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• W3096235457 abstract "•Fetal arch anomalies are difficult to diagnose because of the diversity of variants.•A strategy for 2D echocardiographic diagnosis of arch anomalies is important.•A strategy for 3D STIC in the evaluation of fetal arch anomalies is important. Fetal aortic arch anomalies and pulmonary slings can be difficult to accurately diagnose but have important clinical implications related to vascular rings, congenital heart disease, and chromosomal anomalies. In this article, the authors briefly review the embryology and development of the fetal arch to facilitate understanding of its diverse variants. Two-dimensional echocardiographic characteristics are summarized for each type of these malformations to propose a strategy for fetal diagnosis. The added benefits of three-dimensional echocardiography with spatiotemporal image correlation are also shown. Finally, the authors propose a strategy for volume acquisition and postanalysis to spur postanalysis clinical use of this technology. Fetal aortic arch anomalies and pulmonary slings can be difficult to accurately diagnose but have important clinical implications related to vascular rings, congenital heart disease, and chromosomal anomalies. In this article, the authors briefly review the embryology and development of the fetal arch to facilitate understanding of its diverse variants. Two-dimensional echocardiographic characteristics are summarized for each type of these malformations to propose a strategy for fetal diagnosis. The added benefits of three-dimensional echocardiography with spatiotemporal image correlation are also shown. Finally, the authors propose a strategy for volume acquisition and postanalysis to spur postanalysis clinical use of this technology. It is important for fetal cardiac imaging to include evaluation of the fetal upper mediastinum, including identification of the great arteries including the aorta, pulmonary artery, and their connector, the ductus arteriosus. In fact, branching patterns of the great arteries can vary, as can the spatial relationships of the great arteries relative to the trachea and/or esophagus.1Edwards J.E. Anomalies of the derivatives of the aortic arch system.Med Clin North Am. 1948; 32: 925-949Crossref PubMed Scopus (361) Google Scholar Aortic arch anomalies may be related to vascular rings, congenital heart disease, and chromosomal abnormalities.2Yoo S.J. Min J.Y. Lee Y.H. Roman K. Jaeggi E. Smallhorn J. Fetal sonographic diagnosis of aortic arch anomalies.Ultrasound Obstet Gynecol. 2003; 22: 535-546Crossref PubMed Scopus (94) Google Scholar, 3Galindo A. Nieto O. Nieto M.T. Rodriguez-Martin M.O. Herraiz I. Escribano D. et al.Prenatal diagnosis of right aortic arch: associated findings, pregnancy outcome, and clinical significance of vascular rings.Prenat Diagn. 2009; 29: 975-981Crossref PubMed Scopus (63) Google Scholar, 4Miranda J.O. Callaghan N. Miller O. Simpson J. Sharland G. Right aortic arch diagnosed antenatally: associations and outcome in 98 fetuses.Heart. 2014; 100: 54-59Crossref PubMed Scopus (44) Google Scholar, 5Peng R. Xie H.N. Zheng J. Zhou Y. Lin M.F. Fetal right aortic arch: associated anomalies, genetic anomalies with chromosomal microarray analysis, and postnatal outcome.Prenat Diagn. 2017; 37: 329-335Crossref PubMed Scopus (22) Google Scholar Variations of the left pulmonary artery (LPA) or right pulmonary artery (RPA) origins can lead to adverse outcomes if they form a sling surrounding the trachea.6Li X. Mu Z. Li X. Weng Z. Prenatal diagnosis of anomalous origin of pulmonary artery.Prenat Diagn. 2018; 38: 310-317Crossref PubMed Scopus (7) Google Scholar, 7Tretter J.T. Tretter E.M. Rafii D.Y. Anderson R.H. Bhatla P. Fetal diagnosis of abnormal origin of the left pulmonary artery.Echocardiography. 