-03261-3 -
Current Cardiology Reports Oct 2022Vascular rings are congenital malformations resulting from abnormal development of the great vessels, with the consequent encircling and compression of the trachea,... (Review)
Review
PURPOSE OF REVIEW
Vascular rings are congenital malformations resulting from abnormal development of the great vessels, with the consequent encircling and compression of the trachea, esophagus, or both. We conducted a review of the current literature to identify the different management strategies that can be implemented based on the prognosis of each of these anomalies.
RECENT FINDINGS
Although most vascular rings occur in isolation, they can also be associated with other congenital cardiac and/or respiratory diseases; therefore, thorough investigation is necessary before definitive surgical repair. Clinical presentation varies from asymptomatic to severe, with both respiratory and digestive symptoms. Although early surgical results are acceptable, the long-term outcome is variable; therefore, there is still controversy regarding the appropriate timing of treatment. This is especially true with regard to the Kommerell diverticulum (KD) and in patients without symptoms at the time of initial surgical evaluation. As more sophisticated diagnostic tools have become available and more studies on adults affected by this condition have been published, understanding of this condition and its additional clinical implications has grown and appears to be tilting management toward earlier intervention.
Topics: Adult; Aorta, Thoracic; Heart Defects, Congenital; Humans; Prognosis; Vascular Ring
PubMed: 36190599
DOI: 10.1007/s11886-022-01764-8