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

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

Impact on the trachea in children with prenatally diagnosed vascular ring formed by a right aortic arch.

OBJECTIVE: There has been a rise in the prenatal detection of right sided aortic arch (RAA). When associated with a left-sided arterial duct (LD), this forms a vascular ring encircling the trachea. Infants may have symptoms or signs suggestive of tracheoesophageal compression but many are asymptomatic. The objective of this study was to investigate the relationship between symptoms and severity of tracheobronchial compression assessed by bronchoscopy. METHODS: Retrospective review of all cases of prenatally diagnosed RAA-LD in the absence of associated congenital heart disease at Evelina London Children's Hospital and Kings College Hospital over a 4-year period between April 2015-2019. Clinical records, fetal echocardiograms, and free-breathing flexible bronchoscopy (FB) data were reviewed. RESULTS: One hundred and twelve cases of isolated RAA-LD were identified of whom 82 cases (73%) underwent FB. FB was performed median age of 11 months (range 1-36 months), no complications occurred. Aberrant left subclavian artery (ALSA) was present in 86% (96/112) and mirror image branching (MIB) in 13% (15/112). 34/112 (30%) reported symptoms during follow-up. 36/77 (47%) with ALSA who underwent FB showed moderate-severe compression mostly at distal tracheal and carinal level of whom 38% had parent-reported symptoms. Moderate-severe compression was seen in 3/5 (60%) with MIB mostly at mid tracheal level; 3 were symptomatic but only 2 had tracheal compression. In total 36% (18/50) of investigated asymptomatic patients showed moderate-severe compression. Respiratory symptoms were poorly predictive of moderate-severe tracheal compression (positive predictive value 66%, negative predictive value 64%). CONCLUSION: The absence of symptoms did not exclude significant tracheal compression. The anatomical effect of the vascular ring is under appreciated when symptoms alone are used as a marker of tracheal compression.

Double aortic arch: implications of antenatal diagnosis, differential growth of arches during pregnancy, associated abnormalities and postnatal outcome.

OBJECTIVES: To evaluate the prenatal characteristics of double aortic arch (DAA), assess the relative size of the arches and their growth during pregnancy, describe associated cardiac, extracardiac and chromosomal/genetic abnormalities and review postnatal presentation and clinical outcome. METHODS: This was a retrospective cohort study of all fetuses with a confirmed diagnosis of DAA seen in five specialized referral centers in London, UK, between October 2012 and November 2019. Cases were identified from the hospitals' fetal databases. Fetal echocardiographic findings, intracardiac and extracardiac abnormalities, genetic defects, computed tomography (CT) findings and postnatal clinical presentation and outcome were evaluated. RESULTS: A total of 79 fetuses with DAA were included. Of those assessed postnatally, 48.6% had an atretic left aortic arch (LAA), while 5.1% had an atretic LAA at the first fetal scan and were misdiagnosed antenatally with right aortic arch (RAA). The LAA was atretic in 55.8% of those who underwent CT. DAA was an isolated abnormality in 91.1% of cases; 8.9% of patients had an additional intracardiac abnormality and 2.5% had both intra- and extracardiac abnormalities. Among the 52 cases that underwent genetic testing, 11.5% had genetic abnormalities and, specifically, the 22q11 microdeletion was identified in 3.8% of patients. At a median follow-up of 993.5 days, 42.5% of patients had developed symptoms of tracheoesophageal compression (5.5% during the first month after birth) and 56.2% had undergone intervention. Statistical analysis using the χ-square test showed no significant relationship between morphology of DAA (patency of both aortic arches vs atretic LAA) and the need for intervention (P = 0.134), development of vascular ring symptoms (P = 0.350) or evidence of airway compression on CT (P = 0.193). CONCLUSIONS: Most cases of DAA can be diagnosed easily at midgestation, as typically both arches are patent with a dominant RAA at this stage. However, we found that the LAA had become atretic in approximately half of the cases postnatally, supporting the theory of differential growth of the arches during pregnancy. DAA is usually an isolated abnormality; however, thorough assessment is required to exclude associated intra- and extracardiac anomalies and to determine the need for invasive prenatal genetic testing. Postnatally, early clinical assessment is needed and CT scan should be considered, irrespective of the presence of symptoms. © 2023 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.