2016; 33: 1258-1261Crossref PubMed Scopus (5) Google Scholar, 8Li S. Luo G. Norwitz E.R. Wang C. Ouyang S. Yao Y. et al.Prenatal diagnosis of congenital vascular rings and slings: sonographic features and perinatal outcome in 81 consecutive cases.Prenat Diagn. 2011; 31: 334-346Crossref PubMed Scopus (44) Google Scholar, 9Binsalamah Z.M. Fraser C.D. Mery C.M. A pulmonary artery sling with a vascular ring in a toddler: an uncommon combination.Cardiol Young. 2018; 28: 783-785Crossref PubMed Scopus (1) Google Scholar A vascular ring or sling can present when vessels in the upper mediastinum encircle and compress the trachea and/or esophagus. With some severe rings (e.g., double aortic arch [DAA]) and pulmonary slings, symptoms of trachea compression often present in early infancy.10Evans W.N. Acherman R.J. Ciccolo M.L. Carrillo S.A. Mayman G.A. Luna C.F. et al.Vascular ring diagnosis and management: notable trends over 25 years.World J Pediatr Congenit Heart Surg. 2016; 7: 717-720Crossref PubMed Scopus (21) Google Scholar Accurate fetal diagnosis helps improve prognosis, as it enables proper timing of surgery or auxiliary ventilation after delivery.11Javia L. Harris M.A. Fuller S. Rings, slings, and other tracheal disorders in the neonate.Semin Fetal Neonatal Med. 2016; 21: 277-284Abstract Full Text Full Text PDF PubMed Scopus (20) Google Scholar,12Backer C.L. Monge M.C. Popescu A.R. Eltayeb O.M. Rastatter J.C. Rigsby C.K. Vascular rings.Semin Pediatr Surg. 2016; 25: 165-175Crossref PubMed Scopus (77) Google Scholar Although postnatal symptoms cannot be predicted for other arch anomalies with or without vascular rings, the vasculature can be fully assessed in utero, facilitating perinatal management. As the ductus turns into a ligament, it is more difficult to visualize postnatally. In addition, even in asymptomatic neonates, bronchoscopy can demonstrate tracheal compression in more than 70% of right aortic arch (RAA) cases that were prenatally diagnosed.13Vigneswaran T.V. Kapravelou E. Bell A.J. Nyman A. Pushparajah K. Simpson J.M. et al.Correlation of symptoms with bronchoscopic findings in children with a prenatal diagnosis of a right aortic arch and left arterial duct.Pediatr Cardiol. 2018; 39: 665-673Crossref PubMed Scopus (15) Google Scholar All of the above factors indicate the importance of fetal diagnosis. In addition, fetal detection of these abnormalities might serve to support chromosomal examinations or detailed echocardiography to screen for congenital heart disease.14Bravo C. Gamez F. Perez R. Alvarez T. De Leon-Luis J. Fetal aortic arch anomalies: key sonographic views for their differential diagnosis and clinical implications using the cardiovascular system sonographic evaluation protocol.J Ultrasound Med. 2016; 35: 237-251Crossref PubMed Scopus (32) Google Scholar However, comprehensive diagnosis of these malformations is challenging because of the diversity of their variations and the multiple sonographic planes required. In this article, we briefly review the embryology and development of the fetal arch and then focus on imaging, a description of normal anatomy, and each of the arch anomalies. We also provide insight into new imaging technologies for diagnosing fetal arch anomalies. We propose a strategy for systematic evaluation of these malformations using two-dimensional (2D) and three-dimensional (3D) echocardiography. In the embryonic arterial system, the caudal sides of each of the two ventral aortas fuse to connect with the truncus arteriosus, while the cranial sides of these ventral aortas connect with the ipsilateral dorsal aorta via six pairs of primitive arches. The bilateral dorsal aortas fuse to form the descending aorta at the midline. The