Vascular rings are rare anomalies of congenital cardiovascular. This study aimed to assess the outcome of surgical repair of congenital vascular rings in children. This retrospective study reviewed data from 237 patients with congenital vascular rings who underwent surgical division from January 2008 to October 2022. The mean age of patients was 12 months (Interquartile range, IQR, 6, 32 months) and their mean weight was 9.5kg (IQR, 8, 14kg). The data revealed that 153 patients (65%) were boys. The complete vascular rings consisted of a Double aortic arch (DAA, 33%), Right aortic arch (RAA) with left ligamentum arteriosum (LLA, 39%), and circumflex aorta (0.8%). The incomplete vascular rings consisted of a pulmonary artery sling (PAS, 26%), innominate artery compression syndrome (0.4%), and Left aortic arch (LAA) with Aberrant right subclavian artery (ARSCA, 0.8%). There were 3 hospital deaths (1.3%) and only one late death during the study. The overall mortality was 1.7% (4 patients). Eighteen patients underwent reintervention for complications. The overall survival rates at 1, 5, and 10 years were 98.3%, 98.3%, and 98.3%, respectively. The rate of freedom from residual symptoms at 1, 5, and 10 years was 97%, 91.3%, and 86.2%, respectively. The results show that surgical repair of congenital vascular rings in pediatric patients yields excellent outcomes: most patients' symptoms improved and only a small proportion required reintervention. Although the pulmonary artery sling forms an incomplete vascular ring, it is associated with a higher incidence of tracheal stenosis and an increased risk of persistent postoperative respiratory symptoms. These observations suggest that more proactive intraoperative management of tracheal stenosis should be considered during the primary operation.

A 4-year-old neonate presented with inspiratory stridor and respiratory distress due to tracheal compression. Echocardiography and CT scans documented a double aortic arch with a dominant right arch and hypoplastic left arch. Cardiac surgical correction was indicated. This case highlights the importance of suspecting vascular anomalies in neonates with persistent respiratory symptoms.

Background: The main aim of this study is to increase the recognizability of aortic arch anomalies during fetal echocardiographic examination. To evaluate intervessel distances in the three-vessel view in fetuses with isolated right or double aortic arch (DAA) anomalies during fetal echocardiography. Methods: This retrospective study included six fetuses examined between 19+0 and 21+0 weeks of gestation, with isolated right or DAA anomalies confirmed postnatally. Five fetuses had an isolated right aortic arch (RAA) anomaly, while one had an isolated DAA anomaly. Diameters of the main pulmonary artery (PA) and ascending aorta (Ao) were measured from the three-vessel view. Additionally, intervessel distances between the main PA–Ao, the Ao and superior vena cava (Ao–SVC), and the main PA–SVC were assessed. The standard deviation (SD) values were assessed using nomograms appropriate for gestational age. Results: The diameters of the PA and Ao were not significantly different from those in the normal population in any of the fetuses. In five of the six fetuses, the PA–Ao distance exceeded +3 SD, indicating an increase compared with fetuses without congenital heart defects. Additionally, the PA–SVC distance exceeded +3 SD in four fetuses, indicating a marked increase compared to fetuses without congenital heart anomalies. In contrast, the Ao–SVC distance was below –3 SD in only two fetuses, indicating a decrease, while no significant deviation was observed in the remaining four cases. Conclusions: In fetal echocardiography, isolated right or DAA anomalies can lead to altered intervessel distances on the three-vessel view. Notably, increases in the PA–Ao and PA–SVC distances were observed.

Abstract Objectives Vascular anomalies can cause extrinsic esophageal compression, leading to dysphagia or feeding difficulties in children. Diagnosis typically relies on imaging and endoscopy, which may under‐ or overestimate functional narrowing. Endoluminal functional lumen imaging probe (EndoFLIP) provides luminal parameters in real‐time, but its role in vascular compression is not well‐defined. Methods We retrospectively reviewed children with vascular anomalies who underwent upper endoscopy with EndoFLIP (July 2021–April 2025). Demographics, imaging, endoscopic/EndoFLIP findings, and outcomes were recorded. In select cases, paired measurements at the lower esophageal sphincter (LES) and compression site were compared at matched balloon volumes. Results Eight patients (mean age 12.1 years; range 6–17; 5 females) were included. Vascular anomalies included left aortic arch with aberrant right subclavian artery ( n = 5), double aortic arch ( n = 2), and right aortic arch with anomalous left subclavian artery ( n = 1). EndoFLIP detected narrowing in 6/8 (75%), compared with 4/8 (50%) on endoscopy and 6/7 (86%) on upper gastrointestinal (UGI) series. Two patients had narrowing on EndoFLIP despite normal endoscopy; two others had UGI narrowing but normal EndoFLIP and endoscopy. Paired measurements ( n = 2) showed markedly reduced diameter and distensibility at the compression site versus the LES. Management included surgical repair ( n = 3), dietary modification ( n = 2), and proton‐pump inhibitor therapy ( n = 3), with variable symptom improvement. No procedural complications occurred. Conclusions EndoFLIP was safe, feasible, and provided meaningful complementary physiologic information in this heterogeneous cohort. These preliminary findings support a potential diagnostic role for EndoFLIP in esophageal vascular compression, warranting confirmation in larger, standardized, prospective studies.

