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The Indian Journal of Radiology &... Dec 2022Thoracic vascular malformations are a set of complex congenital abnormalities that result in major alterations of the patient's anatomy. It can be discovered...
Thoracic vascular malformations are a set of complex congenital abnormalities that result in major alterations of the patient's anatomy. It can be discovered incidentally or the anomalies may generate heart failure with a right-to-left shunt, dysphagia, dyspnea, stridor, cough, recurrent bronchopulmonary infections, hypoxemia, paradoxical emboli, and occasionally apneas and massive hemothorax. The knowledge of the embryology, and the normal development of the vascular structures of the thorax, allows to understand these abnormalities-and thus helps in making an accurate diagnosis-with its possible complications, symptoms, and treatments for the patient. The following is a review of the most common thoracic vascular malformations and their imaging findings.
PubMed: 36451940
DOI: 10.1055/s-0042-1757742 -
Archives of Disease in Childhood Oct 2020This study aimed to explore the anatomical features of aortic arch anomalies associated with vascular rings, hoping to identify those which may increase the risk of...
OBJECTIVE
This study aimed to explore the anatomical features of aortic arch anomalies associated with vascular rings, hoping to identify those which may increase the risk of symptomatic presentation and surgical intervention.
METHODS
This was a retrospective observational study at a single cardiac unit. Individuals diagnosed with an aortic arch anomaly, either isolated or non-isolated, between June 2014 and September 2018 were included. The morphology of the aortic arch was established via analysis of postnatal echocardiography, CT or MRI scans. CT and magnetic resonance studies were evaluated for the presence of a Kommerell diverticulum in those with aberrant vessels. Case notes were reviewed for relevant clinical data.
RESULTS
Of those with aberrant subclavian arteries, 24/79 (30.4%) were shown to have a Kommerell diverticulum. Additional forms of congenital heart disease were present in 133/227 (58.6%) individuals. Surgical division of the vascular ring was performed in 30/227 (13.2%), most commonly in the setting of a double aortic arch (70.8%). In those with aberrant subclavian arteries, no children without a Kommerell diverticulum were referred for surgery. In those with a Kommerell diverticulum confirmed on imaging, 11/24 underwent surgery.
CONCLUSION
Individuals with a double aortic arch, or an aberrant subclavian artery arising from a Kommerell diverticulum, have the highest requirement for surgical intervention, especially in isolated anomalies. These individuals should remain under monitoring. The subjective nature of symptoms remains problematic. Longitudinal research is required further to understand the natural history of vascular rings and how it links to morphology.
PubMed: 33106229
DOI: 10.1136/archdischild-2020-319388 -
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 -
American Journal of Otolaryngology 2024Vascular rings are often associated with respiratory and swallowing difficulties due to tracheal or esophageal compression. While the results of a vascular ring repair... (Review)
Review
OBJECTIVE
Vascular rings are often associated with respiratory and swallowing difficulties due to tracheal or esophageal compression. While the results of a vascular ring repair are considered excellent, the long-term effect of tracheal and esophageal remodeling and the persistence of symptoms have scarcely been reported. Our study aims to evaluate the respiratory and swallowing outcomes of vascular ring repair.
STUDY DESIGN AND METHODS
A retrospective cohort study of children who underwent vascular ring repair between 2010 and 2022 in a tertiary-care children's hospital.
RESULTS
There were 108 patients enrolled: sixty-three patients (57.41 %) with a right aortic arch, 42 patients (38.89 %) with a double aortic arch, and 3 patients (2.78 %) with other vascular rings. Forty-three (39.81 %) patients were diagnosed prenatally. Of the 65 patients (60.19 %) diagnosed postnatally, 35/65 (53.85 %) had either respiratory or swallowing symptoms as the indication for diagnostic workup. Persistent respiratory and swallowing symptoms were noted in 34/108 (31.48 %) and 30/108 (27.78 %) patients, respectively, within a year of surgical repair. Fourteen patients underwent repeated laryngoscopy and bronchoscopy that demonstrated residual tracheomalacia; however, only 2/14 (1.9 %) patients required tracheostomy tube placement, and 6-out-of-7 patients were weaned off positive pressure airway support. Persistent respiratory symptoms were significantly more common in patients with a double aortic arch compared to a right aortic arch. No differences were noted in demographics, comorbidities, and preoperative aerodigestive symptoms between patients with residual symptoms and patients with no residual symptoms.
