Fetal Vascular Rings and Pulmonary Slings: Strategies for Two- and Three-Dimensional Echocardiographic Diagnosis
Yu Wang, Ying Zhang
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 the factors into when arch anomalies and pulmonary J.C. and slings Clin Am. Full Text Full Text PDF PubMed Scopus Google Scholar, S. Luo D. Y. Y. et abnormalities of the aortic arch: the 39: PubMed Scopus (7) Google Scholar, R. The fetal and tracheal Fetal Neonatal Med. Full Text Full Text PDF PubMed Scopus Google (1) the origin of the bilateral pulmonary arch relative to the of the ductus relative to the or of the (5) aortic arch branching and of the descending aorta in to the midline. can be transverse when the is the of the fetal 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 R. Z. J. S. S. et of the fetal aortic arch: a sonographic to Obstet Gynecol. PubMed Scopus Google Scholar In the S.J. Lee Y.H. et of the fetal upper an of abnormalities of the and great arteries during Obstet Gynecol. PubMed Scopus Google Scholar the is the of the pulmonary artery and the of the aorta and The the pulmonary and its to the right the aorta and and the is to the the pulmonary artery and The and trachea S. R. D. R. The three vessels and trachea in fetal cardiac Obstet Gynecol. PubMed Scopus Google Scholar when the the of the aortic arch and are arranged in a with the trachea on the right side of the and The is on the right side of the arch and is the left and the of the at a above the and the is bilateral carotid arteries and subclavian arteries in this transverse and is in the diagnosis of aortic arch 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 to the transverse the of the aortic arch can be to the of the aorta, aortic and descending The origin of the and can also be of Ultrasound in for the of fetal Ultrasound Med. 32: PubMed Scopus Google Scholar The the descending aorta is to the of the arch with the descending aorta, is on the left side of the 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 Y. Wang W. X. et for diagnosis of fetal aortic arch anomalies: benefits of three-dimensional with spatiotemporal image correlation and a for volume 2018; 31: Full Text Full Text PDF PubMed Scopus Google Scholar In addition, the origin and of left or right subclavian artery that may can be in this Y. Wang W. X. et for diagnosis of fetal aortic arch anomalies: benefits of three-dimensional with spatiotemporal image correlation and a for volume 2018; 31: Full Text Full Text PDF PubMed Scopus Google Scholar are of arch anomalies, can be into and DAA. to different aortic branching RAA can be into RAA with RAA with left subclavian artery and The arch anomalies are right subclavian artery and branching each with an of 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 K. F. variants and anomalies of the aortic 2017; 37: PubMed Scopus Google Scholar of the malformations are associated with chromosomal abnormalities and congenital heart J.E. right subclavian artery with left aortic arch: associated cardiac Cardiol. PubMed Scopus Google M.L. D. Driscoll et of with anomalies of aortic arch and Cardiol. 37: PubMed Scopus Google Scholar In DAA and pulmonary slings, compression symptoms present in cases early in 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 in other postnatal symptoms are The and clinical of each are summarized in Fetal echocardiography can vascular and the surrounding comprehensive for we the sonographic characteristics for each of the arch anomalies and pulmonary and clinical of fetal aortic arch anomalies and pulmonary of of arch K. F. variants and anomalies of the aortic 2017; 37: PubMed Scopus Google Scholar, J.E. right subclavian artery with left aortic arch: associated cardiac Cardiol. PubMed Scopus Google Scholar, M.L. D. Driscoll et of with anomalies of aortic arch and Cardiol. 37: PubMed Scopus Google Scholar, R. A. D. A. Y. of right subclavian artery with abnormal and microarray Diagn. 2017; 37: PubMed Scopus Google when with and when with with and with RAA with 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 Right aortic arch in the Obstet Gynecol. 28: PubMed Scopus Google associated with other as and C.M. L. P. et al.Prenatal echocardiographic of right aortic Obstet Gynecol. PubMed Scopus Google associated with 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 with compression RAA with 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 J.C. and slings Clin Am. Full Text Full Text PDF PubMed Scopus Google when the is or associated with as and with 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 C.M. L. P. et al.Prenatal echocardiographic of right aortic Obstet Gynecol. 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