-
World Journal For Pediatric &... Jan 2023We investigated the prevalence of isolated vascular rings in the general population of Southern Nevada.
OBJECTIVE
We investigated the prevalence of isolated vascular rings in the general population of Southern Nevada.
METHODS
We identified those prenatally and postnatally diagnosed with an isolated vascular ring between January 2014 and December 2021. We included only those with vascular or ligamentous structures completely encircling the trachea and esophagus. To investigate the prevalence of isolated vascular rings, we included only those with situs solitus, levocardia, and no significant intracardiac malformations.
RESULTS
We identified 112 patients. Of the 112, 66 (59%) were female. There were approximately 211,000 total live births in Southern Nevada for the study period, for an overall prevalence of 5.3 isolated vascular rings per 10 000 live births. However, for the years 2014 to 2017, the average prevalence figure was 3.5 per 10 000 live births, and for the years 2018 to 2021, the average prevalence figure was 7.1 (range 6.5-8.0) per 10 000 live births. Simultaneously, the prenatal detection rate rose from 66% to 86%.
CONCLUSIONS
Isolated vascular rings are common cardiovascular malformations. As prenatal detection rates in the Southern Nevada general population approach 90%, the prevalence figures for isolated vascular rings appear to asymptote at about 7 per 10 000 live births.
Topics: Pregnancy; Humans; Female; Male; Vascular Ring; Cardiovascular Abnormalities; Heart; Trachea; Nevada
PubMed: 36847763
DOI: 10.1177/21501351221122972 -
European Heart Journal. Case Reports Jun 2019Double aortic arch (DAA) and pulmonary artery sling (PAS) are vascular ring formations that present in neonates and infants with symptoms of respiratory stenosis.
BACKGROUND
Double aortic arch (DAA) and pulmonary artery sling (PAS) are vascular ring formations that present in neonates and infants with symptoms of respiratory stenosis.
CASE SUMMARY
The patient was a girl with suspected ventricular septal defect (VSD), right aortic arch (AA), left patent ductus arteriosus, and bilateral superior vena cava (SVC) on foetal echography in the first day of life. The girl was delivered at 40 weeks and 4 days of gestation. Ventricular septal defect, DAA, coarctation of the left AA, and bilateral SVC were diagnosed. Contrast-enhanced computed tomography at Day 16 revealed PAS with concurrent anomalous tracheal branching in addition to DAA. The right A2 segmental artery, which supplies the right upper pulmonary artery, showed abnormal branching from the left pulmonary artery (LPA). At 3 months of age, VSD patching, left AA resection distal to the root of the left subclavian artery, arterial ligament dissection, and LPA replacement were performed.
DISCUSSION
Pulmonary artery sling coexists with anomalous branching of the trachea and abnormal branching of the right pulmonary artery (RPA). Our patient had an extremely rare case of DAA concurrent with PAS and presented with anomalous tracheal and RPA branching. We were concerned that increased pulmonary blood flow caused by the VSD would exacerbate tracheal displacement. Radical surgery at 3 months of age resulted in good postoperative progress.
PubMed: 31449592
DOI: 10.1093/ehjcr/ytz036 -
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 -
JIMD Reports Nov 2020Congenital disorders of glycosylation (CDG) are a group of metabolic disorders well known to be associated with developmental delay and central nervous system anomalies....
BACKGROUND
Congenital disorders of glycosylation (CDG) are a group of metabolic disorders well known to be associated with developmental delay and central nervous system anomalies. The most common CDG is caused by pathogenic variants in the phosphomannomutase 2 gene (), which impairs one of the first steps of N-glycosylation and affects multiple organ systems. Cardiac involvement can include pericardial effusion, cardiomyopathy, and arrhythmia, while an association with cardiovascular congenital anomalies is not well studied.
CASE SUMMARY
We report a 6-year-old individual who initially presented with inverted nipples, developmental delay, and failure to thrive at 3 months of age. At 4 months, due to feeding problems, swallowing exam and echocardiography were performed which revealed a vascular ring anomaly based on a right aortic arch and aberrant left subclavian artery. Subsequent whole exome gene sequencing revealed two pathogenic PMM2-CDG variants (E139K/R141H) and no known pathogenic mutations related to congenital heart defect (CHD).