CONCLUSIONS
Persistent respiratory and swallowing symptoms after vascular ring repair are not uncommon. Postoperative evaluation should be pursued by a dedicated team, and treatment considered as appropriate.
Topics: Child; Humans; Infant; Vascular Ring; Aorta, Thoracic; Retrospective Studies; Trachea; Vascular Surgical Procedures
PubMed: 38101126
DOI: 10.1016/j.amjoto.2023.104147 -
European Journal of Cardio-thoracic... May 2022Our goal was to describe postoperative complications and outcomes in a large contemporary cohort of children with an isolated double aortic arch (DAA) or a right aortic...
OBJECTIVES
Our goal was to describe postoperative complications and outcomes in a large contemporary cohort of children with an isolated double aortic arch (DAA) or a right aortic arch (RAA) with left arterial ligament and to assess the impact of foetal diagnosis on outcomes.
METHODS
We performed a retrospective analysis of all patients who underwent surgery for DAA or RAA with left arterial ligament between 2005 and 2019.
RESULTS
A total of 132 children were operated on for a DAA (n = 77) or a RAA (n = 55). Prenatal diagnosis was made in 100/132 (75.8%). Median age at surgery for DAA was 5.0 (1.7-13.3) months and for RAA was 13.9 (6.4-20.1) months. There was no difference in the age at surgery between the prenatal and postnatal cases (8.6 [4.0-15.6] vs 5.4 months [1.8-17.7]; P = 0.37). No surgical deaths occurred. Vocal cord palsy was the most common complication, occurring in 12/132 (9%): of these, 11 resolved spontaneously and 1 required a temporary tracheostomy. Logistic regression demonstrated that older age at operation was the only predictor for a postoperative complication (P = 0.02). Overall, 21/67 (31%) of prenatally detected, symptomatic cases reported residual symptoms/signs 1 year after surgery compared to 18/28 (64%) of postnatally detected cases. Postnatal diagnosis was associated with persistent postoperative symptoms/signs [P = 0.006, odds ratio = 3.9 (95% confidence interval 1.5-9.4)].
CONCLUSIONS
Surgery to relieve a vascular ring resolves trache-oesophageal compressive symptoms in most cases, but parents/patients should be aware that symptoms/signs may persist in the first postoperative year despite effective release of the vascular ring. Earlier surgery and prenatal diagnosis may improve outcomes.
Topics: Aorta, Thoracic; Aortic Arch Syndromes; Child; Female; Humans; Infant; Pregnancy; Prenatal Diagnosis; Retrospective Studies; Treatment Outcome; Ultrasonography, Prenatal; Vascular Ring
PubMed: 35022705
DOI: 10.1093/ejcts/ezab527 -
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 -
Pediatric Pulmonology Aug 2015Our aim is to compare new non-invasive imaging modalities in the evaluation of vascular ring (VR) and pulmonary artery sling (PAS) and to understand the role of...
PURPOSE
Our aim is to compare new non-invasive imaging modalities in the evaluation of vascular ring (VR) and pulmonary artery sling (PAS) and to understand the role of bronchoscopy in comparison with them in assessing tracheobronchial tree.
METHODS
We have retrospectively analyzed the data from 41 patients with a VR or a PAS diagnosed at Bambino Gesù Children's Hospital of Rome, between 2008 and 2012. Age, gender, presenting symptoms, clinical history, comorbidities, imaging modalities used for diagnosis (cardiac magnetic resonance [CMR], computed tomography [CT], tracheobronchoscopy [TB]) and surgical treatment were recorded.
RESULTS
The vascular anatomy was completely defined in all patients, whether evaluated by CMR or CT, with a diagnostic accuracy of 100% based on surgical observation. All CT exams were performed without sedation with a mean dose-length product (DLP32 ) of 29 ± 9 and an effective dose of 1.56 ± 0.6 mSv, range 0.5-2.5 mSv. CMR required general anesthesia in all patients but involved no exposure to ionizing radiation. CT performed better than CMR in assessing tracheal stenosis when compared to TB. It detected complete tracheal cartilage rings in 2/3 patients with PAS, besides tracheomalacia and/or bronchomalacia in 54% of patients.
CONCLUSIONS
Both cross-sectional imaging modalities (CT and CMR) can reliably and accurately diagnose these congenital vascular anomalies. While CT involves exposure to ionizing radiation, it avoids the risks related to anesthesia needed for CMR, and provides a more accurate assessment of tracheobronchial anatomy. TB remains a fundamental tool in tracheomalacia diagnosis in VR symptomatic patients and PAS.
