Double Aortic Arch Research Articles

Concurrent double aortic arch and circumflex aorta repair in a symptomatic child: a case report

Double aortic arch with circumflex aorta is a rare type of vascular ring. Symptoms result from external compression of the trachea and esophagus. The best surgical approach for patients with double arch and circumflex aorta is debated, and options include the highly complex aortic uncrossing procedure. Herein we describe the surgical treatment of a patient with concurrent double aortic arch and circumflex aorta by division of the non-dominant arch and ligamentum arteriosum, plication and posterior tacking of the diverticulum of Kommerell. This left thoracotomy approach provided complete symptom resolution.

A 33-Year-Old Man With Circumflex Aortic Arch and 4-cm Right Subclavian Artery Aneurysm

This is a 33-year-old man with CHARGE (coloboma, heart defects, atresia choanae, growth retardation, genital abnormalities, ear abnormalities) syndrome and a history of double aortic arch causing tracheal and esophageal compression. Cross-sectional imaging showed a 40-mm right subclavian artery aneurysm with a dominant right vertebral artery coming off it, an aberrant left subclavian artery, and separate origins of the bilateral common carotid arteries from the aortic arch. He underwent total aortic arch replacement, providing a left-sided arch to relieve the compression of the trachea and esophagus with bypass to the right axillary artery and reimplantation of the vertebral arteries and bilateral common carotid arteries. https://www.jvascsurg.org/cms/asset/e03d8a09-b06a-40bd-93b5-aa10b3dc9e62/mmc1.mp4Loading ... Download .mp4 (150.74 MB) Help with .mp4 files Video 1

Evaluation of cardiovascular morphology and associated anomalies in patients with crossed pulmonary arteries on multidetector computed tomography angiography.

The present study sought to study morphology and associated cardiovascular anomalies in patients with crossed pulmonary arteries on multidetector computed tomography (CT) angiography. We retrospectively evaluated all CT angiography studies performed at a tertiary medical center from January2014 to December2021 to identify patients with crossed pulmonary arteries. The associated cardiovascular anomalies as identified on CT angiography were evaluated. Out of 4773 patients who had undergone CT angiography for evaluation of congenital heart diseases, we identified 24 (0.5%) patients (18 males; mean age: 7.7 years) with crossed pulmonary arteries. Tetralogy of Fallot was associated in seven (29.17%) patients, double outlet right ventricle in one (4.17%) patient, and common arterial trunk in four (16.67%) patients. An aortopulmonary window was seen in three (12.5%) patients while atrial septal defect and ventricular septal defect were seen in two (8.33%) and 16 (66.67%) patients, respectively. Aortic arch anomalies were present in 16 (66.67%) patients including interrupted aortic arch and coarctation of aorta with hypoplastic aortic arch seen in two (8.33%) patients each. A double aortic arch with an atretic left arch was seen in one (4.17%) patient. Coronary artery anomalies were seen in three (12.5%) patients. Crossed pulmonary arteries are a rare anomaly and their presence suggests the coexistence of a variety of cardiovascular anomalies, including aortic arch anomalies and outflow tract malformations. Comprehensive CT angiography-based evaluation of cardiovascular morphology is imperative in the presence of crossed pulmonary arteries to facilitate presurgical planning.

A case of pulmonary artery sling, unilateral lung hypoplasia, and congenital heart disease

Vascular rings are rare congenital anomalies that primarily result from an embryological derangement of the paired aortic arches or branching pulmonary arteries. They make up <1% of all cardiac defects. Double aortic arch and right-sided aortic arch with the left ligamentum arteriosum are the most common types, making up 85– 95% of cases, with pulmonary artery slings making up about 10% of cases. The prevalence is estimated as 59 per million children with very few cases reported worldwide. The clinical presentation is variable and this includes severe acute life-threatening episodes, recurrent apnea, severe respiratory distress, feeding difficulties persistent cough, wheezing, or stridor. In some cases, affected individuals may be completely asymptomatic. A high index of suspicion is needed to suspect and diagnose vascular rings as they are uncommon but surgically treatable. We present a case of a 5-day-old term neonate referred to our facility with respiratory distress associated with stridor which started 12 h post-delivery.

Double aortic arch with aortic arch diverticulum leading to the use of tracheal intubation in a newborn.

