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Cureus Dec 2023Even though cardiac computed tomography and magnetic resonance imaging are the gold standard for evaluating the aortic arch in the context of vascular rings in children,... (Review)
Review
Even though cardiac computed tomography and magnetic resonance imaging are the gold standard for evaluating the aortic arch in the context of vascular rings in children, echocardiography is usually the first-line modality. The echocardiographic evaluation of the aortic arch in the context of vascular rings in children has received little attention. This article details the step-by-step echocardiographic assessment of the aortic arch in vascular ring patients.
PubMed: 38249193
DOI: 10.7759/cureus.50899 -
Journal of the American Society of... Mar 2022The impact of fetal echocardiography on the diagnosis and outcomes of vascular ring has not been well examined. We hypothesized that prenatal detection of vascular ring...
BACKGROUND
The impact of fetal echocardiography on the diagnosis and outcomes of vascular ring has not been well examined. We hypothesized that prenatal detection of vascular ring has improved over time and that prenatal diagnosis of vascular ring is associated with earlier intervention and favorable outcomes.
METHODS
This is a single-center, retrospective study of the evolution and outcomes of prenatal diagnosis of vascular ring from 2000 to 2020. We compared clinical presentation, timing of surgical intervention, and outcomes between the prenatally and postnatally diagnosed cases during the same study period.
RESULTS
A total of 170 patients were included: 50 with prenatal and 120 with postnatal diagnosis of vascular ring. Prenatal diagnoses included 42 patients (84%) with right aortic arch (RAA), aberrant left subclavian artery (ALSCA), and a left-sided ductus arteriosus and eight (16%) patients with double aortic arch (DAA). The postnatal cohort consisted mainly of 90 patients (75%) with DAA and 22 (18%) with RAA-ALSCA. None of the postnatally diagnosed cases had undergone a fetal echocardiogram. Numbers (percentage) of prenatally diagnosed cases of vascular ring compared with the postnatal cases improved from 4/31 (13%), to 10/29 (34%), to 14/25 (56%), and to 22/35 (69%), respectively, during 2000-2005, 2005-10, 2010-15, and 2015-20 (P = .032). Vascular ring was an isolated abnormality in 84% and 85% of the prenatal and postnatal cohorts, respectively. Compared with the prenatal cohort, postnatally diagnosed patients with an isolated vascular ring were more frequently symptomatic (66% vs 48%, P < .03) and underwent cross-sectional imaging (69% vs 44%, P = .009) and surgery more frequently (79% vs 48%, P = .003). Surgery was performed at a later patient age (18 [2-147] months vs 4.8 [0.5-42] months, P = .01) and was more often associated with residual symptoms (27/81 [33%] vs 1/20 [5%], P = .01) in the postnatal cohort than in the prenatal cohort.
CONCLUSIONS
The diagnosis of vascular ring by fetal echocardiography has improved over time. A significantly higher incidence of RAA-ALSCA in the prenatal compared with the postnatal cohort suggests that patients with this form of vascular ring often do not present to medical attention with significant symptoms postnatally. Prenatal diagnosis of vascular ring was associated with a lower incidence of symptoms, less frequent use of cross-sectional imaging, earlier age at surgical intervention, and lower likelihood of residual symptoms.
Topics: Aorta, Thoracic; Female; Humans; Infant; Pregnancy; Prenatal Diagnosis; Retrospective Studies; Ultrasonography, Prenatal; Vascular Ring
PubMed: 34600045
DOI: 10.1016/j.echo.2021.09.010 -
Pediatric Pulmonology Dec 2022Vascular rings are congenital anomalies of the aortic arch that compress the trachea and esophagus and may require corrective surgery. Data about the long-term effects...
BACKGROUND
Vascular rings are congenital anomalies of the aortic arch that compress the trachea and esophagus and may require corrective surgery. Data about the long-term effects of vascular rings are scarce. We aimed to evaluate the long-term cardiorespiratory, exercise capacity, and quality of life of vascular ring patients.
METHODS
A single center prospective study evaluating spirometry, echocardiography, six-minute walk test (6MWT), cardiopulmonary exercise testing (CPET), and quality of life questionnaire (SF36) in patients with a diagnosis of vascular ring, with or without corrective surgery.