intersegmental arteries migrate and help form the bilateral subclavian arteries.1Edwards J.E. Anomalies of the derivatives of the aortic arch system.Med Clin North Am. 1948; 32: 925-949Crossref PubMed Scopus (361) Google Scholar,15Allen H.D. Driscoll D.J. Shaddy R.E. Feltes T.F. Moss and Adams’ heart disease in infants, children, and adolescents: including the fetus and young adult. Lippincott Williams & Wilkins, Philadelphia2008Google Scholar During the process of development, three of the six primitive arches regress, with only the third, fourth, and sixth arches retained to form the final arch and branches. In brief, the bilateral third arches migrate upward and thus form the carotid artery system; the bilateral fourth arches migrate horizontally to form the transverse arches; and the bilateral sixth arches help form parts of the pulmonary artery and distal ductus.16Larsen W. Human embryology. Churchill Livingstone, Singapore1993Google Scholar At this stage, a DAA system is formed, with bilateral anatomical structures arranged symmetrically (Video 1 available at www.onlinejase.com). Various congenital variants can present during subsequent embryonic development: some segments can regress to form a left aortic arch (LAA) or an RAA with different aortic branching patterns. In rare cases, bilateral aortic arches persist to form a DAA. Usually, the ductus on one side regresses, while that on the other side is retained.12Backer C.L. Monge M.C. Popescu A.R. Eltayeb O.M. Rastatter J.C. Rigsby C.K. Vascular rings.Semin Pediatr Surg. 2016; 25: 165-175Crossref PubMed Scopus (77) Google Scholar Examiners should take the following factors into consideration when evaluating arch anomalies and pulmonary slings17Smith B.M. Lu J.C. Dorfman A.L. Mahani M.G. Agarwal P.P. Rings and slings revisited.Magn Reson Imaging Clin N Am. 2015; 23: 127-135Abstract Full Text Full Text PDF PubMed Scopus (19) Google Scholar, 18Li S. Wen H. Liang M. Luo D. Qin Y. Liao Y. et al.Congenital abnormalities of the aortic arch: revisiting the 1964 Stewart classification.Cardiovasc Pathol. 2019; 39: 38-50Crossref PubMed Scopus (7) Google Scholar, 19Gardiner H. Chaoui R. The fetal three-vessel and tracheal view revisited.Semin Fetal Neonatal Med. 2013; 18: 261-268Abstract Full Text Full Text PDF PubMed Scopus (47) Google Scholar: (1) the origin of the bilateral pulmonary arteries, (2) arch laterality relative to the trachea, (3) sidedness of the ductus relative to the trachea, (4) presence or absence of any vessel behind the trachea, (5) aortic arch branching patterns, and (6) sidedness of the proximal descending aorta in relation to the midline. Much information can be obtained from serial transverse scans when the sound beam is continuously moved cephalad along the vertical axis of the fetal thorax.2Yoo S.J. Min J.Y. Lee Y.H. Roman K. Jaeggi E. Smallhorn J. Fetal sonographic diagnosis of aortic arch anomalies.Ultrasound Obstet Gynecol. 2003; 22: 535-546Crossref PubMed Scopus (94) Google Scholar,20Achiron R. Rotstein Z. Heggesh J. Bronshtein M. Zimand S. Lipitz S. et al.Anomalies of the fetal aortic arch: a novel sonographic approach to in-utero diagnosis.Ultrasound Obstet Gynecol. 2002; 20: 553-557Crossref PubMed Scopus (114) Google Scholar In the three-vessel view (3VV),21Yoo S.J. Lee Y.H. Kim E.S. Ryu H.M. Kim M.Y. Choi H.K. et al.Three-vessel view of the fetal upper mediastinum: an easy means of detecting abnormalities of the ventricular outflow tracts and great arteries during obstetric screening.Ultrasound Obstet Gynecol. 