Double aortic arch with aortic arch diverticulum leading to the use of tracheal intubation in a newborn.

Evaluation of aortic arch morphologies by computed tomographic angiography in Turkish population.

Background The aim of this study was to evaluate the aortic arch morphologies in the Turkish population using the computed tomography angiography technique. Methods Between August 2009 and August 2019, a total of 2,037 (1,003 males, 1,034 females; mean age: 52.8±20.3 years; range, 3 months to 100 years) thoracic computed tomography angiography scans were retrospectively analyzed. The findings were classified as described previously in the literature. The prevalence of aortic arch morphologies and possible relationship with sex were analyzed. The prevalence of variations reported in previous studies was compared with the current study. Results The normal aortic arch pattern (type A), observed in 1,562 cases (76.7%), was determined statistically significantly more in males than females (p<0.05). The most common variation, bovine aortic arch (type B1) which observed in a total of 315 cases (15.5%), was determined statistically significantly more in females than males (p<0.05). The second most frequent variation, in which the left vertebral artery originates directly from the aortic arch (type C1) was detected in 97 cases (4.7%). There was also observed to be aberrant right subclavian artery in 21 cases (1%), right-sided aortic arch variation in seven cases (0.4%), and double aortic arch anomaly in four cases (0.1%). In terms of the reported frequency of type B variation, a significant difference was determined between the current and previous studies in Türkiye (p<0.05). Conclusion With the largest sample size to date, this study provides comparative information about the prevalence of aortic arch patterns in the Turkish population.

135 A prenatally diagnosed double aortic arch case

Introduction: According to theory by Edwards, it is accepted that in the embryo the aorta is divided into the right and left arches of the ascending aorta and merges with each other at the descending aorta level, and it is anatomically present in the form of a double aortic arch located in the central position at anterior of the spine. Thus, complete vascular ring is formed that surrounds the trachea and esophagus with left and right-sided archs. The right and left aortic arches are each divided into two vessels; these are the right and left common carotid and subclavian arteries, respectively. The left and right pulmonary arteries are connected to the right and left ductus arteriiosus in the subclavian arteries region and to the left and right sided aortic arches, respectively. Therefore, the normal or abnormal development of aortic arch branching depends on which of the left or right aortic arches will regress or continue during embryological development. Methods: In our case, two main vessels, one of which is hypoplasic, originating from the left ventricle, were observed in cardiac ultrasonography performed to the pregnant woman, who was referenced from an external center. Discussion: The patient was followed up by us and gave birth by normal vaginal route at 37 weeks of gestation and the baby was diagnosed with double aortic arch in postnatal MRG. The newborn was followed up by pediatric cardiologists. Conclusions: Double aortic arch anomaly is resulted from both right and left aortic arches maintaining their existence without regressing. While the left ductus arteriosus continues to exist, the right side regresses. Each aortic arch gives branch to a common subclavian and carotid artery. The double aortic arch creates a tight vascular ring around the trachea, which may require postnatal surgery.

A new association of Oculoauriculovertebral spectrum and persistent fifth aortic arch -double lumen aorta: a case report

BackgroundOculo-auriculo-vertebral spectrum is a heterogeneous group of genetic disorder, also known as Goldenhar Syndrome, which has several phenotypic features including craniofacial anomalies, cardiac, vertebral and central nervous system defects. Cardiovascular anomalies include ventricular septal defects, atrial septal defects, patent ductus arteriosus, Tetralogy of Fallot, double outlet right ventricle, aberrant right subclavian artery, coarctation of aorta, transposition of the great arteries, double inlet left ventricle, cor triatriatum, pulmonary artery stenosis, aortic stenosis, persistent left superior vena cava, partially or totally abnormal pulmonary venous return and bicuspid aortic valve. Persistent fifth aortic arch, also named as double lumen aortic arch, is a very rare cardivascular anomaly and usually associate other cardiac defects.Case presentationWe present a 7 month old patient with oculo-auriculo-vertebral spectrum signs as facial asymmetry, short neck, choanal atresia, cleft palate, bilateral preauricular skin tags, bilateral hypoplastic ear lobes, epibulbar dermoid cyst, rib, vertebrae and cardiovascular anomalies. Cardiovascular anomalies detected with echocardiography and computed tomography were malalignment ventricular septal defect and double lumen aorta, known as persistent fifth aortic arch.ConclusionVarious cardiovascular anomalies may accompany Goldenhar Syndrome. We present a case with persistent fifth aortic arch and Oculo-auriculo-vertebral spectrum and this is a new association that was not reported before in the literature.