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

OBJECTIVES: 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). METHODS: 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. RESULTS: 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 17 months. 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. CONCLUSIONS: 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.

Cases of Ultrasound-Diagnosed Right Aortic Arch with Right Arterial Duct and the Treatment.

This paper aims to discuss the value of ultrasound to diagnose right aortic arch with right arterial duct. A retrospective analysis of fetal echocardiography characteristics of 10 fetuses who were diagnosed as right aortic arch with right arterial duct from December 2016 to March 2021 is made, and focus is put on the relationship between the aortic arch and arterial duct, and the position of aortic arch, arterial duct arch and trachea on the three vessels and trachea view (3VT). As a result, all 10 cases with right aortic arch and right arterial duct do not show aberrant left subclavian artery, and aortic arch with arterial duct are still connected as "V-shaped", and do not get vascular rings. In conclusion, 3VT can simply and clearly detect the right aortic arch, and the key to diagnosing the right aortic arch and right arterial duct is thorough inspection of the aortic arch, arterial duct, and trachea in their respective positions.

Emergency CT misdiagnosis in acute aortic syndrome.

OBJECTIVES: This cross-sectional study assessed the accuracy of emergency CT reports at presentation in acute aortic syndrome (AAS). METHODS: Retrospective identification of cases of AAS presenting within a large health board with three acute hospitals receiving adult patients between January 2013 and December 2016. CT studies and reports at presentation were reviewed for discrepancies related to diagnosis, complications and classification by two cardiovascular radiologists. The specialist interest of the original reporters, clinically suspected diagnosis at referral for CT and technical adequacy of the scans were also assessed. False-positive diagnoses were identified and evaluated separately. RESULTS: Among 88 consecutive confirmed cases of AAS at least one discrepancy was identified in 31% (n = 27), including failure to identify or misinterpretation of the AAS itself in 15% (n = 13), haemorrhage in 13% (n = 11), branch involvement in 9% (n = 8), and misclassification in 3% (n = 3). All discrepancies occurred among the 80% (n = 70) of cases reported by radiologists without specialist cardiovascular interest. 26% (n = 23/88) of AAS cases were not clinically suspected at referral for CT and although this was associated with suboptimal protocols, only 51% of CT scans among suspected cases were technically adequate. Seven false-positive diagnoses were identified, three of which related to motion artefact. CONCLUSION: Significant discrepancies are common in the emergency CT assessment of positive cases AAS and this study highlights important pitfalls in CT technique and interpretation. The absence of discrepancies among radiologists with specialist cardiovascular interest suggests both suspected and confirmed cases warrant urgent specialist review. ADVANCES IN KNOWLEDGE: CT angiography is central to the diagnosis of AAS; however, significant radiology discrepancies are common among non-specialists. This study highlights important pitfalls in both CT technique as well as interpretation and supports routine specialist cardiovascular imaging input in the emergency assessment of AAS.

Arterial switch operation for Taussig-Bing anomaly with aortic arch obstruction.

INTRODUCTION: The short-term survival rate after single-stage correction of Taussig-Bing anomaly with aortic arch obstruction remains favorable. However, some cases are encountered occasionally in which single-stage correction was not performed during the neonatal period. Accordingly, we evaluated the midterm outcomes of different surgical strategies. METHODS: Seven patients who underwent an arterial switch operation and intraventricular rerouting as definitive surgery between 2007 and 2017 were investigated. Of these 7 patients, 3 had undergone previous pulmonary artery banding and aortic arch reconstruction. RESULTS: The median body weight at definitive surgery was 3.3 kg (range 2.9-8.3 kg). At definitive surgery, the arrest time for single-stage correction (162.3 ± 21.7 min) was significantly shorter than that of staged repair (206.3 ± 5.1 min, p = 0.020). There was no hospital or late death. One patient in both strategy groups underwent aortic reintervention 54 months and 7.1 months after the definitive operation. Neoaortic valve (perinatal pulmonary valve) diameter decreased significantly from the perinatal valve diameter following definitive surgery (median +4.94z and +2.12z, respectively, p = 0.016) but there was no significant difference in the neopulmonary valve (perinatal aortic valve) diameter. Both single-stage correction and staged repair patients showed a similar trend. At the last follow-up, no patient had greater than mild neoaortic or neopulmonary valve regurgitation. CONCLUSION: The surgical outcomes of both single-stage correction and staged correction for Taussig-Bing anomaly with aortic arch obstruction are excellent. Both strategies produce similar changes in the diameter and regurgitation grade of the neoaortic and neopulmonary valves.

