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Diagnostic and Interventional Imaging (2014) xxx, xxx—xxx
LETTER / Pediatric imaging Right aortic arch and ductus arteriosus: A case diagnosed during the first trimester of pregnancy Keywords: Echocardiography; First trimester; Main vessels; Right aortic arch; Right ductus arteriosus Case report A 25-year-old woman with no significant past history was referred for a first trimester of pregnancy ultrasound. In this investigation which was performed at 11 weeks + 6 days into her pregnancy when the fetus had a cranio-caudal length of 55 mm, color Doppler mode ultrasound showed a mirror image appearance of the convergence of the main vessels of the heart with respect to the trachea (Fig. 1). The color Doppler technical settings were default factory settings with a secondary change only to color gain, PRF and the filter (2D: high harmonic frequency, 80% power, gain −2, C7/M4, SRI II 5/CRI 2; color: low harmonic frequency, 100% power,
gain −9.8, PRF 1.8 kHz, filter WMF max). Based on the reference section of the three-vessel and tracheal view, the diagnosis suggested was a right aortic arch combined with a right ductus arteriosus. A repeat echocardiogram at 16 weeks gestation confirmed the diagnosis and the patient was referred for expert fetal cardiac examination. Specialist echocardiography confirmed the diagnosis of a right aortic arch and ductus arteriosus (Fig. 2) with no other cardiac or morphological abnormalities. As a result of these findings, an amniocentesis was proposed and performed for fetal karyotyping. The fetal karyotype was normal and no 22q11 microdeletion was present. The remainder of the pregnancy proceeded normally and the patient gave birth at term to a healthy child. Discussion Aortic arch abnormalities are well known and have been widely described in the literature for many years. The right aortic arch and double aortic arch have been known about for a long time although a right ductus arteriosus has however only occasionally been described. Yoo et al. described
Figure 1. Visualization during the first trimester of pregnancy of the aortic arch and ductus arteriosus located to the right (11 + 4 weeks of gestation) and to the left (12 weeks of gestation) of the trachea. Ant: anterior; Post: posterior; R: right; L: left; TPA: trunk of the pulmonary artery; DA: ductus arteriosus; AAo: ascending aorta; DAo: descending aorta; T: trachea. http://dx.doi.org/10.1016/j.diii.2014.03.015 2211-5684/© 2014 Éditions franc ¸aises de radiologie. Published by Elsevier Masson SAS. All rights reserved.
Please cite this article in press as: Lafouge A, Quarello E. Right aortic arch and ductus arteriosus: A case diagnosed during the first trimester of pregnancy. Diagnostic and Interventional Imaging (2014), http://dx.doi.org/10.1016/j.diii.2014.03.015
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Letter
Figure 2. Visualization at 17 weeks of the aortic arch and ductus arteriosus located to the right of the trachea. Ant: anterior; Post: posterior; R: right; L: left; TPA: trunk of the pulmonary artery; DA: ductus arteriosus; AAo: ascending aorta; DAo: descending aorta; MB: main bronchus; LPA: left pulmonary artery; SVC: superior vena cava.
a model to explain the pathophysiology of the occurrence of anomalies in the development of aortic arches from the Edwards’ diagram [1]. Not all studies about these aortic arch abnormalities are consistent. Some authors report a routine association with right ductus arteriosus and its associated abnormalities [2] whereas others only very occasionally find co-existent abnormalities and particularly report no karyotype or 22q11 microdeletions [3]. These differences in conclusions are explained principally by the population studied as the population examined by Berg et al. [2] was one at high risk of abnormalities. This was a tertiary care center in which all of the patients were referred with concomitant signs. Conversely, the population examined by Bronshtein et al. [3] involved 19,874 pregnancies at low risk of malformations. Bronshtein et al. found 40 right aortic arches, 4 of which (10%) were associated with a right ductus arteriosus. All of the cases had a normal karyotype, including region 11 of the long arm of chromosome 22. In practice, left or right laterality or double aortic arches and ductus arteriosus are identified from an axial section, examining their respective positions in relation to the trachea. In 1997 Yoo et al. proposed an axial section of the superior mediastinum to view the trunk of the pulmonary artery which extends into the ductus arteriosus, ascending aorta, right superior vena cava and carena using an axial section of the superior mediastinum to view the vessels from left to right and from anterior to posterior [4]. This is called the ‘‘three-vessel’’ view (Fig. 3). In 2002 Yagel et al. proposed a slight amendment to the level at which Yoo’s view was examined. Yagel’s axial view was slightly higher in the mediastinum than Yoo’s above the carena and was proposed because of a need to visualize the
Figure 3. The Yoo’s three-vessel view. Ant: anterior; Post: posterior; R: right; L: left; TPA: trunk of the pulmonary artery; DA: ductus arteriosus; AAo: ascending aorta; DAo: descending aorta; MB: main bronchus; LPA: left pulmonary artery; Az: azygous vein; SVC: superior vena cava.
