Diagnosis Of Double Aortic Arch Research Articles

Ultrasound diagnosis of double aortic arch with atresia: a clinical case

The diagnosis of a double aortic arch (DAA), when both arches are patent, is easily established using modern imaging methods, including echocardiography. In the absence of blood flow in one of the segments of the left aortic arch (LAA), it becomes impossible to obtain an image of it throughout its entire length. This makes the result of the diagnosis of this condition uncertain, since DAA with atresia will visually resemble one of the variants of the right aortic arch. DAA is an absolute indication for surgical treatment, which determines the importance of differential diagnosis of these conditions. We describe a case of echocardiographic diagnosis of DDA with atresia distal to the left subclavian artery in a newborn, the first day of life, who was admitted to the intensive care unit for newborns and premature infants due to prenatally detected anomaly of the aortic arch. The purpose of the publication is to demonstrate the key features of visual diagnostics necessary in making an accurate diagnosis.

Double Aortic Arch as a Rare Condition in a 10-year-old Boy with Recurrent Pneumonia: A Case Report Study

Introduction: A double aortic arch (DAA) is a congenital anomaly of the aortic arch, which is also the most common type of complete vascular ring. This vascular ring can compress the trachea and esophagus, as well as cause respiratory, gastrointestinal, and cardiac symptoms. Case Presentation: In the present case report, a 10-year-old boy with recurrent cough, fever, shortness of breath, chest pain, and a history of recurrent respiratory infections was examined. According to the results of bronchoscopic evaluation, he was found to have a localized tracheomalacia. DAA and dextrocardia condition were also confirmed for this patient by performing a computed tomography angiography of the thorax and the three-dimensional reconstruction of CT angiography. Conclusions: The diagnosis of double aortic arch may be confused with upper respiratory tract infections or foreign body aspiration. Therefore, it was recommended that a particular attention should be paid to the double aortic arch when evaluating cases of stridor in children with unknown causes or recurrent respiratory infections. CT angiography and cardiac MRI were the diagnostic methods of choice for examining vascular rings.

Diagnosis of isolated right aortic arch: Accuracy of prenatal diagnosis, associated genomic disorders and interest of systematic postnatal imaging. The ARCADE multicentre retrospective observational study

Prenatal diagnosis of isolated right aortic arch (RAA) have increased during the past decades. However, diagnosis accuracy, genetic testing, and post-natal follow-up are still not consensual. We aimed to analyse French pediatric cardiologists practice regarding prenatal diagnosis of isolated RAA. We collected all livebirth with isolated RAA prenatally diagnosed in 14 French congenital heart disease centres from 2015 to 2020. We compare, using kappa coefficient of concordance, the anatomical position of aortic arch, ductus arteriosus and aberrant left subclavian artery described by prenatal echo, postnatal echo, and CT scan when available. We also assessed available genetic results and functional postnatal status. We included 309 neonates with prenatal diagnosis of isolated RAA. Regarding patients with an isolated RAA, left ductus arteriosus have been described in 76% of cases. Diagnosis of aberrant left subclavian artery was missing in 73% of cases. Four patients with prenatal isolated RAA had a postnatal diagnosis of double aortic arch (DAA). Correlation between pre- and post-natal echo was excellent regarding aortic arch position (kappa = 0.97). 108 neonates (35%) underwent postnatal CT scan due to onset of respiratory symptoms (53%) or systematic centre protocol (47%). CT-scan revealed five more unknown DAA. Correlation between prenatal echo and CT-scan was good (kappa = 0.79). Furthermore, systematic CT-scan showed 13 major tracheal compression resulting in six arterial ring surgeries during the study period. Lastly, 165 patients (53%) underwent a genetic testing. Nine patients (5.4%) presented a 22q11 deletion. Performance of prenatal diagnosis of isolated RAA is excellent, with a good correlation between pre- and post-natal echocardiography or gold standard-CT scan. Prenatal diagnosis of aberrant left subclavian artery and DAA is still challenging. CT scan improve anatomical description and functional impact of aortic arch anomalies and may be proposed to complete postnatal follow-up. Lastly, amniocentesis should be considered to detect genetic disorders.

EP17.15: A case report and literature review of prenatal ultrasound diagnosis of double aortic arch versus right aortic arch variant in vascular ring formation

EP17.15: A case report and literature review of prenatal ultrasound diagnosis of double aortic arch versus right aortic arch variant in vascular ring formation

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

The Differential Diagnosis of Double Aortic Arch and Right Aortic Arch with Mirror-Image Branches in the Fetus: A Potential Novel Method.