Papel de la imagen multimodal en la periarteritis relacionada con la inmunoglobulina G4 / Role of multimodal imaging in immunoglobulin G4-related periarteritis

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Critical left coronary main trunk stenosis, chronic occluded right coronary artery, left subclavian artery occlusion, severe aortic regurgitation and porcelain aorta in a patient with aortitis.

BACKGROUND: Coronary involvement is rare but can be critical in patients with aortitis. Although cardiac ischemia can be resolved by coronary artery bypass grafting (CABG), patients complicated with cardiac ischemia, calcified aorta, and valve insufficiency pose difficult problems for surgeons. CASE PRESENTATION: A 71-year-old woman was referred to our institution because of unstable angina. She had been previously diagnosed with aortitis and left subclavian artery occlusion. Contrast-enhanced computed tomography revealed severe left coronary main trunk stenosis, right coronary artery occlusion, and porcelain aorta. Ultrasonic echocardiogram showed severe aortic regurgitation. We performed emergent coronary artery bypass grafting, aortic valve replacement and ascending aorta replacement under hypothermic circulatory arrest. CONCLUSIONS: The technique of circumferential calcified intimal removal and reinforcement with felt strips was effective for secure anastomosis. Unilateral cerebral perfusion from the right subclavian artery enabled good visualization and sufficient time to perform distal anastomosis.

Right aortic arch with an aberrant left subclavian artery: sonographic features by HDlive Flow imaging.

The right aortic arch is a congenital vascular anomaly, which may form a vascular ring. However, prenatal identification of the branching pattern of brachiocephalic vessels is often limited. In this paper, we clearly demonstrated the branching pattern of brachiocephalic vessels in a case of right aortic arch with an aberrant left subclavian artery using HDlive Flow imaging.

[Isolated right aortic arch: prenatal diagnosis characteristics, pregnancy outcomes and systematic review]. / Crosse aortique droite isolée : caractéristiques du diagnostic anténatal, issues de grossesses et revue de la littérature.

OBJECTIVE: To investigate prenatal diagnosis characteristics and pregnancy outcomes associated with isolated right aortic arch (RAA). METHODS: A retrospective study including fetuses with isolated RAA, managed between January 2010 and February 2018. Cases were identified from the ultrasound databases of the expert pediatric cardiologists, who made the aforementioned diagnosis. All fetuses were examined by a fetal medicine imaging expert to exclude any extracardiac abnormality. A systematic review was performed to assess the prenatal diagnosis and outcomes of fetuses with isolated RAA. RESULTS: Fifty-six fetuses were diagnosed with an isolated RAA. An isolated double aortic arch (DAA) was diagnosed in one fetus. Mean gestational age at diagnosis was 24 weeks. The sex ratio (boy/girl) was 0.89. No significant abnormality was detected in invasive tests (karyotype and FISH or microarray). Only one fetus was misdiagnosed with isolated RAA. He was the only symptomatic (stridor) newborn baby and was later diagnosed with DAA. Four studies were included in our systematic review representing 115 cases of isolated RAA. One significant chromosomal abnormality was detected: a 22q11 deletion in a newborn baby who had a postnatal finding of a soft palate cleft. There was one major obstetric complication: an intrauterine fetal demise at 41 gestational weeks. CONCLUSION: Diagnosis of isolated RAA can be challenging. Invasive tests are to be discussed. The diagnosis of isolated RAA should not change obstetric monitoring. Nevertheless, an echocardiography should be performed systematically in these new newborn babies within their first month of life.

Fetal Right Aortic Arch: A Quantitative Method of Outcome Prediction.