trachea and the convergence of the terminal end of the ductus arteriosus and the isthmus portion of the aorta [5]. This is the so-called ‘‘three-vessel and tracheal’’ view (Fig. 4). The novel feature of our case is firstly the rare finding of a combination of a right aortic arch with a right ductus arteriosus and secondly the early diagnosis in the first trimester of pregnancy. It is important to emphasize that the general use of screening echocardiography in the first trimester is still debated [6] although our case indicates its early feasibility by using color Doppler modes and/or energy to examine the large vessels of the heart.
Figure 4. The Yagel’s three-vessel and tracheal view. Ant: anterior; Post: posterior; R: right; L: left; TPA: trunk of the pulmonary artery; DA: ductus arteriosus; AAo: ascending aorta; DAo: descending aorta; T: trachea; SVC: superior vena cava.
Please cite this article in press as: Lafouge A, Quarello E. Right aortic arch and ductus arteriosus: A case diagnosed during the first trimester of pregnancy. Diagnostic and Interventional Imaging (2014), http://dx.doi.org/10.1016/j.diii.2014.03.015
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Letter Disclosure of interest The authors declare that they have no conflicts of interest concerning this article. References [1] Yoo SJ, Min JY, Lee YH, Roman K, Jaeggi E, Smallhorn J. Fetal sonographic diagnosis of aortic arch anomalies. Ultrasound Obstet Gynecol 2003;22(5):535—46. [2] Berg C, Bender F, Soukup M, Geipel A, Axt-Fliedner R, Breuer J, et al. Right aortic arch detected in fetal life. Ultrasound Obstet Gynecol 2006;28(7):882—9. [3] Bronshtein M, Zimmer EZ, Blazer S, Blumenfeld Z. Right ductus arteriosus: facts and theory. Eur J Obstet Gynecol Reprod Biol 2011;159(2):282—8. [4] Yoo SJ, Lee YH, Kim ES, Ryu HM, Kim MY, Choi HK, et al. Threevessel 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(3):173—82.
3 [5] Yagel S, Arbel R, Anteby EY, Raveh D, Achiron R. The three-vessels and trachea view (3VT) in fetal cardiac scanning. Ultrasound Obstet Gynecol 2002;20(4): 340—5. [6] Carvalho JS. Screening for heart defects in the first trimester of pregnancy: food for thought. Ultrasound Obstet Gynecol 2010;36(6):658—60.