Fetal echocardiographic markers to differentiate between a right and double aortic arch.

To describe the fetal echocardiographic features of a double aortic arch (DAA) and secondly, to assess the performance of these features to differentiate between a right aortic arch with left duct (RAA-LD) in a blinded cohort of vascular rings. Review of records to identify surgically confirmed cases of DAA diagnosed prenatally from 2014 to 2018 (cohort-A). Prenatal echocardiograms were reviewed and the segments of the aortic arches anterior and posterior to the trachea, aortic isthmuses and the presence/absence of the Z-sign were described. The utility of these markers were assessed in a separate cohort (B) of fetuses with surgically confirmed cases of DAA or RAA-LD. Cohort-A comprised 34 cases with DAA; there was a dominant RAA in 32/34 (94%) and balanced left aortic arch (LAA) and RAA in two cases. The proximal LAA was seen in 29/34 (85%), distal LAA in 15/34 (44%) and the LAA aortic isthmus in 4/34 (12%). The "Z" configuration was present in 29/34 (85%) cases. The most predictive marker for DAA in cohort-B was the Z-sign (sensitivity: 100%, specificity: 81%). The "Z" sign is a useful differentiator between RAA-LD and DAA. The absence of visualization of the left aortic isthmus does not preclude the presence of a DAA.

Importance of Acute Anterior Angulation in Double Aortic Arch Needing Attention at Primary Surgery.

BackgroundVascular rings are rare congenital abnormalities of the aortic arch. There are many embryological variants including a double aortic arch. In symptomatic children, division of ring and release of airway structures may be sufficient. Persistence of symptoms can be related to an anterior angle formed between the two arches. The aim of this study is to evaluate the clinical efficacy in improving symptoms and on changing this angle at the primary surgery.MethodsAll children who had surgery for double aortic arch between 2005 and 2020, were studied. Relevant factors were analyzed for persistent symptoms including anatomical substrates and surgical details.ResultsA total of 87 out of 224 children had surgery for a double aortic arch. At presentation, airway symptoms (n = 74/87) were more common than esophageal symptoms (n = 27/87). Early onset symptoms within 1 year were seen in 49 children. In addition to division of one arch, surgical steps also included realigning the anterior left arch, thereby eliminating the acute angle in 36 children (after 2014). After surgery, symptom relief within 12 months following surgery was seen in 64% of children (56 out of 87) but in 27 out of 36 children (75%) with additional surgical modification, as against 29 out of 51 (57%) in those with division of the arch. Symptoms persisted beyond 1 year needing reintervention in eight children.ConclusionAnterior arch angulation plays an important role in double aortic arch by causing a “nutcracker” phenomenon. Repair in double aortic arch should consider this aspect and include modification of surgical steps by realigning the corresponding aortic arch branches and an anterior pexy in selected cases.

Double aortic arch with atretic proximal left arch.

Aortic arch anomalies are rare and represent about 1% of all congenital cardiac lesions. Double aortic arch with atretic proximal left arch is one of the rare causes of respiratory symptoms in neonates, often missed by preoperative imaging studies. Preoperative identification and differentiating this entity from other arch anomalies is imperative to plan appropriate surgical management.

Contemporary surgical outcome and symptomatic relief following vascular ring surgery in children: effect of prenatal diagnosis.