RESULTS
Twenty-seven patients participated (11.9 ± 6 years, 52% males). The most common diagnosis was double aortic arch (16 patients, 59%). Nineteen patients had corrective surgery (O) and 8 did not (NO). Pulmonary function tests were within normal range in both groups (FEV % predicted O = 87.6 ± 16.5, NO = 83 ± 10.8%). However, 11/27 had abnormal FEV , 5 had abnormal FVC, and 13 (48%) had flattening of the expiratory curve. 6MWD and oxygen uptake were similarly mildly reduced in both groups; (6MWD O = 80.1 ± 10.7% predicted, NO = 74.1 ± 10.9% and oxygen uptake O = 78.5 ± 23.2% predicted, NO = 73.4 ± 14.3%). Peak O pulse (V̇O /HR% predicted) was mildly reduced in the NO group (O = 88.4 ± 17.3%, NO = 75.8 ± 16.2%). Echocardiogram and SF36 scores were normal in all patients.
CONCLUSIONS
Long-term evaluation of patients born with vascular rings revealed mild pulmonary impairment, reduction in 6MWD, and oxygen uptake. The NO group had also mild reduced peak O pulse. Larger, long-term studies assessing functional parameters in operated and non-operated patients are needed to assess disease/surgery limitation in patients with vascular rings. Clinical trial registration number: NCT04781738.
Topics: Male; Humans; Female; Vascular Ring; Quality of Life; Exercise Tolerance; Forced Expiratory Volume; Prospective Studies; Exercise Test; Oxygen; Oxygen Consumption
PubMed: 35971243
DOI: 10.1002/ppul.26112 -
European Journal of Cardio-thoracic... Jun 2022
Topics: Aorta, Thoracic; Decision Trees; Diverticulum; Humans; Subclavian Artery; Vascular Ring
PubMed: 34894130
DOI: 10.1093/ejcts/ezab525 -
Cureus Apr 2022Vascular rings are congenital abnormalities of the aortic arch vascular system that compress the trachea and esophagus. A review of long-term outcomes suggests that... (Review)
Review
Vascular rings are congenital abnormalities of the aortic arch vascular system that compress the trachea and esophagus. A review of long-term outcomes suggests that chronic feeding difficulties can persist following surgical repair of vascular rings. Previous reports of postoperative vascular ring division outcomes indicate that chronic esophageal symptoms may persist following repair, though most available data focuses on persistent respiratory symptoms. It is therefore the aim of this article to summarize and organize recent evidence reporting the frequency, presentation, and management of feeding difficulties following vascular ring repair in pediatric patients. Pathophysiologic mechanisms for postoperative esophageal symptoms may include residual compression from an unresected diverticulum of Kommerell or delayed repair leading to chronic esophageal dysmotility despite correction of esophageal compression. Guidance on the management of feeding difficulties following vascular ring repair is limited. The authors describe success in one case with nasogastric tube feeding and interdisciplinary evaluation. Consensus regarding the management of feeding difficulty following vascular ring repair is needed.
PubMed: 35651405
DOI: 10.7759/cureus.24623 -
BMC Pregnancy and Childbirth May 2023In terms of embryonic origin, vascular ring is a congenital anomaly in which the aortic arch and its branches completely or incompletely encircle and compress the...
BACKGROUND
In terms of embryonic origin, vascular ring is a congenital anomaly in which the aortic arch and its branches completely or incompletely encircle and compress the trachea or esophagus. Early and accurate diagnosis of a vascular ring is the key to treatment. Prenatal diagnosis mainly relies on fetal echocardiography, but the rate of missed diagnosis and misdiagnosis is still very high, and the prognosis has not been evaluated. The aim of this study was to investigate the accuracy of prenatal diagnosis and to evaluate the prognosis semi-quantitatively according to the shape of the ring and the distance between the vessel and the trachea.
METHODS
From 2019 to 2021, 37,875 fetuses underwent prenatal ultrasound examination in our center. All fetal cardiac examinations were performed using the fetal echocardiography method proposed by the American Institute of Ultrasound in Medicine (AIUM) combined with dynamic sequential cross-sectional observation (SCS). For SCS, the standard abdominal section was taken as the initial section, and the probe was moved cephalically along the long axis of the body until the superior mediastinum had disappeared. If a vascular ring was found, the shape of the ring and the distance of the branch to the airway were observed. The distance relationship with the airway was divided into three grades: I-III; the closer the distance, the lower the grade. The vascular rings were monitored every 4 weeks before birth. All were monitored before surgery or 1 year after birth.
RESULTS
A total of 418 cases of vascular rings were detected. There was no missed diagnoses or misdiagnoses by SCS. The vessels formed different shaped rings according to their origin and route. Grade I, "" and "O" rings have a poor prognosis and are associated with the highest risk of respiratory symptoms.
CONCLUSIONS
SCS can accurately diagnose vascular rings before delivery, evaluate the shape and size of the rings to conduct prenatal monitoring of children until birth, which plays a guiding role in airway compression after birth.