To establish objective criteria for predicting postnatal respiratory symptoms and determine surgical indications in fetuses with double aortic arch (DAA). This single-center, retrospective cohort study included fetuses diagnosed with DAA using fetal echocardiography and managed perinatally at Kanagawa Children's Medical Center from January 2013-December 2024. Clinical data, fetal echocardiography, postnatal contrast-enhanced computed tomography (CT) findings, surgical intervention, and outcomes were reviewed. Narrowest tracheal diameter (NTD) Z score in late gestation and narrowest-to-reference tracheal area (NTA/RTA) ratio on postnatal CT were measured. Primary and secondary outcomes included respiratory symptoms shortly after birth and elective surgery, respectively. Groups were compared using t-tests, and the optimal cutoff values were determined using receiver operating characteristic (ROC) curve analysis. Reproducibility was assessed using intraclass correlation coefficient. Twenty-two patients were included: 5 symptomatic and 17 asymptomatic (12, prophylactic surgery; 5, observation). Symptomatic patients had significantly lower NTD Z scores (median - 1.8 vs. -1.2, P = 0.012) and NTA/RTA ratios (median 0.39 vs. 0.67, P < 0.001). NTD Z score ≤ - 1.7 (area under the ROC curve [AUC] 0.87; sensitivity 80%, specificity 88%) and NTA/RTA ratio ≤ 0.5 (AUC 0.94; sensitivity 100%, specificity 82%) were optimal thresholds for predicting early symptoms. In the asymptomatic group, NTA/RTA ratio ≥ 0.56 identified candidates for observation without surgery. The ICC values for both parameters exceeded 0.85, indicating excellent reproducibility. The prenatal NTD Z score and postnatal NTA/RTA ratio accurately predict postnatal respiratory symptoms in fetuses with DAA. These quantitative parameters may guide individualized perinatal management and surgical decision-making.

Ductal stenting in a neonate with double aortic arch, pulmonary atresia, and ventricular septal defect without airway compression

Prenatal Diagnosis of a Double Aortic Arch in the Current Era: A Change in Trajectory?

Introduction: Double aortic arch (DAA) is a rare congenital vascular ring anomaly resulting from the persistence of both fourth embryonic aortic arches, creating a complete encirclement of the trachea and esophagus. It is most frequently diagnosed in infancy when symptoms of airway or esophageal compression are pronounced. Adult presentation is uncommon and may manifest as progressive dysphagia or respiratory symptoms. Case Presentation: We report the case of a 62-year-old male with a 3-month history of progressive dysphagia to solids. Initial esophagography demonstrated smooth, well-circumscribed indentation of the right lateral esophageal wall with leftward displacement, suggestive of a right aortic arch. Subsequent computed tomography angiography (CTA) revealed a double aortic arch with a dominant right arch and hypoplastic left arch, forming a complete vascular ring. There was significant anterolateral tracheal compression with leftward deviation and anterior displacement of the esophagus. Conclusion: This case underscores that DAA, although typically diagnosed in childhood, can remain asymptomatic until late adulthood. CTA with three-dimensional reconstruction is the diagnostic modality of choice, providing essential anatomical detail for diagnosis and surgical planning in symptomatic adult patients.

This retrospective study evaluated the clinical utility of Virtual Reality (VR) in visualizing extracardiac CHD (eCHD) abnormalities involving great vessels, pericardium, or structures outside the heart in nine pediatric patients. Anonymized computed tomography angiography (CTA) DICOM images were processed using Elucis (Version 1.10 elucis next) software to generate interactive 3D models via segmentation. VR models were reviewed for a variety of cases: vascular rings (two with right aortic arch, aberrant left subclavian artery, and diverticulum of Kommerell; two with double aortic arch), pericardial teratomas (n = 2), right superior vena cava draining into the left atrium (n = 1), left pulmonary artery sling (n = 1), and aortopulmonary window (n = 1). VR video images were presented during weekly heart center conferences. A survey conducted among heart center staff assessed the perceived value of VR in clinical practice. A total of 62% found traditional diagnostic modalities very effective, 100% considered VR a valuable diagnostic tool, 65% responded positively to VR image resolution, 50% highlighted its educational benefit, 81% believed VR enhanced diagnostic accuracy and surgical planning, and 100% would recommend its use to colleagues. This study demonstrates the successful integration of VR-based segmentation into clinical workflows, underlining its potential as both an educational resource and a tool to support diagnostic and surgical decision-making.

