Diagnosis Of Right Aortic Arch Research Articles

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

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. 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). 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. 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.

Short-term outcome after the prenatal diagnosis of right aortic arch.

To determine the proportion of children that require surgery in the first year of life and thereafter in order to improve the counseling of parents with a fetus with a right aortic arch (RAA). Fetuses diagnosed with isolated RAA, defined as the absence of intra- or extracardiac anomalies, between 2007 and 2021 were extracted from the prospective registry PRECOR. In total, 110 fetuses were included, 92 with a prenatal diagnosis of RAA and 18 with double aortic arch (DAA). The prevalence of 22q11 deletion syndrome was 5.5%. Six pregnancies were terminated and five cases were false-positive; therefore, the follow-up consisted of 99 neonates. Surgery was performed in 10 infants (10%) in the first year of life. In total, 25 (25%) children had surgery at a mean age of 17months. Eight of these 25 (32%) had a DAA. Only one child, with a DAA, required surgery in the first week of life due to obstructive stridor. Children with a prenatally diagnosed RAA are at a low risk of acute respiratory postnatal problems. Delivery in a hospital with neonatal intensive care and pediatric cardiothoracic facilities seems only indicated in cases with suspected DAA. Expectant parents should be informed that presently 25% of the children need elective surgery and only incidentally due to acute respiratory distress.

Resection of Kommerell\u2019s diverticulum in an infant with prenatal diagnosis of right aortic arch

BackgroundA right aortic arch is a congenital vascular anomaly that is present in up to 0.1% of pregnancies. The anomaly observed by fetal ultrasonography was recently reported to indicate vascular and chromosomal abnormalities that may complicate postnatal management.Case presentationWe report the successful resection of a Kommerell’s diverticulum with left subclavian artery transfer to the left carotid artery in a 5-month-old Japanese boy. The patient was prenatally diagnosed as having a right aortic arch, and a vascular ring was confirmed at 4 months of age with enhanced computed tomography. The pathology of the resected aortic wall revealed severe disruption and fragmentation of elastic fibers associated with a disarray of smooth muscle cells in the tunica media, and cystic medial necrosis with mucoid extracellular matrix deposition.ConclusionThese abnormal pathological findings supported the resection of Kommerell’s diverticulum at this point of time, and division of the ligamentum arteriosus alone was not recommended. Early intervention in this condition once the diagnosis is made may thus be advocated. The fetal diagnosis of a right aortic arch may provide a clue to the possibility of a vascular ring.

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.

Response: “Another merit of fetal MRI in prenatal diagnosis of right aortic arch”

Response: “Another merit of fetal MRI in prenatal diagnosis of right aortic arch”

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

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.

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

The significance of a prenatal diagnosis of right aortic arch.

To analyze a population of fetuses with prenatally diagnosed right aortic arch (RAA). Retrospective study of fetuses with RAA diagnosed prenatally between 2011 and 2015 in two referral centers. Right aortic arch was found in 4.4% (46/1036) of fetuses with cardiovascular abnormalities (CVA). As an isolated anomaly, RAA was present in 30.4% of cases; in 32.6%, other CVA were detected; in 23.9%, CVA and extracardiac anomalies; and in 13.1%, only extracardiac malformations. The most common noncardiac abnormalities were thymus hypoplasia/aplasia (7/17), of which six had deletion 22q.11.2. In another three fetuses, trisomy 21 was present. One intrauterine fetal death occurred at 41 weeks of pregnancy, and two fetuses died after birth. In six of 18 infants with known follow-up, symptoms of dysphagia were reported, of which four infants underwent surgical intervention. In 12 infants, an isolated RAA was clinically silent. The diagnosis of RAA is an indication for a detailed examination of cardiac and noncardiac structures, including the thymus. It is advisable to consider genetic testing, together with the assessment of deletion 22q11.2, especially in the case of accompanying defects. The prognosis depends on underlying cardiac and extracardiac anomalies and possibly coexisting genetic defects. Isolated anomalies are asymptomatic. © 2017 John Wiley & Sons, Ltd.

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

Risk of 22q11.2 deletion in fetuses with right aortic arch and without intracardiac anomalies.

