To investigate the relationship between quantitative tracheal geometry and clinical course among various types of vascular ring and to identify factors correlating with symptom presentation. Patients with vascular ring diagnosed between April 2010 and December 2022 were included. All the patients were classified as type 1 (complete double aortic arch); type 2 (incomplete double aortic arch); type 3 (circumflex aorta); type 4 (right aortic arch and aberrant left subclavian artery with a left retroesophageal diverticulum of Kommerell); or type 5 (mirror-imaged right aortic arch with retroesophageal aortic diverticulum). Their clinical characteristics and quantitative variables on computed tomography (CT) were compared. Of the 50 patients enrolled, those with type 1 tended to have a smaller luminal tracheal diameter at the level of the ring. The median symptom-free survival time was shortest in this group (16.0 days [95% confidence interval (CI): 9.4-51.0]), followed by type 3 (138.0 days [95% CI: 0.0-851.4]). Type 1 (hazard ratio [HR]: 9.0; 95% CI: 2.3-35.0; P = 0.001), type 3 (HR: 4.2; 95% CI: 1.4-13.2; P = 0.013), and the percentage of tracheal narrowing in the anteroposterior projection (HR: 0.87; 95% CI: 0.78-0.96; P = 0.008) were significantly associated with symptom presentation in the time-dependent course. The aortic arch encircling the entire circumference in type 1 and high-pressure vasculature in front of the vertebral body in types 1 and 3 may contribute to raising the risk of symptom presentation.

BACKGROUND: Vascular Rings (VRs) are congenital malformations of the aortic arch that can compress the trachea and esophagus producing symptoms. Approximately, 5%-10% of patients experience persistent symptoms postrepair and 9% require reintervention. There are various approaches to repair-simple ligation and division of the ring or more complex procedures. Our objective was to describe outcomes for VR repair using a \"simple\" surgical approach.
METHODS: We identified patients who underwent VR repair from 2012 to 2022 at our institution. Clinical and surgical data, demographics, intraoperative, and postoperative outcomes were extracted from their electronic medical records. Outcomes were analyzed and regression analysis was used to identify risk factors for residual symptoms after repair.
RESULTS: Ninety three patients with VR repair were included. Prevalence of symptoms were reduced following surgery (80% preoperative versus 13% postoperative, P 0.03). Tracheomalacia also decreased from 16% to 7% postrepair. No deaths were reported in our cohort and 2% of our patients required reintervention. Multivariable regression analysis showed that preoperative tracheomalacia was associated with having persistent symptoms after VR repair (odds ratio 6.2, 95% CI 1.02-37.6).
CONCLUSIONS: Our institutional experience using a simple surgical approach showed a significant decrease in symptoms, a very low reintervention rate and no mortality. Preoperative tracheomalacia can be a risk factor for persistent symptoms postrepair. We believe a simple surgical approach for children with complete VR repair can be effective in relieving symptoms.

暂无摘要。

BACKGROUND: In double aortic arch (DAA) one of the arches can demonstrate atretic portions postnatally, leading to diagnostic uncertainty due to overlap with isolated right aortic arch (RAA) variants. The main objective of this study is to demonstrate the morphological evolution of different DAA phenotypes from prenatal to postnatal life using 3D fetal cardiac magnetic resonance imaging (CMR) and postnatal CT/CMR imaging.
METHODS: 3D fetal CMR was undertaken in fetuses with suspected DAA over a six-year period (Jan 2016 - Jan 2022). All cases with surgical confirmation of DAA were retrospectively studied and morphology on fetal CMR was compared to postnatal CT/CMR and surgical findings.
RESULTS: 32 fetuses with surgically confirmed DAA underwent fetal CMR. All demonstrated a complete DAA with left-sided arterial duct. The RAA was dominant in 30/32 (94%). Postnatal CT/CMR was undertaken at median age of 3.3months (IQR 2.0-3.9) demonstrating DAA with patency of both arches in 9/32 (28%), with 6 showing signs of coarctation of the left aortic arch (LAA). The LAA isthmus was not present on CT/CMR in 22/32(69%), the transverse arch between left carotid and left subclavian artery was not present in 1 case.
CONCLUSIONS: Fetal CMR provides novel insights into perinatal evolution of DAA. The smaller LAA can develop coarctation or atresia related to postnatal constriction of the arterial duct, making diagnosis of DAA challenging with contrast-enhanced CT/CMR. This highlights the potentially important role for prenatal 3D vascular imaging and might improve intepretation of postnatal imaging.

