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Tomography (Ann Arbor, Mich.) Jun 2018Prenatal ultrasonography in the early third trimester showed an unusual branching pattern of the right aortic arch. Echocardiography performed 4 h after birth showed the...
Prenatal ultrasonography in the early third trimester showed an unusual branching pattern of the right aortic arch. Echocardiography performed 4 h after birth showed the right aortic arch with mirror-image branching, patent ductus arteriosus, and patent foramen ovale. Because the location of the ductus arteriosus was unclear on echocardiography, cardiovascular magnetic resonance imaging was performed 3 days after birth. Advanced techniques including contrast-enhanced time-resolved magnetic resonance angiography and 3D time-of-flight magnetic resonance angiography allowed accurate diagnosis of a vascular ring comprising ascending and descending aorta, right aortic arch with mirror-image branching, and diverticulum of Kommerell giving rise to a left ligamentum arteriosum. The infant had hiccups, but no other symptoms. The esophagram was negative for obstruction. The infant was closely monitored; however, she developed esophageal obstruction at 7 months of age because of the vascular ring. She underwent lysis of the left ligamentum arteriosum followed by aortopexy for relief of esophageal obstruction. This report shows the utility of cardiovascular magnetic resonance imaging to evaluate complex congenital aortic arch anomalies.
PubMed: 30206543
DOI: 10.18383/j.tom.2018.00015 -
Pediatric Pulmonology Dec 2019The aim of this study is to document the long-term outcome of patients with a vascular ring.
BACKGROUND AND OBJECTIVES
The aim of this study is to document the long-term outcome of patients with a vascular ring.
METHODS
A single-center retrospective review of clinical symptoms was conducted in all patients born between 1980 and 2013, diagnosed with a complete vascular ring and at least 2 years of follow-up. Data were extracted from patient files and clinical symptoms were assessed by questionnaires sent to the parents. Age and diagnostic tools, type of surgery, postoperative complications, and the prevalence of clinical symptoms (stridor at rest, stridor with exercise, cough, exercise intolerance, dysphagia, and frequent respiratory infections) were reviewed.
RESULTS
Fifty-one patients were included. The diagnosis was made before the age of 2 in 35/51. Surgery was performed in 41/51 patients with a limited number of reversible complications. The median follow-up was 8 (IQR 5-12) years. After 2 years, 21/51 patients were asymptomatic: 2/7 asymptomatic patients at diagnosis became symptomatic and symptoms resolved in 16/44 initially symptomatic patients. Consequently, 30/51 patients still had symptoms after 2 years. Of these, 16 had partial improvement, 5 stable symptoms, and 9 aggravation of symptoms. After 10 years, 26/36 patients were free of complaints. The most frequent long-term symptom was stridor.
CONCLUSION
Surgical treatment of a congenital vascular ring is safe and mostly performed in patients with a double aortic arch. Conservative treatment for patients with little symptoms seems to be justifiable. A considerable number of patients have residual long-term symptoms after surgical relief of the vascular ring.
Topics: Aorta, Thoracic; Child; Child, Preschool; Conservative Treatment; Cough; Deglutition Disorders; Exercise; Female; Humans; Male; Parents; Postoperative Complications; Respiratory Sounds; Retrospective Studies; Surveys and Questionnaires; Vascular Ring
PubMed: 31456344
DOI: 10.1002/ppul.24490 -
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 -
Journal of the American College of... Mar 1995This study evaluated our early experience with video-assisted thoracoscopic vascular ring division and compared this approach with division by means of a conventional... (Clinical Trial)
Clinical Trial Comparative Study
OBJECTIVES
This study evaluated our early experience with video-assisted thoracoscopic vascular ring division and compared this approach with division by means of a conventional open thoracotomy.
BACKGROUND
Video-assisted thoracoscopic techniques reduce surgical trauma and have been applied to several adult thoracic procedures; however, pediatric applications have been limited. We developed instruments and techniques for video-assisted thoracoscopic vascular ring division in the pediatric population.
METHODS
We compared patient characteristics, operative results and postoperative hospital courses of all patients undergoing vascular ring division by a video-assisted approach with a historical control group of all patients undergoing division by an open thoracotomy between January 1991 and December 1992.