Double aortic arch is a congenital vascular ring with tracheal and oesophageal compression, potentially causing stridor and dysphagia. While some recommend early surgery, others favour observation. We present a 7-week-old female with mild symptoms and prenatally diagnosed double aortic arch who suffered cardiac arrest, highlighting the need for early surgery in patients with vascular rings and airway or oesophageal compression.

Background: Vascular ring (VR) is a rare congenital aortic arch anomality causing external compression on trachea and/or esophagus with variable onset and severity. Two common types are right aortic arch with aberrant left subclavian artery (RAoA/ALSCA) and double aortic arch (DAoA). Although RAoA/ALSCA and DAoA are frequently discussed together, the differences between the two VR have been poorly characterized. RQ: Are RAoA/ALSCA and DAoA distinct clinical entities? Methods: Retrospective chart review of pediatric patients diagnosed with isolated VR was performed. Demographic data, classification of VR, clinical presentation, imaging studies, and postoperative course were analyzed. Data is shown as either count (n) and percentage or median and interquartile range (IQR). Results: One hundred seventy-six patients were diagnosed with isolated VR from 1998 to 2024 at our cardiac center, including 121 RAoA/ALSCA, 46 DAoA, and 9 others (Table 1). Most patients were diagnosed before 1 year (75.6%), 93 of which were prenatally diagnosed (69.9%). Sixty percent of patients were symptomatic, and 66.4% of patients underwent surgical repair. When RAoA/ALSCA and DAoA were separately studied, prenatal diagnosis was more common in RAoA/ALSCA (Table 2). Respiratory symptoms were more frequent in DAoA than in RAoA/ALSCA. Higher incidence of surgery was noted in DAoA than in RAoA/ALSCA (96.5% vs. 54.5%, p &lt; 0.0001). There was no difference in age at surgery, post-operative hospital days, or incidence of surgical complications between DAoA and RAoA. When unrepaired and repaired VR were analyzed in more recent years (from 2018 to 2024), RAoA/ALSCA was more common among the unrepaired group (Table 3). The unrepaired group was more likely to be asymptomatic and less likely to have airway compression on axial imaging. Majority of DAoA was repaired (88.9%) whereas 71.4% of RAoA/ALSCA was not repaired. Only 5 patients were symptomatic despite 26 patients showing airway compression in an unrepaired group, whereas symptom was well correlated with airway compression in a repaired group. Conclusions: Although RAoA/ALSCA and DAoA are frequently discussed together as the same clinical entity, there were several notable differences between the two. Majority of DAoA patients were symptomatic and underwent surgical repair more frequently than RAoA/ALSCA. Timing of surgery, incidence of post-operative complications, and length of post operative hospital stay were comparable between the two.

Intro: Vascular rings (VR) are congenital anomalies where the trachea and esophagus are encircled by vascular structures and may require surgery. There are no published studies on the multicenter practice variation in the use of CCT and the quantitative assessment of tracheal narrowing. Method: 10 academic centers contributed to this retrospective study of all consecutive patients with a suspected VR and CCT between 1/2018 and 1/2024. CCT details, clinical data, VR subtype and percentage of tracheal narrowing, defined as the difference in tracheal areas above and at the VR was evaluated. Results: 455 patients (BSA median 0.3 m2 with IQR 0.23-0.46, ages 0-18 years, 53% male) were included with 284 (63%) diagnosed with right aortic arch with aberrant left subclavian artery (RAA) and 138 (30%) with a double aortic arch (DAA). Fifty-four percent of patients developed symptoms in this cohort and 57 (13%) had a confirmed genetic diagnosis. The most common symptom was stridor (52% of symptomatic) and 57% developed symptoms before 6 months of age. The most common scanner platform was Siemens (85%) with dual source (56%) and prospective gating (68%) with only 13 (3%) cases with dynamic airway evaluation. The indication to perform a CCT was to confirm echocardiographic diagnosis (67%) or after symptom onset (28%). Table 1 shows the variation in technique by center. 22% of patients had a change in suspected VR type after CCT (Gwet AC1 0.69, 95% CI: 0.63 – 0.75). Tracheal narrowing &gt;10% was seen in 83% of patients analyzed (Table 2A). Tracheal narrowing was greater in DAA (36.3%) than RAA (22.4%, p&lt;0.0001). Table 2B shows the correlation of tracheal narrowing with symptoms and surgery. With each unit increase in % tracheal narrowing in DAA, the odds of seeing symptoms increased by 3% (OR: 1.03, 95% CI: 1.01-1.05, p = 0.01). A threshold analysis showed the cutoff for predicting the presence of symptoms in DAA is 32.1%. There was a discrepancy noted between CCT and intra-operative diagnosis in 5 patients (1.9%). Conclusion: There is variability among centers in the use CCT for VR. Tracheal narrowing seen on CCT was common and was associated with symptoms but not surgery in the entire cohort and the DAA subgroup. For the RAA subgroup, symptoms were present in those with and without tracheal narrowing.