1997; 9: 173-182Crossref PubMed Scopus (188) Google Scholar the beam is sectioned through the long axis of the pulmonary artery and the short-axis of the aorta and superior vena cava. The RPA arises from the pulmonary trunk and courses its way to the right lung hilum behind the aorta and superior vena cava (Figure 1A and B). When the beam is rotated slightly to the left, the pulmonary artery bifurcation becomes visible (Figure 1C and D). The three-vessel and trachea (3VT) view22Yagel S. Arbel R. Anteby E.Y. Raveh D. Achiron R. The three vessels and trachea view (3VT) in fetal cardiac scanning.Ultrasound Obstet Gynecol. 2002; 20: 340-345Crossref PubMed Scopus (195) Google Scholar presents when the beam traverses the confluence of the aortic arch and ductus, which are arranged in a V shape with the trachea on the right side of the vertex (Figure 1E and F). The superior vena cava is on the right side of the arch and is visibly joined by both the left innominate vein and the posteroanterior drainage of the azygos vein at a plane above the 3VT view (Figure 1G and H). When the beam is turned further cephalad, bilateral common carotid arteries and subclavian arteries appear in this oblique transverse plane (Figure 1I and J), which is useful in the diagnosis of aortic arch variants.14Bravo C. Gamez F. Perez R. Alvarez T. De Leon-Luis J. Fetal aortic arch anomalies: key sonographic views for their differential diagnosis and clinical implications using the cardiovascular system sonographic evaluation protocol.J Ultrasound Med. 2016; 35: 237-251Crossref PubMed Scopus (32) Google Scholar In addition to the transverse planes, the long-axis view of the aortic arch can be used to show the continuity of the ascending aorta, aortic arch, and descending aorta. The origin of the neck and head branches can also be identified.23American Institute of Ultrasound in MedicineAIUM practice guideline for the performance of fetal echocardiography.J Ultrasound Med. 2013; 32: 1067-1082Crossref PubMed Scopus (131) Google Scholar The coronal plane sectioned through the proximal descending aorta is commonly used to show the confluence of the arch with the descending aorta, which is usually on the left side of the spine.8Li S. Luo G. Norwitz E.R. Wang C. Ouyang S. Yao Y. et al.Prenatal diagnosis of congenital vascular rings and slings: sonographic features and perinatal outcome in 81 consecutive cases.Prenat Diagn. 2011; 31: 334-346Crossref PubMed Scopus (44) Google Scholar,24Wang Y. Fan M. Siddiqui F.A. Wang M. Sun W. Sun X. et al.Strategies for accurate diagnosis of fetal aortic arch anomalies: benefits of three-dimensional sonography with spatiotemporal image correlation and a novel algorithm for volume analysis.J Am Soc Echocardiogr. 2018; 31: 1238-1251Abstract Full Text Full Text PDF PubMed Scopus (16) Google Scholar In addition, the origin and course of any aberrant left or right subclavian artery that may exist can be clearly identified in this plane.24Wang Y. Fan M. Siddiqui F.A. Wang M. Sun W. Sun X. et al.Strategies for accurate diagnosis of fetal aortic arch anomalies: benefits of three-dimensional sonography with spatiotemporal image correlation and a novel algorithm for volume analysis.J Am Soc Echocardiogr. 2018; 31: 1238-1251Abstract Full Text Full Text PDF PubMed Scopus (16) Google Scholar There are many types of arch anomalies, which can be categorized into LAA, RAA, and DAA. According to different aortic branching patterns, RAA can be further classified into RAA with mirror-image branching, RAA with aberrant left subclavian artery (ALSA) and circumflex retroesophageal RAA. The most common arch anomalies are aberrant right subclavian artery (ARSA) and mirror-image branching RAA, each with an incidence rate of about 2%.15Allen H.D. Driscoll D.J. Shaddy R.E. Feltes T.F. Moss and Adams’ heart disease in infants, children, and adolescents: including the fetus and young adult. Lippincott Williams & Wilkins, Philadelphia2008Google Scholar,25Hanneman K. Newman B. Chan F. Congenital variants and anomalies of the aortic arch.Radiographics. 