The objective of this study was to explore a new method for the differential diagnosis between fetal double aortic arch (DAA) and right aortic arch with mirror-image branches (RAA-MB). Clinical data and prenatal echocardiographic features of the DAA (n = 22) and RAA-MB (n = 65) confirmed by postnatal or autopsy findings were analyzed retrospectively. The angles between the two aortic arches in the DAA group and between the right aortic arch and the mirror branch were measured. The differences between the two groups and differential diagnosis value of the angles were compared and analyzed based on the receiver operating characteristic curve. The proportion of left-sided ductal arteriosus (100%) was higher in the DAA group than that (32.3%) in the RAA-MB group, (P < 0.05). The proportion of conotruncal anomalies is higher in the RAA-MB group (64.6%) than in the DAA group (18.2%) (P < 0.05). There was a significant difference in the angles between the groups (DAA: 50.3° ± 8.3° vs. RAA-MB: 82.9° ± 13.8°) (P < 0.01). When the cutoff value was 62.8°, the sensitivity and specificity of the differential diagnosis were 95.5% and 96.9%, respectively. Distinguishing the angle measurement between DAA and RAA-MB is helpful in prenatal prognosis. We recommend a cutoff value of 62.8°.

Prenatal Diagnosis of Double Aortic Arch: Associated Findings and Postnatal Clinical Outcomes.

The aim of this study is to share our experience in the prenatal diagnosis and characteristics of double aortic arc and neonatal consequences. We retrospectively analyzed 2153 fetal echocardiography reports between 2014 and 2019 years. Records of 14 fetuses with double aortic arc were examined. Prenatal and postnatal medical records, sonographic images, genetic reports, associated cardiac and extracardiac anomalies, and neonatal clinical results of affected fetuses were reviewed retrospectively. DAA was isolated in 9 of 14 (64.2%) cases, while the other five cases had cardiac or extracardiac accompanying ultrasound findings. Three of cases (21.4%) were associated with other heart pathologies, including ventricular septal defect, double outlet right ventricle, and persistent left superior vena cava. In 10 cases (71.43%), the right aortic arch diameter was dominant. The left aortic arc was dominant in two cases and both arcs were symmetrical in the remaining two cases. 22q11 microdeletion was the only chromosomal abnormality and was detected in two of nine patients who accepted genetic analysis. Intrauterine fetal death occurred in two of the cases. After birth, in 58.3% (7/12) of the live born cases various degrees of symptoms. Surgical repair was performed with the division of the aorta to all symptomatic cases at different times according to severity of the complaints. Since it can cause severe respiratory distress in the postnatal period and may accompany chromosomal anomalies, it is important to provide antenatal diagnosis of double aortic arc and adequate counseling to the family.

Fetal double aortic arch: prenatal sonographic and postnatal computed tomography angiography features, associated abnormalities and clinical outcomes

BackgroundFetal double aortic arch (DAA) malformation is a rare congenital heart disease with few reported cases in the literature. We aimed to investigate the characteristics of prenatal ultrasound and postnatal computed tomography angiography (CTA) of DAA and to describe the associated anomalies and clinical outcomes to improve prenatal diagnosis and assist in perinatal management.MethodsThe obstetric ultrasound imaging databases of seven tertiary referral centers were reviewed retrospectively to identify fetuses with a prenatal diagnosis of DAA between January 2013 and December 2018. Ultrasonographic findings, associated anomalies, genetic abnormalities, postnatal CTA images, and long-term postnatal outcomes were evaluated.ResultsA total of 36 cases out of 40 prenatally diagnosed DAA fetuses were confirmed by postnatal diagnosis (fetal autopsy, CTA, and surgery). In this cohort of 36 confirmed cases, 24 (67%) were isolated anomalies, while 12 (33%) were associated with intracardiac or extracardiac anomalies, and 2 (6%) had a 22q11.2 chromosome deletion. Among nine cases of pregnancy termination with a fetal autopsy, 7 had other abnormalities. Among the remaining 27 live births, 16 (59%) were asymptomatic and 11 (41%) received surgical treatment due to tracheal or esophageal compression symptoms, all with satisfactory outcomes. Prenatal echocardiography showed that DAA was mainly characterized by a bifurcation of the ascending aorta into the right and left aortic arch and the formation of a complete O-shaped vascular ring around the trachea on the three-vessel tracheal view. A variant in the aortic arch branching pattern was found for the first time. The airway obstruction, branching pattern, and atretic arch of DAA were clearly shown by postnatal CTA.ConclusionsFetal DAA has unique features on prenatal echocardiography and postnatal CTA, and systematic prenatal examination and timely postnatal CTA evaluation are required. A certain proportion of intracardiac and extracardiac abnormalities are associated with DAA, but the probability of chromosome abnormalities is low, especially for isolated DAA.The clinical outcomes of isolated DAA are favorable, even if surgery is performed due to symptoms. Determining whether other malformations or chromosomal anomalies exist is crucial for prognosis evaluation and prenatal counseling.