Topics: Bronchoscopy; Child, Preschool; Female; Humans; Infant; Infant, Newborn; Magnetic Resonance Imaging, Cine; Male; Pulmonary Artery; Retrospective Studies; Tomography, X-Ray Computed; Vascular Malformations
PubMed: 24979312
DOI: 10.1002/ppul.23075 -
Molecular and Cellular Biochemistry Sep 2023Monoamine oxidases (MAOs), mitochondrial enzymes with two isoforms, A and B, have been recently recognized as significant contributors to oxidative stress in the...
Monoamine oxidases (MAOs), mitochondrial enzymes with two isoforms, A and B, have been recently recognized as significant contributors to oxidative stress in the cardiovascular system. The present study was purported to assess the effect of metformin and empagliflozin on MAO expression, oxidative stress and vascular reactivity in internal mammary arteries harvested from overweight patients with coronary heart disease subjected to bypass grafting. Vascular rings were prepared and acutely incubated (12 h) with high glucose (GLUC, 400 mg/dL) or angiotensin II (AII, 100 nM) and metformin (10 µM) and/or empagliflozin (10 µM) and used for the assessment of MAO expression (qRT-PCR and immune histochemistry), reactive oxygen species (ROS, confocal microscopy and spectrophotometry), and vasomotor function (myograph). Ex vivo stimulation with GLUC or AII increased both MAOs expression, ROS production and impaired relaxation to acetylcholine (ACh) of the vascular rings. All effects were alleviated by incubation with each antidiabetic drug; no cumulative effect was obtained when the drugs were applied together. In conclusion, MAO-A and B are upregulated in mammary arteries after acute stimulation with GLUC and AII. Endothelial dysfunction and oxidative stress were alleviated by either metformin or empagliflozin in both stimulated and non-stimulated vascular samples harvested from overweight cardiac patients.
Topics: Humans; Reactive Oxygen Species; Mammary Arteries; Metformin; Overweight; Vascular Ring; Oxidative Stress; Monoamine Oxidase
PubMed: 36583793
DOI: 10.1007/s11010-022-04633-8 -
Cardiology in the Young Jul 2022
Topics: Humans; Retrospective Studies; Tracheal Stenosis; Vascular Ring
PubMed: 35581929
DOI: 10.1017/S1047951122000816 -
Journal of Pediatric Surgery Nov 2022Tracheobronchomalacia (TBM), a condition where an abnormality of the tracheal walls causes collapse during the respiratory cycle, is a common cause of airway obstruction...
OBJECTIVES
Tracheobronchomalacia (TBM), a condition where an abnormality of the tracheal walls causes collapse during the respiratory cycle, is a common cause of airway obstruction in childhood. TBM can present with a large spectrum of disease severity and underlying pathologies that may be managed medically and surgically, and it is not always clear which patients would most benefit from surgical intervention. We aim to describe the incidence, patient characteristics, and predictors of surgical intervention in a large cohort of paediatric patients.
METHODS
We performed a retrospective review of all children diagnosed with TBM to a paediatric Otolaryngology unit in the west of Scotland between 2010 and 2020. Odds ratios for clinical predictors of surgery were calculated using logistic regression with uni- and multivariate analysis.
RESULTS
249 patients were identified of which 219 proceeded to data collection. Primary malacia was noted in 161 (73.5%) and secondary in 58 (26.5%). Causes of secondary malacia included compression by the innominate artery (11%) and vascular rings (7.8%). Surgical interventions were performed in 28 patients (12.8%) including division of vascular ring, aortopexy, and surgical tracheostomy. Multivariate analysis showed secondary TBM, acute life-threatening events, and difficulty weaning from mechanical ventilation were independent risk factors for surgical intervention.
CONCLUSIONS
TBM can present with a myriad of airway symptoms and is frequently associated with other airway and mediastinal pathologies necessitating multiple interventions. Children aged <1 year present with a more severe form of the disease and the presence of particular independent risk factors may indicate a need for surgical intervention.
Topics: Airway Obstruction; Child; Humans; Incidence; Retrospective Studies; Trachea; Tracheobronchomalacia; Vascular Ring
PubMed: 35718546
DOI: 10.1016/j.jpedsurg.2022.05.005