2017; 37: 32-51Crossref PubMed Scopus (121) Google Scholar Some of the malformations are closely associated with chromosomal abnormalities and specific congenital heart disease.26Zapata H. Edwards J.E. Titus J.L. Aberrant right subclavian artery with left aortic arch: associated cardiac anomalies.Pediatr Cardiol. 1993; 14: 159-161Crossref PubMed Scopus (99) Google Scholar,27McElhinney D.B. Clark III, B.J. Weinberg P.M. Kenton M.L. McDonald-McGinn D. Driscoll D.A. et al.Association of chromosome 22q11 deletion with isolated anomalies of aortic arch laterality and branching.J Am Coll Cardiol. 2001; 37: 2114-2119Crossref PubMed Scopus (184) Google Scholar In DAA and pulmonary slings, mediastinal compression symptoms present in nearly all cases early in infancy11Javia L. Harris M.A. Fuller S. Rings, slings, and other tracheal disorders in the neonate.Semin Fetal Neonatal Med. 2016; 21: 277-284Abstract Full Text Full Text PDF PubMed Scopus (20) Google Scholar; in other malformations, postnatal symptoms are less common. The incidence and clinical relevance of each malformation are summarized in Table 1. Fetal echocardiography can delineate both vascular anatomy and the surrounding mediastinal structures, providing comprehensive information for clinicians. Below, we summarize the sonographic characteristics for each of the arch anomalies and pulmonary slings.Table 1The incidence and clinical details of fetal aortic arch anomalies and pulmonary slingsType of anomalyIncidenceShape of ringChromosomal abnormalityComplicated CHDSymptomsLAA LAA-ARSA-LDA∗Most common arch anomalies.25Hanneman K. Newman B. Chan F. Congenital variants and anomalies of the aortic arch.Radiographics. 2017; 37: 32-51Crossref PubMed Scopus (121) Google Scholar, 26Zapata H. Edwards J.E. Titus J.L. Aberrant right subclavian artery with left aortic arch: associated cardiac anomalies.Pediatr Cardiol. 1993; 14: 159-161Crossref PubMed Scopus (99) Google Scholar, 27McElhinney D.B. Clark III, B.J. Weinberg P.M. Kenton M.L. McDonald-McGinn D. Driscoll D.A. et al.Association of chromosome 22q11 deletion with isolated anomalies of aortic arch laterality and branching.J Am Coll Cardiol. 2001; 37: 2114-2119Crossref PubMed Scopus (184) Google Scholar, 28Svirsky R. Reches A. Brabbing-Goldstein D. Bar-Shira A. Yaron Y. Association of aberrant right subclavian artery with abnormal karyotype and microarray results.Prenat Diagn. 2017; 37: 808-811Crossref PubMed Scopus (16) Google Scholar0.5%–2%CSome association: 21-3†Higher incidence when complicated with CHD. and 22q11‡Higher incidence when complicated with TCA.Most isolated; uncommonly with VSD, TOF, and AVSD90%–93% asymptomatic; very occasionally with esophageal compressionRAA RAA with mirror-image branchingRAA-L-INA-LDA15Allen H.D. Driscoll D.J. Shaddy R.E. Feltes T.F. Moss and Adams’ heart disease in infants, children, and adolescents: including the fetus and young adult. Lippincott Williams & Wilkins, Philadelphia2008Google Scholar,29Zidere V. Tsapakis E.G. Huggon I.C. Allan L.D. Right aortic arch in the fetus.Ultrasound Obstet Gynecol. 2006; 28: 876-881Crossref PubMed Scopus (73) Google Scholar2%NANo reportCommonly associated with other CHDs, such as TOF, TCA, and VSDNoneRAA-L-INA, RDA-DAO30Campanale C.M. Pasquini L. Santangelo T.P. Iorio F.S. Bagolan P. Sanders S.P. et al.Prenatal echocardiographic assessment of right aortic arch.Ultrasound Obstet Gynecol. 