A. Lafouge a , E. Quarello b,c,∗ a
Gynecology and Obstetrics Offices, 7, avenue Gambetta, 83400 Hyères, France b Reproductive Medicine Institute, 6, rue Rocca, 13008 Marseille, France c Obstetric Ultrasound and Antenatal Diagnosis Unit, Saint-Joseph Hospital, 25, boulevard de Louvain, 13285 Marseille cedex 08, France ∗ Corresponding author. E-mail address: [email protected] (E. Quarello)
Please cite this article in press as: Lafouge A, Quarello E. Right aortic arch and ductus arteriosus: A case diagnosed during the first trimester of pregnancy. Diagnostic and Interventional Imaging (2014), http://dx.doi.org/10.1016/j.diii.2014.03.015
ARTICLE IN PRESS
Diagnostic and Interventional Imaging (2014) xxx, xxx—xxx
LETTER / Pediatric imaging Right aortic arch and ductus arteriosus: A case diagnosed during the first trimester of pregnancy Keywords: Echocardiography; First trimester; Main vessels; Right aortic arch; Right ductus arteriosus Case report A 25-year-old woman with no significant past history was referred for a first trimester of pregnancy ultrasound. In this investigation which was performed at 11 weeks + 6 days into her pregnancy when the fetus had a cranio-caudal length of 55 mm, color Doppler mode ultrasound showed a mirror image appearance of the convergence of the main vessels of the heart with respect to the trachea (Fig. 1). The color Doppler technical settings were default factory settings with a secondary change only to color gain, PRF and the filter (2D: high harmonic frequency, 80% power, gain −2, C7/M4, SRI II 5/CRI 2; color: low harmonic frequency, 100% power,
gain −9.8, PRF 1.8 kHz, filter WMF max). Based on the reference section of the three-vessel and tracheal view, the diagnosis suggested was a right aortic arch combined with a right ductus arteriosus. A repeat echocardiogram at 16 weeks gestation confirmed the diagnosis and the patient was referred for expert fetal cardiac examination. Specialist echocardiography confirmed the diagnosis of a right aortic arch and ductus arteriosus (Fig. 2) with no other cardiac or morphological abnormalities. As a result of these findings, an amniocentesis was proposed and performed for fetal karyotyping. The fetal karyotype was normal and no 22q11 microdeletion was present. The remainder of the pregnancy proceeded normally and the patient gave birth at term to a healthy child. Discussion Aortic arch abnormalities are well known and have been widely described in the literature for many years. The right aortic arch and double aortic arch have been known about for a long time although a right ductus arteriosus has however only occasionally been described. Yoo et al. described
Figure 1. Visualization during the first trimester of pregnancy of the aortic arch and ductus arteriosus located to the right (11 + 4 weeks of gestation) and to the left (12 weeks of gestation) of the trachea. Ant: anterior; Post: posterior; R: right; L: left; TPA: trunk of the pulmonary artery; DA: ductus arteriosus; AAo: ascending aorta; DAo: descending aorta; T: trachea. http://dx.doi.org/10.1016/j.diii.2014.03.015 2211-5684/© 2014 Éditions franc ¸aises de radiologie. Published by Elsevier Masson SAS. All rights reserved.
Please cite this article in press as: Lafouge A, Quarello E. Right aortic arch and ductus arteriosus: A case diagnosed during the first trimester of pregnancy. Diagnostic and Interventional Imaging (2014), http://dx.doi.org/10.1016/j.diii.2014.03.015
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Letter
Figure 2. Visualization at 17 weeks of the aortic arch and ductus arteriosus located to the right of the trachea. Ant: anterior; Post: posterior; R: right; L: left; TPA: trunk of the pulmonary artery; DA: ductus arteriosus; AAo: ascending aorta; DAo: descending aorta; MB: main bronchus; LPA: left pulmonary artery; SVC: superior vena cava.
a model to explain the pathophysiology of the occurrence of anomalies in the development of aortic arches from the Edwards’ diagram [1]. Not all studies about these aortic arch abnormalities are consistent. Some authors report a routine association with right ductus arteriosus and its associated abnormalities [2] whereas others only very occasionally find co-existent abnormalities and particularly report no karyotype or 22q11 microdeletions [3]. These differences in conclusions are explained principally by the population studied as the population examined by Berg et al. [2] was one at high risk of abnormalities. This was a tertiary care center in which all of the patients were referred with concomitant signs. Conversely, the population examined by Bronshtein et al. [3] involved 19,874 pregnancies at low risk of malformations. Bronshtein et al. found 40 right aortic arches, 4 of which (10%) were associated with a right ductus arteriosus. All of the cases had a normal karyotype, including region 11 of the long arm of chromosome 22. In practice, left or right laterality or double aortic arches and ductus arteriosus are identified from an axial section, examining their respective positions in relation to the trachea. In 1997 Yoo et al. proposed an axial section of the superior mediastinum to view the trunk of the pulmonary artery which extends into the ductus arteriosus, ascending aorta, right superior vena cava and carena using an axial section of the superior mediastinum to view the vessels from left to right and from anterior to posterior [4]. This is called the ‘‘three-vessel’’ view (Fig. 3). In 2002 Yagel et al. proposed a slight amendment to the level at which Yoo’s view was examined. Yagel’s axial view was slightly higher in the mediastinum than Yoo’s above the carena and was proposed because of a need to visualize the
Figure 3. The Yoo’s three-vessel view. Ant: anterior; Post: posterior; R: right; L: left; TPA: trunk of the pulmonary artery; DA: ductus arteriosus; AAo: ascending aorta; DAo: descending aorta; MB: main bronchus; LPA: left pulmonary artery; Az: azygous vein; SVC: superior vena cava.