UNASSIGNED: Vascular rings represent 1% of congenital cardiovascular abnormalities. Phenotypic expression varies from asymptomatic to severe forms related to either oesophageal or tracheal compression. While refinement in prenatal screening led to an increase in fetal diagnosis, optimal management in asymptomatic neonates and infants is currently a matter of debate. We report our center experience of vascular ring management over three decades.
UNASSIGNED: In this single-center retrospective study, data were extracted from patient medical records. To obtain information on symptoms/medication at follow-up, clinical records from pediatric clinics were reviewed. For patients followed in other institutions, a web-based questionnaire was sent to referring pediatricians.
UNASSIGNED: Out of 82 patients, 69 were symptomatic (84%). Common symptoms included recurrent respiratory tract infections (43%), stridor (32%), gastro-esophageal reflux (33%), and dysphagia (26%). Diagnosis relied on cardiac ultrasound, barium swallow studies, and chest computerized tomography scan. Surgical repair (thoracotomy 91%) was performed in 79 patients (96%). Median age at repair was 13 [interquartile range (IQR), 4.4-48] months. There was no mortality. Minor complications occurred in 14 patients (18%). Median hospital length of stay was 7 (IQR, 6-9) days. In total, 24% of patients remained symptomatic (median follow-up 54 months). Half of those were asthma-related, with nearly 90% freedom from ring-related symptoms.
UNASSIGNED: Most patients were symptomatic at the time of diagnosis. Vascular rings such as pulmonary slings or tracheal compression syndromes require prompt management. Despite surgery, 24% of patients were not symptom-free at follow-up. Finally, surgery in asymptomatic patients resulted in low morbidity supporting the current recommendation of early surgical repair.
UNASSIGNED: III.

Introduction A double aortic arch (DAA) is a rare congenital vascular anomaly that encircles the trachea and esophagus, resulting in compression of both structures and causing variable symptoms of wheezing, stridor, increased work of breathing, or dysphagia. DAA usually presents in infancy but can be incidentally found later in life. The standard management of DAA is surgical repair. However, observation and follow-up have been recommended in asymptomatic or mild cases. The long-term outcome of surgical repair versus observation is not well-reported. We described the long-term clinical outcome of patients with DAA who were surgically repaired versus non-repaired at our institution.  Methods Electronic medical records were searched for the patients diagnosed with DAA before the age of 18 years. Data from clinical, radiological, and bronchoscopic findings, pulmonary function test (PFT), and cardiopulmonary exercise testing (CPET) were extracted. A structured phone questionnaire of patients\' parents regarding past and current symptoms was also conducted. Results A total of 12 patients (eight males four females) with DAA were identified. Median age was 8.5 (1.5-17) years. The age at diagnosis was 60 (1-192) months. Post diagnosis follow-up period was 20 (2-156) months. Five patients were surgically repaired, and seven patients were not repaired. The median age of surgery was five (1-15) years in repaired patients. The phone questionnaire was completed in only 10 patients (five repaired and five non-repaired). Respiratory symptoms in infancy were reported in all repaired and non-repaired patients and were resolved in all five repaired patients and in four of the five non-repaired patients. One non-repaired patient complained of intermittent dyspnea on exertion. Gastrointestinal symptoms were present in infancy in three repaired and three non-repaired patients and were improved in two repaired and one non-repaired patient. PFT was performed in five patients (one repaired, four non-repaired) and showed normal forced expiratory volume in one second (FEV1), forced vital capacity (FVC), and FEV1/FVC in all patients. Low peak expiratory flow (PEF) was seen in the repaired patient and in three of the non-repaired patients. CPET was conducted in four non-repaired patients and showed maximal oxygen consumption (VO2-max) of 66% predicted (58-88), maximal ventilation (VE-max) of 75% predicted (70-104), and ventilatory reserve of 55% predicted (48-104).  Conclusion Long-term clinical outcome is favorable in both repaired and non-repaired patients with DAA even though both groups reported respiratory symptoms during infancy. Therefore, clinical observation is a legitimate option in certain DAA patients.

Published data estimate the prevalence of the vascular ring at approximately 7 per 10,000 live births. The association of a double aortic arch with a D-transposition of the great arteries has been rarely described in the literature. In this study, we report the prenatal diagnosis of a 28-year-old woman. A fetal echocardiography at a gestational age of 24 weeks + 6 days showed a D-transposition of the great arteries and a double aortic arch with a ventricular septal defect and pulmonary stenosis. On the first night after birth, the baby experienced an increase in lactate levels, with the rate of oxygen saturation consistently below 80%. A few hours after birth, the patient underwent a Rashkind procedure. An echocardiography, CT chest x-ray, and CT angiogram confirmed a diagnosis with a severe reduction of the tracheal lumen (>85%) and bronchomalacia. Then, the patient underwent posterior tracheopexy and aortopexy and later an arterial switch operation, ventricular septal defect closure, and resection of a part of the infundibular septum, accepting the risk of potential neoaortic obstruction. The literature has reported only two cases of patients with a fetal echocardiogram diagnosis. Therefore, our patient is only the third one with a fetal diagnosis and the second one with a complex intracardiac anatomy, characterized not only by a ventricular septal defect but also by two separate components of the obstruction (a bicuspid valve and a dysplastic valve with a posterior deviation of the infundibular septum). In conclusion, a D-transposition of the great arteries with a double aortic arch remains an extremely unusual association. The clinical outcome of these patients presents a high degree of variability and is entirely unpredictable in prenatal life. Our greatest aim as fetal and perinatal cardiologists is to improve the management and outcome of these patients through a fetal diagnosis, recognizing types of congenital heart disease in newborns who require early neonatal invasive procedures.

