Mirror-image Branching Research Articles

Contemporary Midterm Outcomes in Pediatric Patients Undergoing Vascular Ring Repair

This single-institution study assessed themidterm outcomes of patients undergoing complete vascular ring (CVR) repair and the need for reintervention. The study included all patients who underwent surgical repair of an isolated CVR from 1996 to 2018 at our institution. Patients who underwent concomitant intracardiac repair were excluded. Data analysis included demographics, type of anomaly, other congenital heart disease, clinical symptomatology, operative technique, perioperative outcomes, reoperation rates, and mortality. CVR repair through open thoracotomy was performed in 148 patients (80 boys [54%]), median age, 1.04 years (interquartile range, 0.4-5.2 years), and median weight, 12.8 kg (interquartile range, 7.5-26.5 kg). The cohort included 72 patients with double aortic arch (DAA), 69 with right aortic arch (RAA) with aberrant left subclavian artery and left ligamentum arteriosum (LLA), 5 with RAA with left descending aorta and LLA, and 2 with RAA with mirror-image branching and LLA. There was 1 outpatient perioperative death (0.7%) 15 days postoperatively. Perioperative complications occurred in 20 patients (14%): 18 (12%) with chylothorax (3 required reintervention), 1 pneumothorax, and 1 vocal cord paresis. Two of 36 patients (5.5%) without primary diverticulum resection required reoperation and subclavian reimplantation at 3 and 4 years, and 1 patient required aortic translocation 9 years later for persistent symptoms. Freedom from reoperation after CVR repair was 93% at 5 years and 86% at 10 years. A small proportion of patients who do not undergo diverticulum resection and aberrant left subclavian artery reimplantation at the time of CVR repair will require reintervention in the future.

Computed tomography diagnosis of truncus arteriosus type IV: a case report

Truncus arteriosus (TA) is an uncommon congenital cardiac anomaly of which type IV is considered a rare variant. Recognition is crucial for proper treatment planning. The prognosis without treatment is poor. Echocardiography alone may not be useful in evaluation. Computed tomography (CT) finding is complicated. We report an 18 months child with ventricular septal defect (VSD), diagnosed on echocardiography, and further review by CT showed VSD with descending thoracic aorta giving rise to the pulmonary arteries suggestive of pseudo truncus (Collet and Edwards Truncus arteriosus Type IV) and right sided aortic arch with mirror image branching.

Ductus arteriosus location in relation to aortic arch position, branching pattern, and viscero-atrial situs

To investigate ductus arteriosus (DA) location in relation to viscero-atrial situs and branching pattern of the right aortic arch (RAA). Computed tomography (CT) images of paediatric patients (May 2015 to July 2017, n=352) referred for evaluation of cardiovascular malformations were reviewed retrospectively. Patients with RAA were identified and images were analysed for documentation of the viscero-atrial situs, the branching pattern, DA location, and associated malformations. The study included 64 RAA patients, 45 situs solitus, 11 situs ambiguous, and eight situs inversus. Mirror-image branching was observed in 34 situs solitus, 11 situs ambiguous, and seven situs inversus patients. Aberrant left subclavian artery (ALSCA) was present in 10 situs solitus and one situs inversus patient. One patient with situs solitus had isolated left subclavian artery. The location of the DA was identifiable in 32 patients (19 left and 13 right). In situs solitus with mirror-image branching, the DA was left in 10, right in four patients. In situs inversus and ambiguous with mirror-image branching, the DA was right in eight and left in two patients. In patients with ALSCA, the DA was left in seven and right in one patient. The location of the DA was identifiable in 85 patients with left aortic arch (left-sided in 84, right-sided in only one patient). The location of DA in RAA varies according to viscero-atrial situs and branching pattern. In mirror-image branching, the DA is more commonly left-sided with situs solitus and more right-sided with abnormal viscero-atrial situs.

Prenatal echocardiographic assessment of right aortic arch.