Early Prenatal Diagnosis of Double Aortic Arch: Prevalence, Associated Anomalies and Outcome.

Our aims were to describe the feasibility of diagnosis of DAA in early pregnancy and to assess its prenatal prevalence, associated anomalies and outcome. A retrospective cohort review of all DAA cases diagnosed by early prenatal transvaginal scans at 12-17 weeks of gestation between the years 2007-2018 was performed. Associated anomalies, genetic abnormalities and long-term postnatal outcome were evaluated. 12 cases of DAA were diagnosed by early prenatal transvaginal scans at a median of 15 (range: 12-17) weeks of gestation out of a total of 28 654 early scans preformed with a prevalence of at least 1:2378. Associated anomalies/genetic abnormalities were found in 5/12 (42 %) cases. The diagnosis was confirmed postnatally in all newborns. In two cases termination of pregnancy was performed. Four patients (40 %) were symptomatic. Six patients (60 %) underwent surgery due to symptoms or due to severe obstruction on imaging with resolution of symptoms in all except one patient. DAA can be readily diagnosed transvaginally even in the first trimester. Its prevalence is 1:2387. A search for associated anomalies and genetic abnormalities should be performed. If DAA is isolated, the prognosis with or without surgery is usually good.

Prenatal echocardiographic diagnosis of double aortic arch

A double aortic arch is a congenital anomaly associated with the formation of a vascular ring, which can cause varying degrees of airway compression. This can be subclinical or clinical, manifesting as acute stridor, severe respiratory compromise or symptoms of chronic airway compression. An accurate diagnosis of double aortic arch can be made prenatally by fetal echocardiography. In this report, we describe two infants with normal karyotypes in whom a prenatal diagnosis of double aortic arch was made at our fetal cardiology center, between July 2012 and August 2013. The diagnosis was confirmed postnatally by two-dimensional echocardiography and cardiac magnetic resonance imaging (MRI). The presence of a right aortic arch should prompt more careful evaluation to exclude a double aortic arch. Postnatal assessment should comprise echocardiography and cross-sectional imaging in the form of cardiac MRI/computed tomography. Bronchoscopy may be considered to exclude subclinical airway compression and reduce potential long-term respiratory sequelae.

Double Aortic Arch in Adolescence: A Case Report

Introduction: Double aortic arch (DAA) is the most frequently encountered vascular ring malformation characterized by a complete encirclement of the trachea and esophagus by the aortic arch with a wide clinical spectrum. Objective: The aim of this study is to describe a case of DAA in adolesecents. Case Presentation: An 11-year-old boy was admitted for chronic productive cough which initially was diagnosed with pulmonary arterial hypertension. The final diagnosis was DAA which was established after chest computed tomography (CT) scan. Conclusion: Establishing a diagnosis of DAA in adolescents requires thorough understanding and clinical skills in performing diagnostic steps. The management of heart failure symptoms and other comorbidities is essential while preparing for its definite therapy.

A Case of Prenatal Presentation with Double Aortic Arch

Double aortic arch (DAA) is a form of vascular ring found in approximately 1 in 2000–4000 pregnancies on fetal echocardiography. In some cases of DAA, respiratory compromise occurs during infancy due to compression of the trachea, which is completely encircled by the right aortic arch (RAA) and left aortic arch (LAA). RAA and left arterial duct were initially seen on screening fetal echocardiography in a 36-year-old Japanese woman at gestational week (GW) 23. The fetus was later found to have DAA by detailed echocardiography at GW 26. The female neonate born at GW 38 weighing 2894 g with 1- and 5-minute Apgar scores of 8 and 9, respectively, developed tachypnea, stridor, and wheezing soon after birth, and underwent successful surgical correction 10 hours after birth. Thus, prenatal diagnosis of DAA aided in early recovery in this case.

Clinical value of multi-slice spiral computed tomography angiography and three-dimensional reconstruction in the diagnosis of double aortic arch.