trachea and the convergence of the terminal end of the ductus arteriosus and the isthmus portion of the aorta [5]. This is the so-called ‘‘three-vessel and tracheal’’ view (Fig. 4). The novel feature of our case is firstly the rare finding of a combination of a right aortic arch with a right ductus arteriosus and secondly the early diagnosis in the first trimester of pregnancy. It is important to emphasize that the general use of screening echocardiography in the first trimester is still debated [6] although our case indicates its early feasibility by using color Doppler modes and/or energy to examine the large vessels of the heart.
Figure 4. The Yagel’s three-vessel and tracheal view. Ant: anterior; Post: posterior; R: right; L: left; TPA: trunk of the pulmonary artery; DA: ductus arteriosus; AAo: ascending aorta; DAo: descending aorta; T: trachea; SVC: superior vena cava.
Please cite this article in press as: Lafouge A, Quarello E. Right aortic arch and ductus arteriosus: A case diagnosed during the first trimester of pregnancy. Diagnostic and Interventional Imaging (2014), http://dx.doi.org/10.1016/j.diii.2014.03.015
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ARTICLE IN PRESS
Letter Disclosure of interest The authors declare that they have no conflicts of interest concerning this article. References [1] Yoo SJ, Min JY, Lee YH, Roman K, Jaeggi E, Smallhorn J. Fetal sonographic diagnosis of aortic arch anomalies. Ultrasound Obstet Gynecol 2003;22(5):535—46. [2] Berg C, Bender F, Soukup M, Geipel A, Axt-Fliedner R, Breuer J, et al. Right aortic arch detected in fetal life. Ultrasound Obstet Gynecol 2006;28(7):882—9. [3] Bronshtein M, Zimmer EZ, Blazer S, Blumenfeld Z. Right ductus arteriosus: facts and theory. Eur J Obstet Gynecol Reprod Biol 2011;159(2):282—8. [4] Yoo SJ, Lee YH, Kim ES, Ryu HM, Kim MY, Choi HK, et al. Threevessel 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(3):173—82.
3 [5] Yagel S, Arbel R, Anteby EY, Raveh D, Achiron R. The three-vessels and trachea view (3VT) in fetal cardiac scanning. Ultrasound Obstet Gynecol 2002;20(4): 340—5. [6] Carvalho JS. Screening for heart defects in the first trimester of pregnancy: food for thought. Ultrasound Obstet Gynecol 2010;36(6):658—60.
A. Lafouge a , E. Quarello b,c,∗ a
Gynecology and Obstetrics Offices, 7, avenue Gambetta, 83400 Hyères, France b Reproductive Medicine Institute, 6, rue Rocca, 13008 Marseille, France c Obstetric Ultrasound and Antenatal Diagnosis Unit, Saint-Joseph Hospital, 25, boulevard de Louvain, 13285 Marseille cedex 08, France ∗ Corresponding author. E-mail address: [email protected] (E. Quarello)
Please cite this article in press as: Lafouge A, Quarello E. Right aortic arch and ductus arteriosus: A case diagnosed during the first trimester of pregnancy. Diagnostic and Interventional Imaging (2014), http://dx.doi.org/10.1016/j.diii.2014.03.015