To report our experience with fetal diagnosis of right aortic arch (RAA) variants based on the ductus arteriosus (DA) anatomy and brachiocephalic vessel branching pattern in relation to the trachea, and to establish whether the echocardiographic 'V-shaped' or 'U-shaped' appearance of the junction between the DA and aortic arch (AA) in the fetal upper mediastinal view is sufficiently accurate for assessment of fetal AA anatomy. This was a retrospective study of pregnancies with a prenatal diagnosis of fetal RAA that had postnatal confirmation of AA anatomy, referred to our tertiary center during 2011-2017. Prenatal and postnatal medical records, including echocardiographic and computed tomography (CT)/magnetic resonance imaging (MRI) scan reports, were reviewed, and cardiac and extracardiac abnormalities and the results of genetic testing were recorded. Of 55 consecutive pregnancies with a prenatal diagnosis of fetal RAA, six were lost to follow-up, one was terminated and three were excluded due to lack of postnatal confirmation of AA anatomy. Of the remaining 45 pregnancies, AA anatomy was assessed postnatally by CT in 39, by MRI in one and by direct examination at cardiac surgery in five. A U-shaped appearance was found in 37/45 (82.2%) patients, all of which had a complete vascular ring (CVR). Of these 37 patients, on postnatal confirmation, 21 (56.8%) had RAA with Kommerell's diverticulum, left posterior ductus arteriosus (LPDA) and aberrant left subclavian artery (ALSA) (RAA/LPDA/ALSA), 11 (29.7%) had a double AA (DAA), four (10.8%) had RAA with Kommerell's diverticulum, LPDA and mirror-image (MI) branching (RAA/LPDA/MI), and one (2.7%) had RAA with Kommerell's diverticulum, LPDA and aberrant left innominate artery (ALIA) (RAA/LPDA/ALIA). A V-shaped appearance was found in 3/45 (6.7%) patients, all of which had RAA with right DA not forming a CVR and MI branching. In the 5/45 (11.1%) fetuses with neither U- nor V-shaped appearance, RAA with left anterior DA arising from the left innominate artery and MI branching, not forming a CVR, was found. Twelve (26.7%) fetuses had a congenital heart defect (CHD). RAA forming a CVR (U-shaped appearance) was associated with a septal defect in 6/37 (16.2%) fetuses, while RAA not forming a CVR (V-shaped appearance or no U- or V-shaped appearance) was associated with major CHD in 6/8 (75.0%) fetuses. In fetuses with RAA, V-shaped appearance of the junction between the DA and AA indicates only that the transverse AA and DA run together on the same side of the thorax (trachea) while a U-shaped appearance is always a sign of a CVR. Among fetuses with a CVR, RAA/LPDA/MI is more frequent than described previously. Finally, RAA forming a CVR is not usually associated with complex CHD, as opposed to RAA not forming a CVR. Copyright © 2018 ISUOG. Published by John Wiley & Sons Ltd.

Prenatal echocardiographic diagnosis of right aortic arch and its branch type

Objective To investigate the value of prenatal echocardiographic diagnosis of anomalies of the right aortic arch(RAA) and its branch type, and provide rapid diagnostic methods. Methods A retrospective analysis of 55 cases of RAA of prenatal ultrasound diagnosis results, and their imaging characteristics were summarized. Results There were 55 cases of anomalies of the RAA, included 42 cases of RAA with aberrant left subclavian artery(RAA-ALSA) and 13 cases of RAA with mirror image branching pattern(RAA-MB), 1 case of double aortic arch(DAA) was misdiagnosed as RAA. The prenatal echocardiographic characteristics of RAA-ALSA: in three-vessel-aortic arch view, the first branch of the aortic arch was the left carotid artery, which ran in front of the trachea, and ALSA ran behind the trachea and esophagus. The prenatal echocardiographic characteristics of RAA-MB: the first branch of the aortic arch was the brachiocephalic trunk, which ran in front of the trachea and was divided to the left carotid artery and the left subclavian artery. Conclusions Prenatal ultrasound can qunickly and accurately diagnose anomalies of the right aortic arch and its branch type according to the direction, thickness and secondary branches of the first branch of the aortic arch. The location of arterial duct is helpful to judge the formation of vascular ring. Key words: Ultrasonography, prenatal; Right aortic arch; Vascular ring

Fetal aortic arch and its branches anomalies: prenatal ultrasound, vascular cast and gene detection

