Symptomatic vascular ring due to double aortic arch: A report of two cases.

Vascular ring anomalies, represented mainly by the double aortic arch (DAA), account for about 1% of congenital cardiovascular anomalies and are characterized by tracheoesophageal compression due to encircling vascular or ligamentous structures. These anomalies arise from the failure of the right fourth aortic arch to regress during embryonic development, leading to symptoms ranging from respiratory distress to dysphagia. Diagnostic imaging includes chest radiography, CT, MRI, and echocardiography, each with specific considerations, especially in pediatric patients. Management, often surgical, focuses on relieving compression symptoms and has a favorable prognosis. This case series emphasizes the manifestations of the double aortic arch, whether it be respiratory distress or dysphagia, and addresses both right and left dominant arches.

Double innominate vein in a case with double aortic arch: a case report.

BACKGROUND: Double aortic arch (DAA) is a type of vascular ring, causing stridor and dysphagia owing to compression of the trachea and esophagus. In contrast, double innominate veins, which is a relatively rare venous anomaly, usually does not cause symptoms or require intervention. CASE PRESENTATION: A 2 month-old boy presented with mild stridor and a hoarse voice while crying, and was diagnosed as having DAA, small atrial septal defects and double innominate vein. The double innominate veins, crossing over and underneath the ascending aorta, did not contribute to airway compression. To release the narrow vascular ring caused by the DAA, surgical resection of the atretic side of the arch was performed by lateral thoracostomy at 4 months of age, before the appearance of signs of bronchomalacia. The double innominate veins were left untouched, and open-heart surgery was avoided. CONCLUSIONS: DAA should be suspected if airway symptoms are present in early infancy, particularly if a right aortic arch is noted. It is important not to hesitate to perform diagnostic imaging and release the DAA, as this might prevent or minimize airway complications. However, for double innominate veins, operative release is not necessary unless it interferes with surgical procedures or is causing symptoms.

Evaluation of tracheal diameter and angles in fetuses with double aortic arch using prenatal ultrasound: implications for postnatal management.

Objective: This study aims to analyze the value of prenatal ultrasound in the screening, diagnosis, and treatment of double aortic arch (DAA) malformations. Methods: A retrospective analysis was conducted on 31 fetal cases with double aortic arch anomalies over a 12-year period from June 1, 2011 to June 1, 2023. The assessment included combined measurements of fetal tracheal internal diameter Z-score and DAA pinch angle, along with ultrasonographic findings, associated anomalies, genetic abnormalities, postnatal CTA images, and long-term postnatal outcomes. Results: Of the 31 fetal double aortic arch cases, 15 were right aortic arch dominant, 2 were left aortic arch dominant, and 14 had a balanced double arch. Genetic testing was performed on 19 cases, revealing abnormalities in 2 cases, including one Turner syndrome, and one carrier of ichthyosis gene with heterozygous deletion. Out of the total cases, 29 were delivered, and 2 cases were terminated. Prenatal diagnosis accurately identified 29 cases (29/31, 93.5%), which was confirmed by postnatal pathological anatomy, echocardiography, surgery or CTA. Fetal tracheal internal diameter Z-scores were significantly smaller in the symptomatic group than in the asymptomatic group (-1.27 ± 0.49 vs -0.68 ± 0.60, P = 0.018). The area under the curve was 0.776 (95% confidence interval, 0.593-0.960) using a tracheal internal diameter z-score cutoff of -0.73 with a sensitivity of 90% and specificity of 64.7%. The double arch pinch angle was significantly smaller in the symptomatic group than in the asymptomatic group [52.50° (38.25° to 59.00°) vs 60.00° (53.50° to 70.50°), P = 0.035]. The area under the curve was 0.744 (95% confidence interval, 0.554-0.935), and the sensitivity for determining the presence or absence of symptoms was 90% when the cutoff value was 62.5°, with a specificity of 47.1%. Fifteen cases opted for surgery with favorable surgical outcome. Conclusion: Prenatal echocardiography demonstrates good diagnostic efficacy for fetal double aortic arch. It is also essential to detect the presence of other underlying intra- and extracardiac malformations and genetic abnormalities. There is a significant difference in prenatal tracheal internal diameter Z-scores and double arch pinch angle between asymptomatic and symptomatic DAA infants. Symptomatic infants require early surgery, while asymptomatic infants should be monitored.