2019; 54: 96-102Crossref PubMed Scopus (16) Google ScholarNo reportNANo report30% associated with TCANoneRAA-L-INA, LDA-DAO14Bravo C. Gamez F. Perez R. Alvarez T. De Leon-Luis J. Fetal aortic arch anomalies: key sonographic views for their differential diagnosis and clinical implications using the cardiovascular system sonographic evaluation protocol.J Ultrasound Med. 2016; 35: 237-251Crossref PubMed Scopus (32) Google Scholar3.5‰USome association: 22q11No apparent associationRare with mediastinal compression RAA with ALSARAA-ALSA-LDA14Bravo C. Gamez F. Perez R. Alvarez T. De Leon-Luis J. Fetal aortic arch anomalies: key sonographic views for their differential diagnosis and clinical implications using the cardiovascular system sonographic evaluation protocol.J Ultrasound Med. 2016; 35: 237-251Crossref PubMed Scopus (32) Google Scholar,17Smith B.M. Lu J.C. Dorfman A.L. Mahani M.G. Agarwal P.P. Rings and slings revisited.Magn Reson Imaging Clin N Am. 2015; 23: 127-135Abstract Full Text Full Text PDF PubMed Scopus (19) Google Scholar1‰–1.7‰USome association: 22q11§Very high incidence when the thymus is small or absent.5%–15% associated with CHDs, such as VSD and TOFApproximately 40% with mediastinal compressionRAA-ALSA-RDA15Allen H.D. Driscoll D.J. Shaddy R.E. Feltes T.F. Moss and Adams’ heart disease in infants, children, and adolescents: including the fetus and young adult. Lippincott Williams & Wilkins, Philadelphia2008Google Scholar,30Campanale C.M. Pasquini L. Santangelo T.P. Iorio F.S. Bagolan P. Sanders S.P. et al.Prenatal echocardiographic assessment of right aortic arch.Ultrasound Obstet Gynecol. 2019; 54: 96-102Crossref PubMed Scopus (16) Google Scholar0.3%NANo reportCloser association with TCA: TOF most commonNone Retroesophageal RAA17Smith B.M. Lu J.C. Dorfman A.L. Mahani M.G. Agarwal P.P. Rings and slings revisited.Magn Reson Imaging Clin N Am. 2015; 23: 127-135Abstract Full Text Full Text PDF PubMed Scopus (19) Google Scholar,31Hilmes M. Hernandez R. Devaney E. Markedly hypoplastic circumflex retroesophageal right aortic arch: MR imaging and surgical implications.Pediatr Radiol. 2007; 37: 63-67Crossref PubMed Scopus (14) Google ScholarVery rareURare50%–60% for additional cardiac anomaliesMost asymptomatic; seldom with mediastinal compressionDAA14Bravo C. Gamez F. Perez R. Alvarez T. De Leon-Luis J. Fetal aortic arch anomalies: key sonographic views for their differential diagnosis and clinical implications using the cardiovascular system sonographic evaluation protocol.J Ultrasound Med. 2016; 35: 237-251Crossref PubMed Scopus (32) Google Scholar,15Allen H.D. Driscoll D.J. Shaddy R.E. Feltes T.F. Moss and Adams’ heart disease in infants, children, and adolescents: including the fetus and young adult. Lippincott Williams & Wilkins, Philadelphia2008Google Scholar,27McElhinney D.B. Clark III, B.J. Weinberg P.M. Kenton M.L. McDonald-McGinn D. Driscoll D.A. et al.Association of chromosome 22q11 deletion with isolated anomalies of aortic arch laterality and branching.J Am Coll Cardiol. 2001; 37: 2114-2119Crossref PubMed Scopus (184) Google Scholar0.5%OSome association: 22q11¶Higher incidence when complicated with CHD; higher incidence for DAA with atretic left arch than with hypoplastic left arch.Mostly isolated; seldomly TOFMost with mediastinal compressionPulmonary sling32Sezer S. Acar D.K. Ekiz A. Kaya B. Bornaun H. Aslan H. Prenatal diagnosis of left pulmonary artery sling and review of literature.Echocardiography. 2019; 36: 1001-1004Crossref PubMed Scopus (8) Google Scholar59 per millionCNo report30% associated with CHDsAirway obstruction presents early in most casesAVSD, Atrioventricular septal defect; CHD, congenital heart disease; DAO, descending aorta; LDA, left ductus arteriosus; NA, not applicable; RDA, right ductus arteriosus; TCA, truncus arteriosus; TOF, tetralogy of Fallot; VSD, ventricular septal defect.∗ Most common arch anomalies.† Higher incidence when complicated with CHD.‡ Higher incidence when complicated with TCA.§ Very high incidence when the thymus is small or absent.