To assess the risk of 22q11.2 deletion in fetuses with a prenatal diagnosis of right aortic arch without intracardiac anomalies (RAA-noICA). This was a retrospective study of all fetuses with RAA-noICA diagnosed prenatally at three referral centers, between 2004 and 2014. A detailed sonographic examination was performed in each case, including visualization of the thymus and of the head and neck vessels to identify the presence of an aberrant left subclavian artery (ALSA). Karyotyping and fluorescence in situ hybridization analysis for diagnosis of 22q11.2 deletion were always offered either prenatally or postnatally. Clinical and echocardiographic examinations were performed in livebirths and a postmortem examination in cases of termination of pregnancy. During the study period, 85 fetuses were diagnosed prenatally with RAA-noICA. Genetic or clinical data were not available for three cases and these were excluded from analysis. 22q11.2 deletion was found in 7/82 cases (8.5% (95% CI, 3.8-17.3%)). The thymus was small or non-visualized in all seven cases and additional abnormal sonographic findings were present in four. 22q11.2 deletion is present in a clinically significant proportion of fetuses with a prenatal diagnosis of RAA-noICA. In such cases, a detailed sonographic examination, with assessment of the thymus in particular, may be useful to further define the level of risk for 22q11.2 deletion. Copyright © 2015 ISUOG. Published by John Wiley & Sons Ltd.

Diagnóstico prenatal del arco aórtico derecho, importancia de los planos de Yagel. Nuestra experiencia, descripción de 2 casos

Right aortic arch, which belongs to a group of rare malformations, is an uncommon finding (incidence of 0.1%) and few cases of prenatal forms have been described in the literature. Nevertheless, it is not unusual to find these anomalies as the cause of respiratory or digestive disease (refractory to treatment) in adult patients and even as severe vascular processes with high morbidity and mortality. Its variants may cause compressive symptoms and be associated with heart defects (up to 90%) and/or 22q11 microdeletion (up to 46%). The 3 vessels and trachea view is essential for the prenatal diagnosis of right aortic arch. Detection of this condition must be considered an indication for foetal echocardiography, to distinguish its variants, and/or specific chromosome/genetic testing. Isolated right aortic arch is usually asymptomatic, although the outcome of right aortic arch associated with other abnormalities is poor.

P45 Is it important to identify an isolated right aortic arch in fetal life?

Background Isolated right aortic arch (RAA) is felt to be a rare finding, present in around 0.1%. The 3 vessel and tracheal view has been part of the fetal medicine departmental screening since 1998 and has now been incorporated into the national screening guidelines. The objective is to define the prenatal associations and postnatal outcomes of fetuses diagnosed with a RAA in utero. Methods Retrospective analysis of fetuses diagnosed with an isolated RAA diagnosed at the Harris Birthright Centre for Fetal Medicine between 2000 and 2014. Prenatal and postnatal findings were ascertained from clinical records. Diagnosis of RAA was made in the transverse three-vessel/tracheal view where the aortic arch was demonstrated passing in front of the trachea.(Figure 1) Prenatal data were combined with outcomes obtained from medical records and telephone interviews with patients. Results 100 cases of isolated right aortic arch were identified in fetal life at a median gestation of 21weeks (range:11–36). The nuchal translucency (NT) was elevated in 10/64 (16%) where the measurement was known and an extracardiac abnormality detected in 23/100 cases of whom 2 had a raised NT. Genetic abnormalities were confirmed in 13/58 (21%) tested cases comprising: 22q11.2 microdeletion (n = 5), trisomy 21 (n = 2), combined 22q11 microdeletion with mosaicism of 45XO (n = 1), unbalanced translocation (n = 1), balanced translocation (n = 1), duplication of chromosome 16p13 with a recessive disorder of lymphogenesis (n = 1) and other syndromes (n = 3). Of those with abnormal genetics 4/13 had no other abnormal fetal sonographic markers. An aberrant left subclavian artery (ALSCA) was identified in 39/98 (40%), the arterial duct was left sided in 67/75 (89%). In the postnatal period, 3 cases were diagnosed with a double aortic arch. Spontaneous intrauterine death occurred in 6 cases and termination of pregnancy in 9 cases. There were 80 live births and one neonatal death. Five children presented with symptoms of airway obstruction at a median age of 2.9 years (range 1–8). Pulsatile airway compression was identified in all and surgical division of the vascular ring was performed at median age: 2.9years (range: 1–8). Within the past two years, three asymptomatic infants (with RAA, ALSCA, left arterial duct) have been shown by bronchoscopy and cross-sectional imaging to have significant airways compression. Thus, we are currently considering whether all infants with RAA, ALSCA and left duct merit investigation irrespective of symptoms. Conclusions Significant genetic abnormalities were identified of which some had no other abnormal fetal sonographic findings. Many patients were discharged after their first postnatal review, but despite this 8 children have required surgical relief of a vascular ring as a result of pulsatile compression of the trachea. Lack of symptoms does not exclude significant airways compression and the true incidence of airway compromise may be underestimated.