Long-Term Outcome of Surgically Repaired and Non-repaired Double Aortic Arch in Children.

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.

Prenatal aortic arch development in double aortic arch: Understanding postnatal closure of left aortic arch: A case report.

Double aortic arch (DAA) is a rare congenital abnormality characterized by a vascular ring that often requires surgical intervention due to respiratory complications. The DAA and right aortic arch with mirror-image branches (RAA-MB) represent abnormalities in development of the aortic arch. However, prognosis differs significantly, as the DAA forms vascular rings, whereas the RAA-MB typically does not. Distinguishing between the conditions becomes particularly challenging in cases of DAA with closure of the posterior portion of the left aortic arch (LAA) because the postnatal manifestations closely resemble those of RAA-MB. Herein, we present a case of DAA in which longitudinal observation of the LAA and RAA diameters during pregnancy aimed in predicting postnatal closure of the LAA. A 37-year-old female with suspected DAA was referred to our hospital at 26 weeks of gestation. Initial measurements revealed comparable diameters for the LAA and RAA; however, the LAA diameter decreased to approximately half that of the RAA by term owing to growth restrictions. Postnatal contrast computed tomography confirmed the closure of the posterior portion of the LAA and RAA with Kommerell diverticulum. Our findings suggest that careful monitoring of DAA throughout fetal development, especially during the third trimester, may aid in predicting atretic changes in the nondominant arch after birth, allowing an easy distinction between the DAA and RAA-MB after birth.

Case Report: An unusual case of a transposition of the great arteries with a double aortic arch: a highly complex fetal diagnosis with an unpredictable outcome.

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.

Perioperative and Anesthetic Considerations in Vascular Rings and Slings.

Vascular rings represent an increasingly prevalent and diverse set of congenital malformations in which the aortic arch and its primary branches encircle and constrict the esophagus and trachea. Perioperative management varies significantly based on the type of lesion, its associated comorbidities, and the compromise of adjacent structures. Multiple review articles have been published describing the scope of vascular rings and relevant concerns from a surgical perspective. This review seeks to discuss the perioperative implications and recommendations of such pathology from the perspective of an anesthesia provider.

Associated Anomalies and Outcome in Patients with Prenatal Diagnosis of Aortic Arch Anomalies as Aberrant Right Subclavian Artery, Right Aortic Arch and Double Aortic Arch.

We aimed to evaluate retrospectively associated anomalies and outcome in prenatal aortic arch anomalies (AAAs). We included ninety patients with aberrant right subclavian artery (ARSA), right aortic arch (RAA) with mirror image branching (RAA-mirror) or aberrant left subclavian artery (RAA-ALSA) and double aortic arch (DAA) between 2011 and 2020. In total, 19/90 (21.1%) had chromosomal anomalies, the highest rate being within the ARSA subgroup (17/46, 37%). All (13/13) of the RAA-mirror subgroup, 10/27 (37.0%) of RAA-ALSA, 13/46 (28.3%) of ARSA and 0/4 within the DAA subgroup had additional intracardiac anomaly. The rate of extracardiac anomalies was 30.7% in RAA-mirror, 28.3% in ARSA, 25.0% in DAA and 22.2% in the RAA-ALSA subgroup. A total of 42/90 (46.7%) had isolated AAAs: three (7.1%) with chromosomal anomalies, all trisomy 21 (3/26, 11.5%) within the ARSA subgroup. Out of 90, 19 (21.1%) were lost to follow-up (FU). Two (2.2%) intrauterine deaths occurred, and six (6.7%) with chromosomal anomalies terminated their pregnancy. In total, 63 (70.0%) were liveborn, 3/63 (4.8%) with severe comorbidity had compassionate care and 3/60 (5.0%) were lost to FU. The survival rate in the intention-to-treat cohort was 53/57 (93%). Forty-one (77.4%) presented with vascular ring/sling, two (4.9%) with RAA-ALSA developed symptoms and one (2.4%) needed an operation. We conclude that intervention due to vascular ring is rarely necessary. NIPT could be useful in isolated ARSA cases without higher a priori risk for trisomy 21 and after exclusion of other anomalies.