¶ Higher incidence when complicated with CHD; higher incidence for DAA with atretic left arch than with hypoplastic left arch. Open table in a new tab AVSD, Atrioventricular septal defect; CHD, congenital heart disease; DAO, descending aorta; LDA, left ductus arteriosus; NA, not applicable; RDA, right ductus arteriosus; TCA, truncus arteriosus; TOF, tetralogy of Fallot; VSD, ventricular septal defect. In LAA with ARSA, the aortic arch branching patterns are normal except that the right subclavian artery has a retroesophageal origin. The 3VT view normally shows a V shape, and the ARSA courses rightward behind the trachea and the esophagus. The coronal plane better shows the origin and course of ARSA. Spectral Doppler is routinely used to obtain the arterial flow spectrum to confirm the diagnosis. There are three subtypes of this arch variation according to different ductal positions (relative to the trachea) and connections. The most common situation is retention of the left ductus, which arises from the LPA and then courses its way to connect with the left innominate artery (L-INA) (Figure 2A and B). For fetal sonographic diagnosis, the pulmonary artery and aortic arch are in parallel alignment on the left and right sides of the trachea, respectively, in the 3VT view (Figure 2C). The L-INA can be found when tracing the leftward-coursing vessel that arises from the ascending aorta. The pulmonary branching pattern and the connection of the ductus can be determined by searching along the pulmonary trunk. The 3D reconstructed image better shows the vasculature in space (Figure 2D). Another variant of this anomaly is that the right ductus persists and connects the RPA with the descending aorta (Figure 3A). A 3D image can show the aortic branching patterns, which constitute a true mirror image of a normal LAA (Figure 3B). The 3VT view shows a normal V configuration, with both arch and ductus on the right side of the trachea (Figure 3C). When the vessel arising from the aortic arch and coursing leftward is traced, it is shown to be the L-INA (Figure 3D). The rarest condition for this anomaly is that in which the left ductus persists and connects the LPA with the descending aorta (Figure 4A). Note that a ductal diverticulum is shown in the 3D image, with no vessels originating from it (Figure 4B). In sonographic findings, the 3VT view shows a U shape, indicating an RAA with left ductus (Figure 4C). The aortic branching pattern is clear when the L-INA is seen to originate from the ascending aorta (Figure 4D). Two subtypes of this arch variation may present according to the left/right ductal position in relation to the trachea. Figure 5A and B show the branching pattern of this variation with a left ductus. A characteristic U configuration presents in the 3VT view, which is formed by the left ductus, right arch, and posterior aortic diverticulum (Figure 5C). The distal part of the ALSA might appear when the sound beam is turned slightly cephalad up from the 3VT view. The origin of the ALSA from the Kommerell diverticulum can clearly be seen in the coronal view of the proximal descending aorta (Figure 5D). When the right ductus persists (Figure 5E and F), the retroesophageal left subclavian artery (LSA) arises directly from the descending aorta but not a diverticulum. The 3VT view shows a normal V configuration, except that both the arch and ductus are on the right side of the trachea (Figure 5G). In addition, the origin of the retroesophageal LSA is easy to detect in the 3VT view, with its oblique leftward course better detected in the coronal plane of the proximal descending aorta (Figure 5H). Circumflex retroesophageal RAA is also known as RAA with left descending aorta or right circumflex aorta. The ascending aorta courses upward to the right of the trachea, while the arch turns leftward and downward