Objective To explore the application values of prenatal ultrasound, vascular cast in the diagnosis of fetal aortic arch and its branches anomalies and to analyze the genetic characteristics by gene detection. Methods Twenty-two cases of the vascular cast specimens of the fetal aortic arch and its branches anomalies were analyzed and studied by comparing with their prenatal ultrasonography. Then the characteristics of each type of fetal aortic arch and its branches anomalies, the missed diagnosis and misdiagnosis were summarized and the results of their gene detection were also analyzed. Results The 22 cases of fetal aortic arch and its branches anomalies were as follows: 2 cases of double aortic arch showed the ascending aorta was divided into two branches after converging as the descending aorta. Three cases were left aortic arch with aberrant right subclavian artery. Twelve cases were right aortic arch: 8 cases were right aortic arch with mirror-image branching, 3 cases were right aortic arch with aberrant left subclavian artery, 1 case was right aortic arch with isolated left subclavian artery. Of the 8 right aortic arch with mirror-image branching, 3 cases of left arterial duct showed the vertical walking between the fusion site of the left innominate artery and the pulmonary artery. Right aortic arch with aberrant left subclavian artery with arterial duct showed U shaped vascular ring. Five cases were other types, including 2 cases of the coarctation of aortic arch, 1 case of interrupted aortic arch, 1 case of pulmonary artery sling, and 1 case of abnormal origin of right pulmonary artery. The ultrasonic missed diagnosis were the 6 deformities: 3 cases of arterial duct and 3 cases of aberrant subclavian artery. The ultrasonic misdiagnosis were the 5 deformities: 2 cases of arterial duct location, 1 case of aberrant subclavian artery, 1 case of isolated left subclavian artery, and 1 case of the coarctation of aortic arch. Genetic test results: In the 18 cases of the genetic detection, 2 cases were positive, 1 case was 22q11.2 microdeletion syndrome and 1 case was carrying KMT2D gene variant. Conclusions There are various kinds of fetal aortic arch and its branches anomalies, which are often associated with intracardiac malformations and venous branches variation. And prenatal ultrasound is of great value in diagnosing them.Vascular cast can visually display their characteristic changes, which is helpful to improve the differential diagnosis of the different aortic arch and its branches anomalies. The detailed genetic detection can improve the further understanding of its etiology. Key words: Ultrasonography, prenatal; Fetus; Aortic arch anomalies; Vascular cast; Gene detection

Complex Vascular Ring Diagnosed on Cardiovascular MR in a 3-Day-Old Infant.

Prenatal ultrasonography in the early third trimester showed an unusual branching pattern of the right aortic arch. Echocardiography performed 4 h after birth showed the right aortic arch with mirror-image branching, patent ductus arteriosus, and patent foramen ovale. Because the location of the ductus arteriosus was unclear on echocardiography, cardiovascular magnetic resonance imaging was performed 3 days after birth. Advanced techniques including contrast-enhanced time-resolved magnetic resonance angiography and 3D time-of-flight magnetic resonance angiography allowed accurate diagnosis of a vascular ring comprising ascending and descending aorta, right aortic arch with mirror-image branching, and diverticulum of Kommerell giving rise to a left ligamentum arteriosum. The infant had hiccups, but no other symptoms. The esophagram was negative for obstruction. The infant was closely monitored; however, she developed esophageal obstruction at 7 months of age because of the vascular ring. She underwent lysis of the left ligamentum arteriosum followed by aortopexy for relief of esophageal obstruction. This report shows the utility of neonatal cardiovascular magnetic resonance imaging to evaluate complex congenital aortic arch anomalies.

Abstract 137: Right Cerebral Insufficiency Associated with Right Aortic Arch with Mirror-Image Branching: a Case Study

We report a case of right cerebral insufficiency in a 65-year-old woman with right aortic arch with mirror-image branching. The patient was found to have intermittent occlusion at the origin of the right common carotid artery between the aortic arch and the sternoclavicular joint. Duplex showed intermittent bizarre carotid artery waveforms directly related to patient positioning. The patient underwent a transposition of the right common carotid to the right subclavian artery with subsequent resolution of symptoms. Right aortic arch with mirror-image branching can rarely present with right cerebral insufficiency due to intermittent mechanical occlusion of the right common carotid artery.

Characteristics of prenatally detected right aortic arch cases in a single institution