Hematemesis on an esophageal foreign body revealing a double aortic arch: a case study.

A double aortic arch is a rare abnormality of the aortic arch caused by the persistence of the distal part of the right dorsal aorta. It can be manifested by respiratory and/or digestive symptoms. We report a case of double aortic arch revealed by an esophageal foreign body complicated by haematemesis in a 13-year-old boy having required multidisciplinary care.

Prenatal diagnosis of right aortic arch: associated anomalies and fetal prognosis according to different subtypes.

OBJECTIVES: Right aortic arch (RAA) is a rare anomaly with an incidence of 0.1 % in the adult population and low-risk fetuses. Our aim in this study was to evaluate associated anomalies and conditions according to subtypes. METHODS: This was a retrospective study examining consecutive pregnancies diagnosed with RAA in our hospital between 2018 and 2022. Fetuses with RAA were divided into three groups, RAA with right-sided ductus arteriosus (RAA-RDA), RAA with left-sided ductus arteriosus (RAA-LDA), and RAA with a double aortic arch (RAA-DAA). RESULTS: A total of 81 fetuses were diagnosed as having RAA during the study period. The rate of cardiac anomalies (82.8 %) in the RAA-RDA group was higher than in the RAA-LDA (17.6 %) and RAA-DAA (22.2 %) groups (p<0.001). No statistically significant difference was found between the groups in terms of maternal age, diagnosis week, pregnancy outcome, extracardiac anomalies, and genetic anomalies. Three (8 %) of 36 fetuses with isolated RAA who resulted in live birth developed symptoms related to the vascular ring, and one (2.7 %) newborn with RAA-DAA underwent surgery. CONCLUSIONS: The incidence of cardiac anomalies is high in fetuses with RAA-RDA. Ultrasound examinations should be performed for cardiac anomalies and additional structural anomalies. Vascular ring formation is a rare but important complication due to compression risk to the trachea and esophagus.

Utility of the Angle between the Right Aortic Arch and First Branch for Detecting Double Aortic Arch via Fetal Echocardiography.

INTRODUCTION: The presence of a double aortic arch (DAA) is manifested by compressive symptoms, requiring surgery. DAA cases are classified as either complete or incomplete type. DAA and a right aortic arch with mirror image branching (mRAA) have a similar configuration to the first branch artery. The first branch of the mRAA is the left brachiocephalic artery, which appears to be the same as that of an incomplete DAA due to blood flow interruption. The present retrospective study aimed to evaluate the differences between DAA and mRAA by fetal echocardiography. METHODS: This single retrospective cohort study included all patients diagnosed with complete DAA, incomplete DAA, or mRAA at our facility between 2010 and 2022. The patients were diagnosed with complete DAA, incomplete DAA, or mRAA after birth and remaining fetal echocardiograms. The patients were divided into the DAA (complete DAA: n = 4, incomplete DAA: n = 3) and mRAA (n = 4) groups. The following three outcomes were compared: (1) angle between the right aortic arch and first branch (RF angle), (2) ratio of height to width of the region bounded by the aortic arch, first branch of the aortic arch, and descending aorta, and (3) maximum tracheal diameter on a three-vessel trachea view. RESULTS: The incomplete DAA cases were difficult to diagnose via fetal echocardiography. On fetal echocardiography, the RF angle was significantly steeper in the DAA group than in the mRAA group (median 57° [36°-69°] vs. 75° [62°-94°]; p < 0.05). The DAA and RAA groups showed no significant differences in the ratio of height to width of the region bounded by the aortic arch, first branch of the aortic arch, and descending aorta (median 0.57 [0.17-0.68] vs. 0.73 [0.56-1.0]) and maximum tracheal diameter (median 2.5 [1.4-3.3] vs. 3.2 [2.8-3.5] mm). The cut-off value for the presence of DAA was an RF angle <71°. CONCLUSION: The DAA group (complete and incomplete DAA) had a significantly steeper RF angle than the mRAA group. Therefore, RF angle measurement could improve the fetal diagnosis and postnatal prognosis of DAA.