OBJECTIVE: To investigate right aortic arches (RAA) quantitatively and risk-stratify fetuses with RAA prenatally. METHOD: A total of 143 singleton fetuses were enrolled. Four measurements were studied, including the angle and distance between the aortic arch (AO) and ductal arch (DA), the diameters of the AO and DA, and the distance growth rate (DGR). RESULT: A significant increase in mean distance was observed in the study group (4.89 ± 1.07 mm) compared to the control group (1.62 ± 0.33 mm, p<.01). The distance increased from the second (1.52 ± 0.35 mm, p<.01, 4.06 ± 0.78 mm, p<.01) to third trimester (1.71 ± 0.29 mm, p<.01, 5.13 ± 1.10 mm, p<.01) in the normal and study groups. Fetuses with abnormalities in addition to RAAs had significantly lower growth rates (12.4 ± 5.2%) than those with an isolated RAA (33.7 ± 12.0%). CONCLUSION: Parameters, especially DGR, may be clinically significant. DGR may be restricted when an RAA exists with other malformations. Decreasing DGR in fetuses with RAAs suggests the presence of other malformations.

Type B Interrupted Right Aortic Arch: Diagnostic and Surgical Approaches.

Interrupted right aortic arch is a rare congenital cardiovascular anomaly typically associated with other forms of congenital heart disease. We report two cases of interrupted right aortic arch associated with isolated left pulmonary artery and bilateral ductus arteriosus in the first case and with truncus arteriosus in the second case. Computed tomography was complementary to echocardiography in delineating the anatomic details in both cases, informing surgical repair with reconstruction of a left aortic arch in the first case and right arch in the second case.

Atretic anomalous left subclavian artery as part of an unusual vascular ring.

In this report, a unique case of a symptomatic vascular ring formed by right aortic arch, aberrant left subclavian artery, and left ligamentum arteriosus in which there is atresia of the proximal left subclavian artery is described. Imaging modalities were non-diagnostic and the patient was sent to surgery based on strong clinical suspicion. Her anatomy was delineated in the operating room and the ring was successfully repaired.

Prenatal echocardiographic assessment of right aortic arch.

OBJECTIVES: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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 Ultrasonic Diagnosis and Prognostic Analysis of Isolated Left Subclavian Artery (ILSCA) and Left Brachiocephalic Trunk (ILBCT) Malformation.

The purpose of the study was to explore the prognosis, as well as antenatal ultrasonic features of isolated left subclavian artery (ILSCA) and isolated left brachiocephalic trunk (ILBCT) malformations, in order to improve prognosis and provide guidance for prenatal diagnosis. The origin and routing of cephalic and cervical vessels were observed in patients diagnosed with right aortic arch or right arterial duct arch in our hospital from March 2015 to March 2017, and the spectrum features related to ILSCA and ILBCT were analyzed. Fetuses diagnosed as ILSCA, or, and ILBCT were followed up for 3 months after birth. At the same time, a literature review was carried out for ILBCT and ILSCA in Pubmed. In our study, two cases with ILSCA and ILBCT were both diagnosed prenatally. They are not accompanied by other congenital malformations or chromosome abnormalities. No abnormality was found during postnatal follow-up except that left radial pulsation was weakened and blood pressure of the left upper limb decreased in baby with ILSCA. In baby with ILBCT, in addition to these abnormal changes, the left common carotid artery pulse disappearance too. In pubmed, three of 12 ILSCA or ILBCT did not have other congenital malformation or chromosome abnormalities. They were not diagnosed until the age of 3, 10, and 47 because of school exams or atypical symptoms, such as headaches, chest pain. Symptom of ILBCT or ILSCA without other abnormality is silent, and therefore they cannot be diagnosed timely after birth prenatal diagnosis is necessary for they can be treated in time.

Vascular ring associated with d-transposition of the great arteries: when should we suspect aortic arch anomalies?

A male neonate with d-transposition of the great arteries was diagnosed with the concomitant anomaly of left circumflex aortic arch and right-sided ductus arteriosus, which formed a vascular ring. Initial postnatal echocardiography had demonstrated an obscured aortic isthmus mimicking coarctation of the aorta, which could be a diagnostic clue to circumflex aortic arch.

Ventana aortopulmonar, ductus ausente y arteria subclavia izquierda aislada / Aortopulmonary window, absent ductus, and left subclavian artery isolation

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Right aortic arch with isolated left subclavian artery: a rare association with coarctation of the left pulmonary artery.

We present a case report of rare association of right aortic arch with isolated left subclavian artery with coarctation of the left pulmonary artery.