This study aimed to elucidate the diagnostic process, the associated anomalies and the perinatal outcomes of right aortic arch (RAA) in a group of low-risk patients. The obstetric imaging database and digital patient files were reviewed between January 2015 and June 2016. There were 12 RAA cases detected prenatally. Seven foetuses had an aberrant left subclavian artery and one foetus had mirror image branching. The prevalence of RAA was 1.8 in 1000. Invasive prenatal diagnosis was offered to patients and seven tests were performed. There was one associated cardiac anomaly (8.3%) and one extra-cardiac anomaly (8.3%) in the same foetus which cordocentesis revealed trisomy 21. There were also two 22q11.2 microdeletion cases with isolated RAA with aberrant left subclavian arteries. All patients have given live births except one patient has chosen a termination of pregnancy for 22q11.2 microdeletion. The median follow-up time of the newborns was 21 months. None of the babies required surgery for RAA during follow-up. All the babies are alive, except for the one with trisomy 21, who dead at 5 months due to the surgical complications of an oesophageal atresia operation. Patients with an RAA foetus should be offered for foetal karyotyping and analysis for 22q11.2 microdeletion. When isolated, RAA has usually a good prognosis and rarely requires cardiac surgery.Impact StatementWhat is already known on this subject? Prenatal diagnosis of RAA should prompt a detailed cardiac and extra-cardiac ultrasound examination. When isolated, RAA is associated with 22q11 deletion and aneuploidies in 4.6% and 5.1%, of cases respectively.What the results of this study add? Our study showed that 28.5% of isolated RAA cases are associated with 22q11.2 microdeletion. Cardiac surgery is rarely required when RAA is an isolated anomaly.What the implications are of these findings for clinical practice and/or further research? Invasive prenatal testing for karyotypes and 22q11.2 microdeletion should be offered to patients with RAA, even in the case of an isolated one. Further larger studies are needed to confirm this finding.

Aortic arch anomalies detected in foetal life by echocardiography

Aortic arch anomalies refer to congenital malformations of position or branching pattern of the aortic arch. To-date, only a few small studies have documented prenatal detection of aortic arch anomalies. In this article, we share our experience in detecting aortic arch anomalies. Foetal echocardiograms, clinic and genetic histories of 33 patients who had been diagnosed with aortic arch anomaly from 2007 to 2015 were reviewed. In 15 patients, right aortic arch with mirror image branching; in 13 patients, right aortic arch with left ductus arteriosus and aberrant left subclavian artery; in three patients, left aortic arch with aberrant right subclavian artery; in one patient bilateral ductus and right aortic arch with aberrant left subclavian artery and in one patient double aortic arch were detected. In a patient with isolated right aortic arch, 22q11 microdeletion had been revealed. Given this data, we strongly suggest foetal karyotype analysis when aortic arch anomalies are identified.Impact StatementWhat is already known on this subject: The data about the prenatal diagnosis of aortic arch anomalies are limited.What the results of this study add: In our study, 653 patients were examined by foetal echocardiography during the study period. Thirty three patients who had been diagnosed with aortic arch anomaly prenatally and confirmed after delivery were enrolled in the study. In 15 patients, right aortic arch with mirror image branching; in 13 patients, right aortic arch with left ductus arteriosus and aberrant left subclavian artery; in three patients, left aortic arch with aberrant right subclavian artery; in one patient bilateral ductus and right aortic arch with aberrant left subclavian artery and in one patient double aortic arch were detected. Trisomy 18 was detected in the patient with bilateral ductus arteriosus and Di George syndrome (22q11 microdeletion) was determined in two patients with right aortic arch. While in the first patient, there were no other intracardiac anomalies; in the second patient with 22q11 microdeletion, Fallot tetralogy accompanied the right aortic arch.What the implications are of these findings for clinical practice and/or further research: This results showed that aortic arch anomalies can be associated with genetic anomalies even when they are found without other congenital heart disease. Given these data, we strongly suggest foetal karyotype analysis and genetic testing when aortic arch anomalies are identified.

Prenatal Diagnosis of Right Aortic Arch with Mirror Image Branching: Importance of the Three-vessel-trachea View

Prenatal Diagnosis of Right Aortic Arch with Mirror Image Branching: Importance of the Three-vessel-trachea View

Prenatal Diagnosis of Right Aortic Arch with Mirror Image Branching: Importance of the Three-vessel-trachea View: Editorial Comment

Prenatal Diagnosis of Right Aortic Arch with Mirror Image Branching: Importance of the Three-vessel-trachea View: Editorial Comment

Catheterization and surgical collaboration in the treatment of complex congenital heart disease with discontinuous pulmonary arteries.

A term, male neonate presents with pulmonary atresia, discontinuous branch pulmonary arteries (PA) arising from independent ducti arteriosus, and dextrocardia presents a challenging treatment plan. The right pulmonary artery (RPA) arose from the ascending aorta while the left pulmonary artery (LPA) arose from the left subclavian artery, with the proximal segments of each spaced about 1 cm apart, making primary unifocalization suboptimal. The cardiac anatomy included: dextrocardia with situs inversus, double outlet right ventricle (DORV) with malposed great arteries and pulmonary atresia, left sided inferior and superior venae cava, and a right aortic arch with mirror image branching. The neonate underwent cardiac catheterization on day of life 12 wherein the bilateral patent ducti arteriosus (PDA) were stented as an alternative to high-risk surgery. In addition, the pulsatile flow via the stents provided a stimulus for PA growth, facilitating transcatheter rehabilitation of distal PA hypoplasia, and optimizing later PA reconstruction. The patient underwent surgery at 13 months of age, with autologous tube creation to unifocalize the Pas and enable the Glenn procedure. Postoperative autograft stenosis was later managed via cardiac catheterization. This report describes the clinical management and utility of staged percutaneous intervention for complex anatomy.