Topics: Female; Humans; Pregnancy; Cross-Sectional Studies; Prenatal Diagnosis; Prognosis; Ultrasonography, Prenatal; Vascular Ring
PubMed: 37131147
DOI: 10.1186/s12884-023-05637-y -
Pediatric Radiology Mar 2023This article reviews the physiology of the ductus arteriosus, the pathophysiology of the patent ductus arteriosus (PDA), and the role advanced imaging such as computed... (Review)
Review
This article reviews the physiology of the ductus arteriosus, the pathophysiology of the patent ductus arteriosus (PDA), and the role advanced imaging such as computed tomography (CT) and magnetic resonance imaging (MRI) can play in guiding diagnosis and percutaneous or surgical intervention. A PDA can have variable clinical and radiologic presentations and can be important to characterize in patients with vascular rings, aortic maldevelopment and congenital heart disease. An understanding of the PDA and the application of CT and MRI can allow the radiologist to provide key information to physicians who plan to close a PDA or maintain PDA patency in the setting of ductal-dependent congenital heart disease.
Topics: Humans; Ductus Arteriosus, Patent; Ductus Arteriosus; Heart Defects, Congenital; Aorta; Tomography, X-Ray Computed
PubMed: 36221034
DOI: 10.1007/s00247-022-05518-0 -
Translational Pediatrics Jun 2023Vascular rings, including double aortic arch and right aortic arch with aberrant left subclavian and left ligamentum, are part of a larger group of vascular-related... (Review)
Review
Vascular rings, including double aortic arch and right aortic arch with aberrant left subclavian and left ligamentum, are part of a larger group of vascular-related aerodigestive compression syndromes that also includes innominate artery compression syndrome, dysphagia lusoria, aortic arch anomalies, and aneurysms of either the aorta or pulmonary artery. Additionally, post-surgical airway compression is a distinct entity in itself. The approach to the diagnosis and management of these varied phenomena has been streamlined by the multidisciplinary team at Boston Children's Hospital. Echocardiography, computed tomographic angiography, esophagram, and three-phase dynamic bronchoscopy are routinely performed in these patients in order to produce a comprehensive understanding of the unique anatomic challenges that each patient presents. Adjunctive diagnostic techniques include modified barium swallow, routine preoperative and postoperative screening of the vocal cords, and radiographic identification of the artery of Adamkiewicz. Beyond the vascular reconstruction, which ranges from subclavian-to-carotid transposition to descending aortic translocation, we liberally apply tracheobronchopexy and rotational esophagoplasty to relieve respiratory and esophageal symptoms. Due to the heightened risk for recurrent laryngeal nerve injury, intraoperative recurrent laryngeal nerve monitoring has become routine in these cases. The comprehensive care of these patients requires the coordinated efforts of a large team of dedicated personnel in order to achieve the optimal result.
PubMed: 37427058
DOI: 10.21037/tp-23-39 -
Cardiology in the Young Nov 2021Vascular rings cause respiratory symptoms in children. Treatment consists of surgical division; however, data regarding mid-term results are scarce. The purpose of this...
BACKGROUND
Vascular rings cause respiratory symptoms in children. Treatment consists of surgical division; however, data regarding mid-term results are scarce. The purpose of this study was to evaluate clinical results of vascular ring surgery.
METHODS
Retrospective chart review of consecutive patients who underwent vascular ring surgery. Mid-term follow-up consisted of clinic visits and telephone questionnaire over a 1-year period sampling at five points in time.
RESULTS
Follow-up of 85 patients who underwent vascular rings surgery revealed significant symptomatic improvement within 6 months. In total, 50% were symptomatic to some degree at their last documented clinic visit complaining of stridor (36.8%), cough (34.2%), wheezing (10.5%), dyspnoea on exertion (10.5%), or recurrent respiratory infections (23.7%). By telephone questionnaires, 83% described a significant improvement in respiratory symptoms, 63.4% had some residual symptoms, 36.6% stridor, 38.8% chronic cough, 32.4% bronchodilator use,18.3% had at least one episode of pneumonia per year, 31% of children had dyspnoea or respiratory limitation, and 12.7% while doing physical activities. We found no association between the age at surgery or anatomic variant to the presence of symptoms at mid-term follow-up.
CONCLUSIONS
Surgical division of vascular rings results in significant clinical improvement within 1 year; nevertheless, some patients remain symptomatic to some degree. We found no association between the age at surgery or anatomic variant to the presence of symptoms at mid-term follow-up. Further evaluation is warranted to assess the nature of residual symptoms and explore whether anatomical causes can be identified leading to surgical modifications.
PubMed: 34776035
DOI: 10.1017/S1047951121004224