DISCUSSION
This is the first report of vascular ring anomaly in a patient with PMM2-CDG. We conducted a literature review of PMM2-CDG patients with reported CHD. Of the 14 patients with PMM2-CDG and cardiac malformation, the most common CHD's were tetralogy of Fallot, patent ductus arteriosus, and truncus arteriosus. The potential important link between CDG and CHD is stressed and discussed. Furthermore, the importance of multidisciplinary care for CDG patients including early referral to pediatric cardiologists is highlighted.
PubMed: 33204593
DOI: 10.1002/jmd2.12160 -
Wisconsin Medical Journal Mar 1963
Topics: Aortic Arch Syndromes; Aortic Diseases; Heart Defects, Congenital; Humans; Pulmonary Valve Insufficiency; Respiratory Insufficiency
PubMed: 13950378
DOI: No ID Found -
Asian Journal of Surgery Nov 2022
Topics: Foot; Hand; Humans; Lower Extremity; Upper Extremity; Vascular Ring
PubMed: 35715294
DOI: 10.1016/j.asjsur.2022.05.098 -
The Pan African Medical Journal 2023Anomalies of the arterial branches of the arch of the aorta are rare, with the aberrant right subclavian artery being the most common of this anomaly. Majority of the...
Anomalies of the arterial branches of the arch of the aorta are rare, with the aberrant right subclavian artery being the most common of this anomaly. Majority of the anomalies are asymptomatic and often discovered as incidental findings. In the great majority of the symptomatic cases, the presentation may be either with breathlessness or dysphagia or both. This is in addition to the nature of the intrinsic arterial disease of the aberrant vessel, especially in adult patients; and unless borne in mind, the diagnosis is often missed leading to delays and wrong treatment. In this report we present a case of dysphagia in an adult male Nigerian initially diagnosed as œsophageal stricture from herbal potion ingestion but review of his imaging investigations gave a final diagnosis of dysphagia lusoria from an aberrant right subclavian artery. The difficulty in making a diagnosis and the need for a multidisciplinary review of the imaging investigations are highlighted. The patient was successfully treated by a combined trans-thoracic and cervical approach with division and re-implantation of the aberrant vessel unto the right common carotid artery. He has remained symptom-free for 2 years after surgery. Although the great majority of these anomalies are often asymptomatic, it is important they are borne in mind both in imaging investigations as well as in procedures involving structures in the upper visceral mediastinum. Various surgical approaches have been documented in the management of symptomatic ones; it is however recommended that options that ensure revascularization of the affected limb be selected.
Topics: Humans; Male; Adult; Deglutition Disorders; Aorta, Thoracic; Vascular Ring; Cardiovascular Abnormalities; Subclavian Artery
PubMed: 38020356
DOI: 10.11604/pamj.2023.45.183.38569 -
Seminars in Thoracic and Cardiovascular... 2020Our series of vascular ring patients treated at Ann & Robert H. Lurie Children's Hospital of Chicago has now exceeded 450 patients (see Central Picture). The...
Our series of vascular ring patients treated at Ann & Robert H. Lurie Children's Hospital of Chicago has now exceeded 450 patients (see Central Picture). The recommendations from this review are based on our experience with those patients. Since 1990, we have used preoperative cross-sectional imaging in all vascular ring patients. This has led us to refine our operative strategies and carefully tailor the operation to the individual's unique anatomy. In particular for right aortic arch patients, we now address the Kommerell diverticulum in most cases and carefully consider Kommerell diverticulum resection with left subclavian artery transfer. In double aortic arch patients, we also look for a Kommerell diverticulum and resect the diverticulum if present. This will decrease the number of patients presenting for a reoperation after initial vascular ring surgery. Since 1947, we have operated on 217 patients with a right aortic arch. The median age is 1.5 years. Since 2006, 47 out of 111 patients have had Kommerell diverticulum resection and left subclavian artery transfer to the left carotid artery. Mean length of stay is 4.5 days. In the same time period, 187 patients have been treated for a double aortic arch. The median age is 1 year. Twelve of these patients also had resection of a Kommerell diverticulum. The mean length of stay in this group was 3 days. Advanced preoperative cross-sectional imaging leads to precise operative techniques tailored to the unique anatomy of each vascular ring patient.