Prenatal cardiac magnetic resonance imaging of right aortic arch with mirror image branching and retroesophageal left ductus arteriosus

Objective To evaluate the utility of fetal cardiac magnetic resonance imaging (MRI) in diagnosing right aortic arch with mirror image branching and retroesophageal left ductus arteriosus (RLDA). Methods This retrospective study included six infants diagnosed with right aortic arch with mirror image branching and RLDA postnatally by cardiac computed tomography (CT) that had fetal echocardiography (echo) and MRI initially performed. The six fetal MRI cases were examined using 1.5 T MRI unit. Steady-state free precession (SSFP) sequence and single-shot turbo spin echo (SSTSE) sequence were used to evaluate the fetal great vessels and airway. The gestational age of six fetuses at time of fetal MRI ranged from 23 to 35 weeks (mean, 26.7 weeks). Results Of six cases with mirror image right aortic arch and RLDA confirmed by postnatal CT, 4/6 were correctly diagnosed by fetal cardiac MRI and 3/6 were correctly diagnosed by prenatal echo. All six cases were not associated with other congenital heart defect. All ductus arteriosus were closed after birth. Conclusions Fetal cardiac MRI can be a useful adjunct for evaluating fetal right aortic arch with mirror-image and RLDA.

Prenatal ultrasonic diagnosis and differential diagnosis of isolated right aortic arch with mirror-image branching.

This study sought to evaluate the fetal echocardiography features of isolated right aortic arch (RAA) with mirror-image branching and to improve the rate and accuracy of prenatal diagnosis of this condition. We reviewed fetal echocardiograms from all cases of isolated RAA with mirror-image branching diagnosed at our institution between August 2012 and December 2015 and classified these cases into normal and abnormal types of ductus arteriosus based on the course of the arterial duct arch. We confirmed the diagnoses by postnatal echocardiography. A total of 11 cases of isolated RAA with mirror-image branching, with the left ductus and the descending aorta located on the left side of the spine, were diagnosed using fetal echocardiography. Ten cases involved normal ductus arteriosus, with the left ductus connecting the left pulmonary artery to the descending aorta, five of which were referred to our institution for suspicions of double aortic aorta. 1 case involved abnormal ductus arteriosus, with the left ductus connecting the left pulmonary artery to the left innominate artery. RAA with mirror-image branching can be detected via fetal echocardiography, which can reveal the relationship between of the aortic arch and the trachea and can enable the identification of the course of brachiocephalic branching. The identification of isolated RAA with mirror-image branching is crucial for distinguishing this condition from other types of aortic arch anomalies, particularly double aortic aorta, which can have a rather different prognosis.

Double Aortic Arch Causing Severe Tracheal Compression

A DOUBLE aortic arch results in a vascular ring that may compress the trachea and esophagus.1 Although the incidence of double aortic arch is unknown, they represent less than 1% of all congenital heart disease. Symptoms commonly include stridor, dyspnea, cough, recurrent upper respiratory tract infections, dysphagia, and feeding difficulty.1–3 Although more common in children, adults may present with new symptoms or chronic symptoms misdiagnosed as other diseases, as was the case in a 47-yr-old woman who presented with long-standing dyspnea (presumed for decades to be due to asthma) and dysphagia. Her symptoms had progressed to include new orthopnea with inability to lay supine and worsening dysphagia. Three-dimensional volume-rendered (A) and axial (B) contrast-enhanced computerized tomography demonstrated an incomplete double aortic arch with mirror image branching and an atretic left arch segment, with resulting compression and narrowing of the distal trachea and esophagus. After airway topicalization, severe tracheal compression was noted during awake fiberoptic intubation (C) performed in the sitting position. The 7.5-mm endotracheal tube was advanced past the area of tracheal compression, general anesthesia was induced with sevoflurane, and the patient was positioned supine. Operative repair typically consists of division of the lesser or atretic aortic arch via a left thoracotomy, resulting in postoperative symptom resolution.1–3 The potential for tracheal compression resulting in the inability to intubate must be assessed before induction of general anesthesia to ensure safe airway management (fig. left aortic arch [LAA], left common carotid [LCC], left subclavian artery [LSA], right aortic arch [RAA], right common carotid [RCC], right subclavian artery [RSA]).The authors declare no competing interests.