Our goal was to describe postoperative complications and outcomes in a large contemporary cohort of children with an isolated double aortic arch (DAA) or a right aortic arch (RAA) with left arterial ligament and to assess the impact of foetal diagnosis on outcomes. We performed a retrospective analysis of all patients who underwent surgery for DAA or RAA with left arterial ligament between 2005 and 2019. A total of 132 children were operated on for a DAA (n = 77) or a RAA (n = 55). Prenatal diagnosis was made in 100/132 (75.8%). Median age at surgery for DAA was 5.0 (1.7-13.3) months and for RAA was 13.9 (6.4-20.1) months. There was no difference in the age at surgery between the prenatal and postnatal cases (8.6 [4.0-15.6] vs 5.4 months [1.8-17.7]; P = 0.37). No surgical deaths occurred. Vocal cord palsy was the most common complication, occurring in 12/132 (9%): of these, 11 resolved spontaneously and 1 required a temporary tracheostomy. Logistic regression demonstrated that older age at operation was the only predictor for a postoperative complication (P = 0.02). Overall, 21/67 (31%) of prenatally detected, symptomatic cases reported residual symptoms/signs 1 year after surgery compared to 18/28 (64%) of postnatally detected cases. Postnatal diagnosis was associated with persistent postoperative symptoms/signs [P = 0.006, odds ratio = 3.9 (95% confidence interval 1.5-9.4)]. Surgery to relieve a vascular ring resolves trache-oesophageal compressive symptoms in most cases, but parents/patients should be aware that symptoms/signs may persist in the first postoperative year despite effective release of the vascular ring. Earlier surgery and prenatal diagnosis may improve outcomes.

Stridor After Tracheoesophageal Fistula Repair: Where Is the Lesion?

Stridor After Tracheoesophageal Fistula Repair: Where Is the Lesion?

Minimally Invasive Surgical Technique in Double Aortic Arch with Distal Atretic Left-Side in Infant: From a Single-Surgeon Clinical Experience

Background: Double aortic arch (DAA) with distal left-sided aortic arch atresia (LAAA) can form complete vascular ring by ligamentum connection. We aimed to introduce an uncommon DAA-LAAA diagnosis and treatment by the minimally invasive surgical technique (MIST). Methods: We retrospectively reviewed 7 cases of DAA-LAAA that were treated from January 2017 to July 2021. All infant patients underwent surgical repair by minimally invasive surgical technique. Mean follow-up was 14.43 months (range, 5–21 months). Results: There were seven patients with DAA-LAAA, including six males and one female. Median age was 19.29 months (range, 9.0–29.0 months). Median weight was 11.30 kg (range, 9.6–13.0 kg). Three patients were found severe tracheal compression by cardiac computed tomography angiography (cCTA). Six patients with isolated DAA-LAAA were performed operations through left subaxillary minithoracotomy, and one patient with ventricular septal defect (VSD) was performed operation concurrently under the cardiopulmonary bypass (CPB) through right subaxillary minithoracotomy. All patients had symptom improvement in the postoperative period and discharged successfully. Follow-up data showed good results in short-term. Conclusions: We introduce a new surgical pathway for DAA-LAAA treatment with good symptomatic relief in short-term. MIST is a safe, feasible and economical approach for infant patients.

3D black blood cardiovascular magnetic resonance atlases of congenital aortic arch anomalies and the normal fetal heart: application to automated multi-label segmentation

BackgroundImage-domain motion correction of black-blood contrast T2-weighted fetal cardiovascular magnetic resonance imaging (CMR) using slice-to-volume registration (SVR) provides high-resolution three-dimensional (3D) images of the fetal heart providing excellent 3D visualisation of vascular anomalies [1]. However, 3D segmentation of these datasets, important for both clinical reporting and the application of advanced analysis techniques is currently a time-consuming process requiring manual input with potential for inter-user variability.MethodsIn this work, we present novel 3D fetal CMR population-averaged atlases of normal and abnormal fetal cardiovascular anatomy. The atlases are created using motion-corrected 3D reconstructed volumes of 86 third trimester fetuses (gestational age range 29-34 weeks) including: 28 healthy controls, 20 cases with postnatally confirmed neonatal coarctation of the aorta (CoA) and 38 vascular rings (21 right aortic arch (RAA), 17 double aortic arch (DAA)). We used only high image quality datasets with isolated anomalies and without any other deviations in the cardiovascular anatomy.In addition, we implemented and evaluated atlas-guided registration and deep learning (UNETR) methods for automated 3D multi-label segmentation of fetal cardiac vessels. We used images from CoA, RAA and DAA cohorts including: 42 cases for training (14 from each cohort), 3 for validation and 6 for testing. In addition, the potential limitations of the network were investigated on unseen datasets including 3 early gestational age (22 weeks) and 3 low SNR cases.ResultsWe created four atlases representing the average anatomy of the normal fetal heart, postnatally confirmed neonatal CoA, RAA and DAA. Visual inspection was undertaken to verify expected anatomy per subgroup. The results of the multi-label cardiac vessel UNETR segmentation showed 100% per-vessel detection rate for both normal and abnormal aortic arch anatomy.ConclusionsThis work introduces the first set of 3D black-blood T2-weighted CMR atlases of normal and abnormal fetal cardiovascular anatomy including detailed segmentation of the major cardiovascular structures. Additionally, we demonstrated the general feasibility of using deep learning for multi-label vessel segmentation of 3D fetal CMR images.

