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Observational Study
. 2020 Oct-Dec;61(4):1173-1184.
doi: 10.47162/RJME.61.4.19.

Prenatal findings and pregnancy outcome in fetuses with right and double aortic arch. A 10-year experience at a tertiary center

Ana Maria Petrescu  1 , Dan Ruican, Ciprian Laurenţiu Pătru, George Lucian Zorilă, Ştefania Tudorache, Alexandru Cristian Comănescu, Anca Maria Istrate-Ofiţeru, Anne Marie Badiu, Mihai Ioana, George Alin Stoica, Dominic Gabriel Iliescu
Affiliations
Observational Study

Prenatal findings and pregnancy outcome in fetuses with right and double aortic arch. A 10-year experience at a tertiary center

Ana Maria Petrescu et al. Rom J Morphol Embryol. 2020 Oct-Dec.

Abstract

Objective: Our objective was to evaluate the accuracy of the prenatal diagnosis and the relation between the type of right aortic arch (RAA) with other intra- or extracardiac (EC) and chromosomal anomalies.

Methods: A retrospective, observational study was conducted between 2011-2020 in a Romanian tertiary center. All RAA cases, including double aortic arch (DAA), were extracted from the databases and studied thoroughly.

Results: We detected 18 RAA cases: five (27.78%) type I (mirror image, "V" type), 11 (61.12%) type II ("U" type), and two (11.10%) DAA cases. Heart anomalies were associated in 38.89% (overall), 60% (type I), 36.37% (type II), and 0% (DAA) cases. Tetralogy of Fallot represented the most prevalent cardiac malformation (in 22.23% of cases). EC anomalies were present in 44.44% of fetuses (20% of type I, 54.55% of type II, and 50% of DAA cases). Genetic abnormalities were found in 41.17% of pregnancies, with 22q11.2 deletion in 23.53%. 55.55% of the cases had a good neonatal evolution and 44.45% of the pregnancies were terminated. An overall good outcome of pregnancy was noted in 40% of type I RAA, 63.64% of type II RAA, and 50% of DAA cases. All RAA cases examined in the first trimester were correctly diagnosed.

Conclusions: RAA can be accurately diagnosed and classified by means of prenatal ultrasound since early pregnancy. A detailed anatomy scan and genetic testing, including 22q11 deletion, should be offered to all pregnancies when RAA is discovered. When isolated, RAA associates a good outcome, indifferently the anatomical type.

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Conflict of interest statement

The authors declare that they have no conflict of interests.

Figures

Figure 1
Figure 1
– (A) Normal embryological development and involution (with dotted lines) of the six pairs of aortic arches (noted with Nos. from 1 to 6); (B) Normal LAA with LDA; (C) RAA with RDA – type I; (D) RAA with LDA forming Kommerell’s diverticulum – type II; (E) DAA surrounding the trachea and esophagus. Ao: Aorta; DA: Ductus arteriosus; DAA: Double aortic arch; DAo: Descending aorta; E: Esophagus; LAA: Left aortic arch; LCA: Left common artery; LDA: Left ductus arteriosus; LP: Left pulmonary artery; LSA: Left subclavian artery; P: Pulmonary trunk; RAA: Right aortic arch; RCA: Right common artery; RDA: Right ductus arteriosus; RP: Right pulmonary artery; RSA: Right subclavian artery; T: Trachea
Figure 2
Figure 2
Presenting Case No. 12 (Table 1) – type I RAA case with right DA and cardiac anomaly: (A) Ultrasound directional power Doppler, four-chamber view; (B) VSD with overriding aorta; (C) RAA with right DA; (D) Pathology examination – RAA; (E) Right DA; (F) Heart dissection – VSD. Ao: Aorta; DA: Ductus arteriosus; DAo: Descending aorta; L: Left; LCA: Left carotid artery; LSA: Left subclavian artery; PA: Pulmonary trunk; R: Right; RAA: Right aortic arch; RCA: Right carotid artery; RSA: Right subclavian artery; RVOT: Right ventricular outflow tract; VSD: Ventricular septal defect
Figure 3
Figure 3
Presenting Case No. 13 – type II RAA case with a good postpartum evolution following surgical correction of associated abnormalities: (A) Ultrasound duplex mode – grey scale and directional power Doppler, four-chamber view, showing normal situs, area and axis of the heart; (B) RAA and left DA, connected by the Kommerell’s diverticulum appear as a “U”-shaped vascular ring around the trachea and esophagus – “U” sign; (C) First day postpartum radiography showing esophageal pouch (esophageal atresia was confirmed); (D) Postpartum CT for esophageal atresia management reveals dilated stomach and duodenum – “double bubble” sign, suggesting duodenal atresia and horseshoe kidney; (E) 47,XY,+mar – normal karyotype with chromosome marker. Ao: Aorta; CT: Computed tomography; DA: Ductus arteriosus; Dd: Duodenum; EA: Esophageal atresia; KD: Kidney; L: Left; PA: Pulmonary trunk; R: Right; RAA: Right aortic arch; S: Stomach; T: Trachea
Figure 4
Figure 4
DAA in a second trimester pregnancy termination (Case No. 14). Ultrasound cardiac sweep with high-definition directional power Doppler mode: (A) Four-chamber view, with normal cardiac situs; (B) Left ventricular outflow tract plane, with aorta emerging from the left ventricle and coursing to the right of the spine; (C) Right ventricular outflow tract plane (pulmonary artery) and a DAA is identified in the three vessels plane, with a narrower left aortic arch; (D) Left-sided DA. Pathology examination: (E) RAA view with emergent vessels; (F) Left aortic arch with emergent vessels; (G) Vascular ring formed by the two arches; (H) Heart isolation with DAA and emergent vessels of each arch. Ao: Aorta; DA: Ductus arteriosus; DAA: Double aortic arch; DAo: Descending aorta; LCA: Left carotid artery; LSA: Left subclavian artery; LVN: Left vagus nerve; PA: Pulmonary trunk; RAA: Right aortic arch; RCA: Right carotid artery; RSA: Right subclavian artery; T: Trachea
Figure 5
Figure 5
Distribution of the genetic anomalies and IC/EC malformations and association with the outcome of the pregnancies. aCGH: Microarray-based comparative genomic hybridization; DAA: Double aortic arch; EC: Extracardiac; IC: Intracardiac; iRAA: Isolated right aortic arch; TOP: Termination of pregnancy
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