İzole Ayna Görünümü Dallanma ile Birlikte Sağ Aortik Ark ve Sağ Duktal Ark Prenatal Tanısı: Üç Damar Trakea Kesitinin Önemi

İzole Ayna Görünümü Dallanma ile Birlikte Sağ Aortik Ark ve Sağ Duktal Ark Prenatal Tanısı: Üç Damar Trakea Kesitinin Önemi

Unilateral Pulmonary Artery Agenesis with Right Sided Aortic Arch

Unilateral pulmonary artery agenesis (UPAA) is a rare congenital anomaly due to a malformation of the sixth aortic arch during embryogenesis. It can occur as isolated or may be associated with other congenital cardiovascular malformations. Right-sided aortic arch (RAA) is infrequently seen entity that can be related to various forms of congenital heart disease. Among these, with mirror-image branching of the major arteries is the most encountered subtype. We have presented here a rare case of UPAA with RAA and Kommerell’s diverticulum.

Congenital Variants and Anomalies of the Aortic Arch.

Congenital variants and anomalies of the aortic arch are important to recognize as they may be associated with vascular rings, congenital heart disease, and chromosomal abnormalities, and can have important implications for prognosis and management. The purpose of this article is to review cross-sectional imaging techniques used in the evaluation of the aortic arch, describe the embryology and anatomy of the aortic arch system, discuss aortic arch variants and anomalies, and review other malformations of the aortic arch, including interrupted aortic arch, hypoplastic aortic arch, and aortic coarctation. Aortic arch variants and anomalies will be reviewed in the context of a theoretical double aortic arch system. Arch anomalies can be associated with symptoms, such as dysphagia lusoria in the setting of left aortic arch with aberrant right subclavian artery. Arch variants that form a vascular ring, such as double aortic arch, can result in respiratory distress due to tracheal compression. Certain arch anomalies are strongly associated with congenital heart disease, including right aortic arch with mirror image branching. Other malformations of the aortic arch have important associations, such as type B interrupted aortic arch, which is associated with a locus 22q11.2 microdeletion. Noninvasive imaging at CT angiography and MR angiography allows for comprehensive evaluation of the aortic arch and branch vessels in relation to surrounding structures. Familiarity with the spectrum and imaging appearances of aortic arch variants, anomalies, and malformations is essential for accurate diagnosis and classification and to guide management. Online supplemental material is available for this article. ©RSNA, 2016.

Long-term outcomes of complete vascular ring division in children: a 36-year experience from a single institution.

Complete vascular rings are rare and cause tracheoesophageal compression. Following surgical division, some patients have persisting tracheomalacia. We aim to assess the long-term outcomes of complete vascular ring division. All patients (n = 132) who underwent surgical division of a complete vascular ring between 1978 and 2014 were identified from the hospital database and retrospectively reviewed. Complete vascular rings consisted of a double aortic arch (n = 80), right aortic arch with an aberrant subclavian artery and left ligamentum arteriosum (n = 50), right aortic arch with mirror image branching and left ligamentum arteriosum (n = 1), and a left aortic arch with right descending aorta and right ligamentum arteriosum (n = 1). Kommerell's diverticulum was identified in 10 patients. Preoperative tracheomalacia was identified via bronchoscopy in 25 patients. Concomitant tracheal reconstruction was not performed in any patient. Kommerell's diverticulum was resected in 1 patient. The hospital mortality rate was 1.5% (2/132). There were no late deaths. The overall survival rate was 98.3 ± 1.2% (95% CI: 93.4, 99.6) at 20 years. Postoperatively, persistent tracheal compression was reported in 3 patients, and tracheomalacia in 16 patients. The rate of freedom from reoperation was 88.6 ± 4.0% (95% CI: 77.9, 94.3) at 20 years. No patient required tracheal surgery during the follow-up period. Follow-up was 92% (121/132) complete, with a median follow-up of 11.4 years (range 44 days to 36 years). At the last follow-up, 7 patients had mild tracheomalacia. Outcomes of division of a complete vascular ring are excellent. Tracheomalacia often improves following division of the vascular ring. Respiratory symptoms following complete vascular ring division are uncommon.