VP22.12: Fetal double aortic arch: prenatal sonography and postnatal computed tomography

Abstract Background Fetal double aortic arch (DAA) malformation is a rare congenital heart disease with few reported cases in the literature. We aimed to investigate the characteristics of prenatal ultrasound and postnatal computed tomography angiography (CTA) of DAA and to describe the associated anomalies and clinical outcomes to improve prenatal diagnosis and assist in perinatal management. Methods The obstetric ultrasound imaging databases of seven tertiary referral centers were reviewed retrospectively to identify fetuses with a prenatal diagnosis of DAA between January 2013 and December 2018. Ultrasonographic findings, associated anomalies, genetic abnormalities, postnatal CTA images, and long-term postnatal outcomes were evaluated. Results A total of 36 cases out of 40 prenatally diagnosed DAA fetuses were confirmed by postnatal diagnosis (fetal autopsy, CTA, and surgery). In this cohort of 36 confirmed cases, 24 (67%) were isolated anomalies, while 12 (33%) were associated with intracardiac or extracardiac anomalies, and 2 (6%) had a 22q11.2 chromosome deletion. Among nine cases of pregnancy termination with a fetal autopsy, 7 had other abnormalities. Among the remaining 27 live births, 16 (59%) were asymptomatic and 11 (41%) received surgical treatment due to tracheal or esophageal compression symptoms, all with satisfactory outcomes. Prenatal echocardiography showed that DAA was mainly characterized by a bifurcation of the ascending aorta into the right and left aortic arch and the formation of a complete O-shaped vascular ring around the trachea on the three-vessel tracheal view. A variant in the aortic arch branching pattern was found for the first time. The airway obstruction, branching pattern, and atretic arch of DAA were clearly shown by postnatal CTA. Conclusions Fetal DAA has unique features on prenatal echocardiography and postnatal CTA, and systematic prenatal examination and timely postnatal CTA evaluation are required. A certain proportion of intracardiac and extracardiac abnormalities are associated with DAA, but the probability of chromosome abnormalities is low, especially for isolated DAA.The clinical outcomes of isolated DAA are favorable, even if surgery is performed due to symptoms. Determining whether other malformations or chromosomal anomalies exist is crucial for prognosis evaluation and prenatal counseling.

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Clinical value of multi-slice spiral computed tomography angiography and three-dimensional reconstruction in the diagnosis of double aortic arch

Experimental and Therapeutic Medicine ◽

10.3892/etm.2014.1763 ◽

2014 ◽

Vol 8 (2) ◽

pp. 623-627 ◽

Cited By ~ 4

Author(s):

XIN CHEN ◽

YANJUAN QU ◽

ZHI-YUAN PENG ◽

JINGGUO LU ◽

XIAOJING MA ◽

...

Keyword(s):

Computed Tomography ◽

Aortic Arch ◽

Computed Tomography Angiography ◽

Three Dimensional ◽

Spiral Computed Tomography ◽

Double Aortic Arch ◽

Three Dimensional Reconstruction ◽

Spiral Computed ◽

Clinical Value ◽

Dimensional Reconstruction

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Fetal double aortic arch: prenatal sonographic and postnatal computed tomography angiography features, associated abnormalities and clinical outcomes

10.21203/rs.3.rs-42144/v2 ◽

2020 ◽

Author(s):

Qiao Guo ◽

Yifan Kong ◽

Shi Zeng ◽

Jiawei Zhou ◽

Xiaofang Wang ◽

...

Keyword(s):