The aim of this study was to evaluate the clincal value of multi-slice spiral computed tomography (MSCT) in the diagnosis of double aortic arch (DAA) and analyze the associated angiography and 3D reconstruction imaging to increase the accuracy of DAA diagnosis. Clinical and imaging data from 15 patients diagnosed with DAA by MSCT were summarized and compared with the corresponding surgical observations. The DAA diagnosis by MSCT for the 15 patients was confirmed by the surgical observations. A total of 13 cases were categorized as type I (double arches are open), including nine with a larger right arch, two with a larger left arch and two with balanced arches. Two cases were categorized as type II (one atretic arch), characterized by left atretic arch. Among the 15 patients, ultrasound diagnosis succeeded in nine cases and failed in the rest. A single malformation was identified in only two cases, whereas the rest had single or multiple combination(s) of intracardiac and extracardiac malformations, including seven with different levels of tracheobronchial stenosis. MSCT was demonstrated to precisely diagnose DAA complicated by malformation and tracheobronchial stenosis. MSCT is an essential therapeutic strategy and serves as a primary method in DAA diagnosis.

Late diagnosis of double aortic arch: Consequences on long‐term follow‐up

Double aortic arch is the most common congenital anomaly of the aortic arch system, in which the trachea and esophagus are completely encircled by vascular segments of the aortic arch and its branches, often resulting in variable airway compression. One case of late diagnosis of this congenital malformation and long-term consequences of late surgical treatment with persistent tracheo-broncomalacia and dynamic airway obstruction is reported. This report emphasizes the importance of an early diagnosis to minimise the progressive airways damage and subsequent respiratory symptoms, that need an accurate medical follow-up.

Double Aortic Arch with Mirror-Image Dextrocardia

Double aortic arch (DAA) is a very rare congenital vascular anomaly, characterized by the encircling trachea and esophagus, resulting feeding and/or respiratory problems. In this article, a two-month-old girl with situs inversus totalis and DAA was presented. The baby was symptomatic with noisy breathing and coughing since birth. Double aortic arch was detected by echocardiography, barium esophagography, cardiac MRI and conventional angiography. The present report emphasis that vascular ring should have been evaluated in a patient with respiratory and/or feeding problems. Chest radiography, barium esophagography, echocardiography, multislice computed tomography, magnetic resonance imaging angiography and conventional cardiovascular angiography can be used for the diagnosis of DAA.

Prenatal diagnosis of double aortic arch and D‐transposition of the great arteries: a case report

Prenatal DiagnosisVolume 30, Issue 4 p. 382-383 Research Letter Prenatal diagnosis of double aortic arch and D-transposition of the great arteries: a case report W. M. Yao, W. M. Yao Department of Ultrasonography, Women's Hospital, School of Medicine, Zhejiang University, Hangzhou, ChinaSearch for more papers by this authorJ. M. Wang, J. M. Wang Department of Ultrasonography, Women's Hospital, School of Medicine, Zhejiang University, Hangzhou, ChinaSearch for more papers by this authorH. F. Huang, H. F. Huang Department of Reproductive Endocrinology, Women's Hospital, School of Medicine, Zhejiang University, Hangzhou, ChinaSearch for more papers by this authorY. H. Ye, Corresponding Author Y. H. Ye yeyh1999@hotmail.com Department of Reproductive Endocrinology, Women's Hospital, School of Medicine, Zhejiang University, Hangzhou, ChinaDepartment of Reproductive Endocrinology, Women's Hospital, School of Medicine, Zhejiang University, Hangzhou, Zhejiang 31006, China.Search for more papers by this author W. M. Yao, W. M. Yao Department of Ultrasonography, Women's Hospital, School of Medicine, Zhejiang University, Hangzhou, ChinaSearch for more papers by this authorJ. M. Wang, J. M. Wang Department of Ultrasonography, Women's Hospital, School of Medicine, Zhejiang University, Hangzhou, ChinaSearch for more papers by this authorH. F. Huang, H. F. Huang Department of Reproductive Endocrinology, Women's Hospital, School of Medicine, Zhejiang University, Hangzhou, ChinaSearch for more papers by this authorY. H. Ye, Corresponding Author Y. H. Ye yeyh1999@hotmail.com Department of Reproductive Endocrinology, Women's Hospital, School of Medicine, Zhejiang University, Hangzhou, ChinaDepartment of Reproductive Endocrinology, Women's Hospital, School of Medicine, Zhejiang University, Hangzhou, Zhejiang 31006, China.Search for more papers by this author First published: 12 February 2010 https://doi.org/10.1002/pd.2472Citations: 3AboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat No abstract is available for this article.Citing Literature Volume30, Issue4April 2010Pages 382-383 RelatedInformation