Diagnosis and Outcomes of Isolated Vascular Rings and Pulmonary Artery Slings in a Tertiary Paediatric Institution – 20-Year Experience

Vascular rings and slings, including right aortic arch with aberrant left subclavian artery (RAA-ALSCA), double aortic arch (DAA) and pulmonary artery sling (PAS), are congenital anomalies that may cause airway or oesophageal compression. Prenatal detection has improved, yet comparisons in clinical outcomes of antenatal versus postnatally diagnosed cases are not well described. This study describes a state-wide tertiary paediatric institution’s clinical profile and mid-term outcomes of prenatal versus postnatally diagnosed isolated vascular rings.

Is secondary tracheomalacia associated with airway inflammation and infection?

Recurrent lower respiratory tract infections are among the most prevalent symptoms in secondary tracheomalacia due to mediastinal vascular anomalies (MVAs). It is not known whether this condition could result in persistent lower respiratory tract inflammation and subclinical infection. A retrospective study was performed on records of children with tracheomalacia due to MVAs and recurrent respiratory infections who underwent computed tomography scan, bronchoscopy, and bronchoalveolar lavage (BAL) as part of their clinical evaluation. Thirty-one children were included in the study: 21 with aberrant innominate artery, four with right aortic arch, one with double aortic arch, and five with aberrant innominate artery associated with right aortic arch. Cytological evaluation of bronchoalveolar lavage fluid showed increased neutrophil percentages and normal lymphocyte and eosinophil proportions. Microorganism growth was detected in 13 BAL samples, with a bacterial load ≥104 colony-forming units/mL in eight (25.8%) of them. Most isolates were positive for Haemophilus influenzae. Bronchiectasis was detected in four children, all with BAL culture positive for H. influenzae. Four patients underwent MVA surgical correction and 27 conservative management, i.e., respiratory physiotherapy in all and high-dose amoxicillin/clavulanic acid (40 mg/kg/day) for 2-4 weeks in those with significant bacterial growth. There was an excellent outcome in most of them. Neutrophilic alveolitis is detectable in secondary tracheomalacia but is associated with a clinically significant bacterial load only in a quarter of the patients. Caution should be used regarding inappropriate antibiotic prescriptions to avoid the emergence of resistance, whilst airway clearance maneuvers and infection preventive measures should be promoted.

Diagnosing the unexpected: double aortic arch with vascular ring in the fifth decade of life.

A 42-year-old man without cardiovascular risk factors was referred for cardiology consultation due to atypical chest pain. He had a previous history of allergic asthma, without recent exacerbations. Coronary computed tomography (CT) angiography excluded coronary artery disease. However, the cardiac CT disclosed a double aortic arch (DAA) (Panels A–C: 3D volume-rendered CT scans). This DAA presented a co-dominant configuration, with the left subclavian (grey square) and left common carotid arteries (grey dot) arising from the left arch, and the right arch originating the right subclavian (black square) and right common carotid (black dot) arteries (Panels A and B). The anomalous conformation resulted in a complete vascular ring (Panel C), without producing compression of the trachea (asterisk, Panel D). A reduction of the endoluminal caliber of the oesophagus was noted, but the patient...

Double aortic arch: an elusive diagnosis in a child with CHD.

An 18-month-old male with pulmonary atresia and ventricular septal defect presented with stridor after neonatal systemic-to-pulmonary artery shunt surgery, that persisted on follow-up. CT angiography revealed a vascular ring with balanced double aortic arch.

Prenatal Ultrasound Diagnosis of Double Aortic Arch versus Right Aortic Arch Variant in Vascular Ring Formation – Case Report and Review of the Literature

Prenatal Ultrasound Diagnosis of Double Aortic Arch versus Right Aortic Arch Variant in Vascular Ring Formation – Case Report and Review of the Literature