Ultrasound diagnosis of fetal right aortic arch in high-risk and low-risk pregnant women

Objective To investigate the clinical value of ultrasound diagnosis of fetal right aortic arch (RAA).Methods From March 5,2009 to September 2,2012,18 600 pregnant women in three hospitals in Foshan City,Guangdong Province,were divided as RAA high-risk group (n=5000) and low-risk group (n =13 600).During 18 to 42 gestational weeks,detailed ultrasound examinations of fetal aortic arch were done.All babies received echocardiography,CT or MRI within 15 months after birth to observe the cardiovascular condition,and aborted fetus was autopsied.RAA type,complicated structural abnormality and pregnancy outcomes between the two groups were compared with Chi-square test.Results Fifteen fetal RAA were diagnosed prenatally in the low-risk group,and the detection rate was 0.11％ (15/13 600),which was lower than that in the high-risk group (0.60％,30/5000)(x2=98.20,P＜0.01).The correct rate of prenatal ultrasound diagnosis of RAA was 96％ (43/45),while the other two cases were diagnosed as RAA with aberrant left subclavian artery and confirmed to be double aortic arch and RAA with mirror image after birth.In the high-risk group,the incidence of RAA with mirror image,cardiac and extra-cardiac malformation was 73％(22/30),67％(20/30) and 27％(8/30),respectively,which were higher than those in the low-risk group(0/15,1/15 and 0/15)(x2 =30.000,25.200 and 28.000,P＜0.01 respectively).The incidence of RAA with aberrant left subclavian artery and the 15-month survival rate of RAA infants in the high risk group was lower than those in the low-risk group [20％ (6/30) vs 12/15,40％ (12/30) vs 15/15] (x2=7.700 and 15.610,P＜0.05,respectively).Conclusions The incidence of fetal RAA is higher in the high-risk pregnant women than in the low-risk pregnant women,and babies often complicated with other malformations with poor prognosis. Key words: Ultrasonography, prenatal; Aorta, thoracic; Congenital abnormalities; Vascular malformations ; Pregnancy

Prenatal diagnosis of right aortic arch with mirror‐image branching without associated anomalies

A pregnant woman at 32 weeks of gestation was referred because of untypical visualization of a three-vessel tracheal view. Detailed ultrasound examination demonstrated an abnormal position of descending aorta, positioned at the right-front side of the spine at the level of four-chamber view. The descending aorta was connected with left pulmonary artery through ductus arteriosus, which configured a vascular ring. Right aortic arch with mirror-image branching without any associated anomaly was suspected prenatally. Amniocentesis showed no chromosomal abnormality. A male, 2440-g infant was delivered vaginally at 37 weeks and 4 days of gestation. Echocardiography confirmed no associated congenital heart defect. Three-dimensional reconstruction using 64-slice spiral computed tomography angiography at 2-months old confirmed the diagnosis of right aortic arch with mirror-image branching. The infant is now 1 year old and well developed without any symptoms.

P26.10: Prenatal diagnosis of right aortic arch (RAA): clinical experience in two Spanish hospitals

P26.10: Prenatal diagnosis of right aortic arch (RAA): clinical experience in two Spanish hospitals

Prenatal diagnosis of right aortic arch by fetal echocardiography

Prenatal diagnosis of right aortic arch by fetal echocardiography

Prenatal diagnosis of right aortic arch by fetal echocardiography

*Corresponding author. E-mail address: fatimasilva.15@gmail.com (F. Silva). Right aortic arch is a cardiovascular malformation or a variant of normality which can be diagnosed by prenatal ultrasound. This malformation may be associated with DiGeorge syndrome (arising from defects in chromosome 22) and can result in posterior compression of the trachea and esophagus, leading to dysphagia and respiratory impairment after birth. Figure A Right aortic arch passing posterior to the trachea. AO: aorta; PA: pulmonary artery; RV: right ventricle; SVC: superior vena cava; TR: trachea.

Right Aortic Arch With Aberrant Left Subclavian Artery and Anomalous Origin of Right Pulmonary Artery From Ascending Aorta

The authors report a case of a neonate that was operated on with the diagnosis of right aortic arch and aberrant left subclavian artery and anomalous origin of right pulmonary artery from ascending aorta. Computed tomography (CT) scan suggested double aortic arch and cardiac catheterization suggested anomalous origin of right pulmonary artery from ascending aorta versus aorto-pulmonary window. The final diagnosis was made at the operation. There was a right aortic arch and aberrant left subclavian artery and persistent ductus arteriosus. Surgical repair consisted of section of the ductus arteriosus and reimplantation of the right pulmonary artery in the main pulmonary artery.