OBJECTIVE: We evaluate long-term symptomatic improvement in vascular ring patients who underwent thoracoscopic division at a single quaternary pediatric surgery center.
METHODS: All pediatric patients who underwent vascular ring division without Kommerell\'s diverticulum resection between 01/2007-12/2022 were included. Surgeries were performed by pediatric general and thoracic surgeons. Patient demographic and clinical characteristics were obtained from retrospective chart review. Data on long-term symptomatic improvement were collected with structured telephone interviews.
RESULTS: 60% of patients were male. Median age at operation was 24 months (IQR: 11, 60 months) with a median weight of 11.3 kg (IQR: 8.7, 19.8 kg). All patients were symptomatic preoperatively with dysphagia being the most frequent complaint (42%), followed by chronic cough (21%). Of 41 patients eligible for the long-term follow-up survey, 8 patients with a primary diagnosis of a double arch with an atretic segment in the non-dominant arch and 9 with a right dominant arch with left ligamentum arteriosum and aberrant left subclavian artery (LSCA) were contacted and consented for participation. Median interval from surgery to survey completion was 95 months (IQR 28, 135 months). Most patients had no, or only minor, symptoms related to breathing and swallowing at the time of long-term follow-up. 88% of patients experienced postoperative symptom improvement, and only one patient reported worsening of symptoms over time.
CONCLUSIONS: Division of an atretic arch and/or ligamentum for patients with an aberrant LSCA without Kommerell\'s resection may be adequate to ensure long-term improvement of breathing and swallowing problems attributable to vascular rings.
METHODS: Level IV.

OBJECTIVE: The objective was to describe the successful thoracoscopic treatment of esophageal entrapment resulting from a vascular ring anomaly (VRA) comprising a persistent right aortic arch (PRAA) and left ligamentum arteriosum (LA) in a Babydoll sheep wether.
METHODS: Case report.
METHODS: Eight month old Babydoll sheep wether, 13 kg.
METHODS: The patient presented with a weight half that of its sibling, persistent regurgitation following eating, and delayed growth noted from the age of approximately 2 months, coinciding with the introduction of solid feed into the diet. Plain thoracic radiographs were within normal limits but computed tomography angiography (CTA) confirmed multiple congenital vascular anomalies. The primary finding was esophageal and tracheal entrapment by a PRAA and left LA. Thoracoscopic transection of the LA was performed with a bipolar vessel sealing device with the aid of transesophageal endoscopy.
RESULTS: Immediate improvement in attitude and absence of regurgitation were observed. The patient was discharged and subsequently reintroduced to grazing and long-stem hay, which were previously not tolerated. By 6 months post discharge, the patient\'s weight was 36 kg, comparable to an age-matched sibling and considered appropriate for the stage of growth.
CONCLUSIONS: Thoracoscopic transection of the LA in sheep is a feasible treatment for esophageal compression resulting from a VRA. Surgical intervention resolved the clinical signs and allowed normal digestive rumination, restoring bidirectional esophageal function in a ruminant.

We report the case of a 36-year-old female whose dysphagia revealed a congenital anomaly of the thoracic aorta: the right aortic arch with mirror image branching. This is a rare embryonic developmental anomaly where the aorta wraps around the right bronchus and the supra-aortic trunks emerge from the arch in the opposite order to normal. Most of the patients are asymptomatic unless there is a significant compression of mediastinal structures. Major compression of the esophagus or trachea, aneurysmal disease, dissection of the thoracic aorta, or the presence of a Kommerell diverticulum larger than 2 cm may require a surgical repair. There is no standard treatment and it must be adapted to the clinical presentation and the anatomic configuration of each patient. Our patient did not receive any treatment for her condition.
Nous rapportons le cas d’une patiente de 36 ans dont le tableau de dysphagie a permis de mettre en évidence une anomalie congénitale de l’aorte thoracique : l’arc aortique droit avec image en miroir. Il s’agit d’une anomalie de développement embryonnaire rare où l’aorte s’enroule autour de la bronche souche droite et où les troncs supra-aortiques émergent de la crosse dans l’ordre inverse et opposé à la normale. La grande majorité des patients est asymptomatique, à moins qu’il existe une compression des structures médiastinales. Une compression majeure de l’oesophage ou de la trachée, une maladie anévrismale, une dissection de l’aorte thoracique ou la présence d’un diverticule de Kommerell de plus de 2 cm peuvent justifier une sanction chirurgicale. Il n’y a pas de traitement standard et celui-ci doit être adapté à la présentation clinique et à la configuration anatomique du patient. Notre patiente n’a bénéficié d’aucun traitement pour son affection.