in a sharply oblique course (Figure 6A and B). In circumflex retroesophageal RAA, the arch itself crosses the midline, usually at the T4-T5 vertebral body. This differs from RAA-ALSA with a left ductus, in which the arch itself is on the right side (the retroesophageal part is the aortic diverticulum), with the descending aorta initially on that side as well but then gradually turning to the left before reaching the level of the diaphragm.15Allen H.D. Driscoll D.J. Shaddy R.E. Feltes T.F. Moss and Adams’ heart disease in infants, children, and adolescents: including the fetus and young adult. Lippincott Williams & Wilkins, Philadelphia2008Google Scholar,25Hanneman K. Newman B. Chan F. Congenital variants and anomalies of the aortic arch.Radiographics. 2017; 37: 32-51Crossref PubMed Scopus (121) Google Scholar,31Hilmes M. Hernandez R. Devaney E. Markedly hypoplastic circumflex retroesophageal right aortic arch: MR imaging and surgical implications.Pediatr Radiol. 2007; 37: 63-67Crossref PubMed Scopus (14) Google Scholar For fetal sonographic diagnosis, the 3VT view presents a “flasklike” shape with a narrow mouth and a wide bottom. The ascending aorta, pulmonary artery/ductus, and arch are located on the right, left, and posterior aspects of the trachea, respectively (Figure 6C). The coronal view of the proximal descending aorta clearly shows that the arch crosses the midline (Figure 6D). In this malformation, both the left and right arches are present, with the ipsilateral carotid artery and subclavian artery arising from their respective arches.33Dillman J.R. Attili A.K. Agarwal P.P. Dorfman A.L. Hernandez R.J. Strouse P.J. Common and uncommon vascular rings and slings: a multi-modality review.Pediatr Radiol. 2011; 41: 1440-1454Crossref PubMed Scopus (30) Google Scholar, 34Etesami M. Ashwath R. Kanne J. Gilkeson R.C. Rajiah P. Computed tomography in the evaluation of vascular rings and slings.Insights Imaging. 2014; 5: 507-521Crossref PubMed Scopus (32) Google Scholar, 35Trobo D. Bravo C. Alvarez T. Perez R. Gamez F. De Leon-Luis J. Prenatal sonographic features of a double aortic arch: literature review and perinatal management.J Ultrasound Med. 2015; 34: 1921-1927Crossref PubMed Scopus (16) Google Scholar Usually, the left-sided ductus is conserved while the right one regresses. When both arches are widely patent, the ductus drains into the end of the LAA, while the bilateral arches drain into a confluence to form a large descending aorta (Figure 7A). When left arch hypoplasia occurs (far more common than symmetric bilateral arches), the left ductus and RAA drain into a convergence to form a large descending aorta, with the end of the left arch as part of the ductus (Figure 7B). The 3D image shows aortic branching patterns, the vascular ring, and configurations of the great arteries in the posterior mediastinum in 3D perspective for these two types of DAA (Figure 7C and D), which is similar to autopsy findings. For DAA with symmetric arches, the 3VT view shows a striking sign of ascending aortic “bifurcation.” Further scanning shows that the two branches arising from the ascending aorta drain into a confluence and encircle the trachea (Figure 7E). Yoo et al.2Yoo S.J. Min J.Y. Lee Y.H. Roman K. Jaeggi E. Smallhorn J. Fetal sonographic diagnosis of aortic arch anomalies.Ultrasound Obstet Gynecol. 2" @default.
• W3096235457 created "2020-11-09" @default.
• W3096235457 creator A5049224717 @default.
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• W3096235457 date "2021-04-01" @default.
• W3096235457 modified "2023-09-27" @default.
• W3096235457 title "Fetal Vascular Rings and Pulmonary Slings: Strategies for Two- and Three-Dimensional Echocardiographic Diagnosis" @default.
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