RESULTS
Eight patients (median age 5 months, range 40 days to 5.5 years; median weight 6.2 kg, range 1.8 to 17.1) underwent video-assisted thoracoscopic vascular ring division. Four had a double aortic arch with an atretic left arch and a left ligamentum, and four had a right aortic arch with aberrant left subclavian artery and a left ligamentum. All eight had successful ring division with symptomatic relief and no mortality. A limited thoracotomy was performed in three patients to divide patent vascular structures, and the hospital period was prolonged in one because of chylothorax. These eight patients were compared with a historical cohort of eight pediatric patients having vascular ring division performed by a conventional thoracotomy. The two groups did not differ in age, weight, intensive care unit or postoperative hospital stay, duration of intubation or thoracostomy tube or hospital charges. Total operating room time was longer for the group undergoing video-assisted operation.
CONCLUSIONS
Early results for video-assisted thoracoscopic vascular ring division are comparable to those of the conventional surgical approach. With further refinement in technique and instrumentation, video-assisted surgical intervention may become a viable alternative to open thoracotomy for management of the symptomatic vascular ring.
Topics: Aorta, Thoracic; Child, Preschool; Female; Follow-Up Studies; Humans; Infant; Male; Thoracoscopy; Thoracotomy; Vascular Surgical Procedures; Video Recording
PubMed: 7884102
DOI: 10.1016/0735-1097(94)00461-X -
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 -
Pediatric Radiology Dec 2015Vascular rings can be challenging to diagnose because they can contain atretic portions not detectable with current imaging modalities. In these cases, where the... (Review)
Review
Vascular rings can be challenging to diagnose because they can contain atretic portions not detectable with current imaging modalities. In these cases, where the compressed airway and esophagus are not encircled by patent, opacified vessels, there are useful secondary signs that should be considered and should raise suspicion for the presence of a vascular ring. These signs include a double aortic arch, the four-vessel sign, the distorted subclavian artery sign, a diverticulum of Kommerell, a ductal diverticulum contralateral to the aortic arch, and a descending aorta contralateral to the arch or circumflex aorta. If none of these findings is present, a ring can be excluded with confidence.
Topics: Aorta, Thoracic; Aortic Arch Syndromes; Diagnosis, Differential; Diagnostic Imaging; Humans; Infant, Newborn; Subclavian Artery; Vascular Malformations
PubMed: 26260202
DOI: 10.1007/s00247-015-3424-7 -
World Journal For Pediatric &... Jan 2023
Topics: Humans; Vascular Ring; Aorta, Thoracic
PubMed: 36475407
DOI: 10.1177/21501351221140295 -
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 -
Journal of the Medical Association of... May 2015Vascular ring is a rare congenital vascular disease. The result of vascular ring surgery in Thailand remains unknown as it lacks serial data. The purpose of the present...
BACKGROUND
Vascular ring is a rare congenital vascular disease. The result of vascular ring surgery in Thailand remains unknown as it lacks serial data. The purpose of the present study was to evaluate the result of vascular ring surgery at Siriraj Hospital and demonstrate the usefulness of the computed tomograph angiographic scanning (CTA) for preoperative anatomical diagnosis of the vascular ring for planning the surgical correction.
MATERIAL AND METHOD
We reviewed our experiences for all patients that underwent vascular rings surgery at our institute between 1979 and 2009, about preoperative diagnostic imaging, operative technique, and clinical outcome.
RESULTS
Twelve medical records of patients that underwent vascular rings surgery were reviewed. The age at time of operation ranged from one month to two years (median 3 months). The surgical approaches were eight median sternotomies, three left thoracotomies, and one right thoracotomy. There were two cases ofpostoperative complication (residual tracheal and left pulmonary artery stenosis and surgical bleeding). There was one hospital mortality (pneumonia and sepsis).
CONCLUSION
Surgical correction of vascular ring is effective and safe. CTA can give the precise anatomic diagnosis of the vascular rings, lead to good surgical planning, proper surgical approach, and good outcome.
Topics: Adult; Aged; Angiography; Aorta, Thoracic; Child, Preschool; Female; Heart Defects, Congenital; Humans; Infant; Male; Middle Aged; Pulmonary Artery; Tertiary Care Centers; Thailand; Tomography, X-Ray Computed; Tracheal Stenosis; Vascular Diseases; Vascular Surgical Procedures
PubMed: 26058276
DOI: No ID Found -
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