Management of esophageal cancer in patients with a right aortic arch or double aortic arch: a case series of 34 cases.

BACKGROUND: Few cases describing patients with a right aortic arch (RAA) or double aortic arch (DAA) and esophageal cancer (EC) have been reported. METHODS: We analyzed RAA and DAA cases treated with esophagectomy in our center's database and reported in English-language studies until April 1, 2023. Our study assessed the malformation characteristics and surgical details of EC patients with RAA and DAA. RESULTS: We extracted data of 24 EC patients with RAAs and 10 EC patients with DAAs. In both groups, the patients were more likely to be Japanese and male, to have squamous cell carcinoma and to have tumors located in the upper thoracic esophagus or middle thoracic esophagus. Left thoracotomy was commonly applied for RAA patients. For DAA patients, the proportions of left-sided and right-sided approaches were similar. Esophagectomy under video-assisted thoracoscopic surgery (VATS) in RAA or DAA patients had been performed on a routine basis in recent years. There were two anastomotic leakages in each group. Specifically, Kommerell diverticulum rupture occurred in 1 RAA patient; gastric tube dilation occurred in 1 DAA patient; and recurrent laryngeal nerve (RLN) injury occurred in 2 RAA patients. The postoperative course was uneventful for most patients in both cohorts. CONCLUSIONS: Esophageal carcinoma is rarely seen in patients with an RAA or DAA. To adequately dissect superior mediastinal LNs, an auxiliary incision (such as sternotomy), the left door open method or a preceding cervical procedure should be used appropriately. Esophagectomy, whether via thoracotomy or thoracoscopic surgery, can be performed safely for both RAA and DAA.

Persistence of aerodigestive symptoms after vascular ring repair.

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.

An asymptomatic double aortic arch with separate right vertebral artery and left subclavian artery originating from Kommerell Diverticulum combined with congenital asplenia and absence of celiac trunk.

This report describes the unique case of a completely patent Double Aortic Arch (DAA) combined with Kommerell Diverticulum (KD), absence of celiac trunk and congenital asplenia. The anatomical variants described were identified and assessed in a 51-year old female from a computed-tomography angiography (CTA) images with 3D-volume rendered reconstructions during her follow-up after hysterectomy. The reconstructed CTA images showed a DAA with the left common carotid artery stemming from the hypoplastic left aortic arch while the left subclavian artery originated from a KD in the descending thoracic aorta. A symmetric arrangement of the aortic arch branches was demonstrated, comprising a full vascular ring. Since the patient had been completely asymptomatic and with no symptoms of compression of the esophagus or trachea, no surgical management was advised. The abdomen CTA imaging revealed absence of the celiac trunk with direct origin of the common hepatic and the left gastric artery from the superior mesenteric artery as well as asplenia. We presented a case of asymptomatic DAA of completely patent arches with the right vertebral artery branching separately of and the left SCA originating from KD in the descending aorta. The term KD can be identified also in other arch anomalies than the one originally described. Since anatomical anomalies can be frequently combined, thorough imaging inspection with CTA of both thorax and abdomen is suggested.