Topics: Aorta, Thoracic; Deglutition Disorders; Diverticulum; Esophageal Stenosis; Female; Heart Defects, Congenital; Humans; Infant; Male; Tomography, X-Ray Computed; Tracheal Stenosis; Vascular Malformations; Vascular Ring; Vascular Surgical Procedures
PubMed: 32354547
DOI: 10.1053/j.pcsu.2020.02.004 -
Asian Cardiovascular & Thoracic Annals Feb 2022Vascular rings are aortic arch anomalies with a spectrum of manifestations ranging from asymptomatic lesions detected incidentally to an acute presentation secondary to... (Review)
Review
BACKGROUND
Vascular rings are aortic arch anomalies with a spectrum of manifestations ranging from asymptomatic lesions detected incidentally to an acute presentation secondary to tracheo-esophageal compression. Circumflex retro-esophageal aortic arch is an extreme form of true vascular ring. It remains an uncharted territory to many surgeons.
METHODS
A comprehensive search of peer reviewed journals was completed based on the key words, "Circumflex aorta," "Circumflex retro-esophageal aorta" and "circumflex arch" using Google scholar, Scholars Portal Journals and PubMed. The reference section for each article found was searched to obtain additional articles. Literature on the circumflex aorta was reviewed starting from the embryogenesis to the latest management strategies.
RESULTS
Right circumflex aorta is more prevalent compared to left circumflex aorta. It can occur in isolation or in association with other intracardiac lesions. Mainly presents in children, however reported in adults too. The presentation may vary from asymptomatic lesion to acute respiratory distress secondary to airway compression. Computerized tomography (CT) and magnetic resonance imaging (MRI) are important tools in delineating the vascular anatomy. Aortic uncrossing is the definitive procedure. However, the role of concomitant tracheobronchopexy is emerging. Native tissue-to-tissue anastomosis is commonly preferred, but cases of extra-anatomic grafts are reported.
CONCLUSION
Circumflex aorta is amenable to complete repair. Preoperative delineation of anatomy is important for successful surgical outcome. Division of the retro-esophageal segment is crucial in relieving the compressive symptoms. In addition, tracheobronchopexy is helpful in addressing residual tracheomalacia but this accounts for a high-risk surgery.
Topics: Adult; Aorta; Aorta, Thoracic; Child; Heart Defects, Congenital; Humans; Trachea; Treatment Outcome; Vascular Ring
PubMed: 33957784
DOI: 10.1177/02184923211015092 -
Association Between Airway Stenosis Degree and Respiratory Distress in Infants With a Vascular Ring.Cureus Oct 2023Background Although the number of cases of prenatally diagnosed vascular rings is increasing, some cases may remain asymptomatic, and no indicator of the appearance of...
Background Although the number of cases of prenatally diagnosed vascular rings is increasing, some cases may remain asymptomatic, and no indicator of the appearance of dyspnea has been established. Thus, we aimed to determine the relationship between the degree of airway compression by the vascular ring on contrast-enhanced computed tomography (CT) and respiratory distress. Methods This is a retrospective study of nine patients diagnosed with vascular rings at a single hospital from July 2010 to December 2019. Data regarding the patient's clinical characteristics, such as prenatal diagnosis, vascular ring type, complicated cardiac disease, and presence or absence of surgery, were recorded. Airway assessment on contrast-enhanced CT was measured in the axial cross-section. Statistical analysis was performed using Statistical Product and Service Solutions (SPSS) (version 25.0; IBM SPSS Statistics for Windows, Armonk, NY). Results Five of the eight patients had respiratory distress. Patients with respiratory distress were less likely to have been diagnosed prenatally (p = 0.04) and had smaller stenosis degree of anteroposterior diameter (p = 0.03). Conclusion Contrast-enhanced CT is useful in patients with vascular rings. Our study suggests that the stenosis degree of the anterior-posterior diameter of the airway is related to dyspnea.
PubMed: 37965390
DOI: 10.7759/cureus.47022