OP06.07: Prenatal diagnosis of double aortic arch (DAA): report of 6 cases

OP06.07: Prenatal diagnosis of double aortic arch (DAA): report of 6 cases

Diagnosis of the Double Aortic Arch and Its Differentiation from the Conotruncal Malformations

PurposeThe clinical and radiological characteristics of the double aortic arch (DAA) and its differentiation from conotruncal malformations (CTM) were reported in order to familiarize pediatric practitioners with these congenital heart diseases.Materials and MethodsFrom July 1994 to December 2006, a total of 6 patients (4 male and 2 female, aged 16 days to 6.5 years) with DAA were enrolled in this retrospective study. The study modalities included chart recordings, plain chest radiographs, barium esophagograms, echocardiograms, cardiac catheterization, cardiac angiograms, surgery, magnetic resonance imaging, and chromosome analysis. Patients with incomplete vascular rings or with right aortic arches and left ligamentum were excluded. In addition, the clinical and radiological profiles of 38 patients with CTM, including dextro-transposition of the great arteries (d-TGA) (n = 28), hemitruncus arteriosus (HTA) (n = 3), type I truncus arteriosus (TA) (n = 4), and the aortopulmonary window (APW) (n = 3), were comparatively reviewed.ResultsAll 6 patients with DAA presented with postprandial choking and respiratory distress that prompted their initial visit to the hospital. One of the 6 patients presented with congestive heart failure, and none with cyanosis. Esophagograms showed indentations in 5 patients with DAA. All patients with d-TGA presented with cyanosis and heart failure, while patients with HTA, type I TA, and APW manifested overt heart failure. Suprasternal and subcostal approaches of the echocardiography may offer diagnositic windows for DAA. As for CTM, parasternal and subcostal approaches could always determine the causality. Cardiac catheterization with angiography comprehensively delineated the pathology.ConclusionIn case of postprandial choking and respiratory distress in neonates and infants, barium esophagograms can indicate the presence of DAA. Diagnosis of DAA and its differentiation from the CTM can be achieved by echocardiography, angiography, or magnetic resonance imaging.

Angiography and Dynamic Airway Evaluation with MDCT in the Diagnosis of Double Aortic Arch Associated with Tracheomalacia

3Department of Surgery, The Chinese University of Hong Kong, Prince of Wales Hospital, Shatin, New Territories, Hong Kong. ouble aortic arch is a well-known congenital vascular anomaly causing extrinsic tracheal compression in neonates that can present with life-threatening episodes of stridor, cyanosis, or apnea. Surgical correction of the underlying vascular ring is the key for successful treatment for such patients [1]. However, previous studies have shown that as many as 30% of pediatric patients might still have persistent airway obstruction after surgical procedures [2, 3]. These symptoms may be related to airway wall weakening as a result of long-standing extrinsic airway compression. Such patients may benefit from undergoing a second operation after evaluation with both bronchoscopy and cross-sectional imaging. Recently, MDCT with multiplanar and 3D reconstruction has become an important tool in the evaluation of thoracic aortic anomalies in pediatric patients, obviating conventional angiography [4, 5]. Dynamic inspiratory–expiratory imaging by MDCT has been shown to be a promising method in the evaluation of patients with dynamic airway obstruction and to correlate well with bronchoscopic results [6]. In this case report, we describe the preand postoperative MDCT angiography findings in an infant with double aortic arch who failed extubation after undergoing corrective surgery. A dynamic airway evaluation was performed during the same setting of MDCT angiography by alternately applying and withholding positive-pressure ventilation, which simulated the inspiratory–expiratory scan. The child was found to have tracheomalacia, which explained the airway obstruction that persisted postoperatively.

Double aortic arch

A newborn was referred to our institution because of severe respiratory distress and cyanosis requiring resuscitation and intubation. A chest x-ray film did not indicate lung parenchymal disease. Tracheoscopy revealed a pulsatile compression of the trachea. Two-dimensional echocardiography, barium esophagram (Fig 1), magnetic resonance imaging (Fig 2), and angiography confirmed the suspected diagnosis of double aortic arch with severe compression of the esophagus and trachea. Fig. 2Magnetic resonance image showing left (LAA) and right (RAA) aortic arches and compressed trachea and esophagus. View Large Image Figure Viewer Download Hi-res image The right aortic arch was sectioned, resulting in immediate relief of the compressed structures and clinical symptoms.