Incidental diagnosis of co-dominant double aortic arch in an adult: A rare cause of chronic cough and chest pain

The online version contains supplementary material available at 10.1007/s12055-025-02036-0.

Changes in the congenital heart disease spectrum at China national children's medical center: a review of 10-year data

BACKGROUND AND OBJECTIVES While symptomatic vascular ring patients are typically referred for surgical relief, the management of asymptomatic cases remains controversial. We assessed outcomes of symptomatic and asymptomatic vascular rings managed over 14 years at our center, focusing on surgical and conservative approaches. METHODS This is a retrospective chart review of 228 children diagnosed with vascular ring anomaly between January 2011 and December 2023. Data collected included anatomical subtypes, symptoms, diagnostics, management, and long-term outcomes. The primary outcome was symptom-free survival, with subgroup analyses for isolated vascular rings and comparisons by anatomical type. RESULTS Of the 228 patients, 177 had isolated vascular rings, most commonly right aortic arch (RAA) with aberrant left subclavian artery (ALSA) and double aortic arch (DAA). 60% (n=106) were symptomatic, and 83% of symptomatic patients underwent surgery with symptom resolution in 77%. 17% of symptomatic patients were managed conservatively, with 61% experiencing symptom improvement without surgery. Surgical outcomes were favorable, with low reintervention rates and no perioperative mortality, although persistent symptoms were observed in 20% of RAA and 28% of DAA cases. Age at surgery did not significantly affect symptom resolution in RAA patients. 40% of patients (n=71) never developed symptoms. CONCLUSIONS While surgical repair is effective, symptoms may persist in some cases. Asymptomatic patients can often be safely observed, with surgery reserved for those who develop symptoms. Multidisciplinary aerodigestive evaluations may help in guiding conservative management for select symptomatic patients.

Double aortic arch (DAA) is a rare congenital vascular malformation that forms a ring around the esophagus and trachea, with a prevalence of 0.005% and chromosomal abnormalities associated in only 24% of cases. The most common symptoms are noisy breathing and persistent cough, often misdiagnosed as primary respiratory diseases. We describe a clinical case diagnosed prenatally, analyzed in light of the most recent literature, to identify management and follow-up strategies.

Dysphagia Caused by a Double Aortic Arch with Kommerell Diverticulum in an Adult.

BackgroundDouble aortic arch (DAA) is a rare congenital vascular anomaly resulting in a complete vascular ring that encircles and compresses the trachea and esophagus, leading to respiratory and gastrointestinal symptoms. Accurate diagnosis and timely surgical intervention are essential for symptom relief and preventing complications. However, data on surgical outcomes and long-term follow-up are limited. This study retrospectively analyzed surgical outcomes and perioperative management of DAA to elucidate optimal diagnostic and therapeutic approaches.MethodA retrospective cohort study enrolled patients undergoing DAA repair (August 2015–May 2024). Participants were stratified into isolated DAA or DAA with associated intracardiac anomalies groups. Demographic, operative, and outcome variables were compared.ResultsAmong 10 patients undergoing double aortic arch repair, 6 comprised the isolated DAA group (3 males/3 females; mean age 3.70 ± 3.18 months; mean weight 6.28 ± 2.77 kg) and 4 had associated intracardiac anomalies (3 males/1 female; mean age 6.70 ± 6.12 months; mean weight 6.15 ± 3.59 kg). Isolated DAA patients and those with intracardiac anomalies showed no statistically significant differences in: symptom onset (28.17 ± 37.66d vs. 30.50 ± 41.96d), anatomic subtypes (dominant right arch 83% vs. 50%), extracardiac anomaly rates (50% vs. 75%), or clinical manifestations—respiratory (67% vs. 100%) and other systemic (17% vs. 75%) (all P > 0.05). All procedures were successfully completed with significantly shorter operative time in the isolated group (104.83 ± 22.23 vs. 233.25 ± 38.55 min, P < 0.001). No significant intergroup differences (P > 0.05) were observed in preoperative ventilation, blood loss, ventilator duration, Cardiac Intensive Care Unit stay, drainage duration, hospitalization, or complication rates. During mean 12.7-month follow-up (1–36 months), near-complete symptom resolution occurred in 9/10 survivors, with one death from respiratory failure in a comorbid patient 10 days post-discharge.ConclusionSurgical repair of double aortic arch demonstrates acceptable safety and efficacy in both infants and children, with favorable short-to-midterm clinical outcomes regardless of concomitant intracardiac anomalies.

Double aortic arch: a rare cause of adult-onset dysphagia