Computed Tomography ◽

Prenatal Diagnosis ◽

Aortic Arch ◽

Clinical Outcomes ◽

Computed Tomography Angiography ◽

Double Aortic Arch ◽

Chromosomal Anomalies ◽

Branching Pattern ◽

Associated Anomalies ◽

Fetal Autopsy

Abstract Background: Fetal double aortic arch (DAA) malformation is a rare congenital heart disease with few reported cases in the literature. We aimed to investigate the characteristics of prenatal ultrasound and postnatal computed tomography angiography (CTA) of DAA and to describe the associated anomalies and clinical outcomes to improve prenatal diagnosis and assist in perinatal management. Methods: The obstetric ultrasound imaging databases of seven tertiary referral centers were reviewed retrospectively to identify fetuses with a prenatal diagnosis of DAA between January 2013 and December 2018. Ultrasonographic findings, associated anomalies, genetic abnormalities, postnatal CTA images, and long-term postnatal outcomes were evaluated. Results: A total of 36 cases out of 40 prenatally diagnosed DAA fetuses were confirmed by postnatal diagnosis (fetal autopsy, CTA, and surgery). In this cohort of 36 confirmed cases, 24 (67%) were isolated anomalies, while 12 (33%) were associated with intracardiac or extracardiac anomalies, and 2 (6%) had a 22q11.2 chromosome deletion. Among nine cases of pregnancy termination with a fetal autopsy, 7 had other abnormalities. Among the remaining 27 live births, 16 (59%) were asymptomatic and 11 (41%) received surgical treatment due to tracheal or esophageal compression symptoms, all with satisfactory outcomes. Prenatal echocardiography showed that DAA was mainly characterized by a bifurcation of the ascending aorta into the right and left aortic arch and the formation of a complete O-shaped vascular ring around the trachea on the three-vessel tracheal view. A variant in the aortic arch branching pattern was found for the first time. The airway obstruction, branching pattern, and atretic arch of DAA were clearly shown by postnatal CTA. Conclusions: Fetal DAA has unique features on prenatal echocardiography and postnatal CTA, and systematic prenatal examination and timely postnatal CTA evaluation are required. A certain proportion of intracardiac and extracardiac abnormalities are associated with DAA, but the probability of chromosome abnormalities is low, especially for isolated DAA.The clinical outcomes of isolated DAA are favorable, even if surgery is performed due to symptoms. Determining whether other malformations or chromosomal anomalies exist is crucial for prognosis evaluation and prenatal counseling.

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Fetal double aortic arch: prenatal sonographic and postnatal computed tomography angiography features, associated abnormalities and clinical outcomes

10.21203/rs.3.rs-42144/v1 ◽

2020 ◽

Author(s):

Qiao Guo ◽

Yifan Kong ◽

Shi Zeng ◽

Jiawei Zhou ◽

Xiaofang Wang ◽

...

Keyword(s):

Computed Tomography ◽

Aortic Arch ◽

Clinical Outcomes ◽

Computed Tomography Angiography ◽

Pregnancy Termination ◽

Vascular Ring ◽

Double Aortic Arch ◽

Chromosomal Anomalies ◽

Branching Pattern ◽

Fetal Autopsy

Abstract Background: Fetal double aortic arch (DAA) malformation is a rare congenital heart disease. There is a lack of large sample on comparative study of prenatal ultrasound diagnosis with postnatal computed tomography angiography (CTA) diagnosis, surgery, and autopsy results.Methods: A retrospective cohort study was carried out on cases of fetal DAA that underwent a comprehensive ultrasound examination and were seen during a six-year period at seven tertiary referral centers.Results: A total of 36 cases out of 40 prenatally diagnosed DAA fetuses were confirmed by postnatal diagnosis (fetal autopsy, CTA, and surgery). In this cohort of 36 confirmed cases, 24 (67%) were isolated anomalies, while 12 (33%) were associated with intracardiac or extracardiac anomalies, and 2 (6%) had a 22q11.2 chromosome deletion. There were 9 cases of pregnancy termination with a fetal autopsy. Among the remaining 27 live births, 16 (59%) were asymptomatic and 11 (41%) received surgical treatment due to tracheal or esophageal compression symptoms, all with satisfactory outcomes. Prenatal echocardiography showed that DAA was mainly characterized by a bifurcation of the ascending aorta into the right and left aortic arch and the formation of a complete O-shaped vascular ring around the trachea on the three-vessel tracheal view. A variant in the aortic arch branching pattern was found for the first time. The airway obstruction, branching pattern and atretic arch of DAA were clearly shown by postnatal CTA.Conclusions: Fetal DAA has unique features on prenatal echocardiography and postnatal CTA, and steps and tips for systematic prenatal examination are required. The clinical outcomes of isolated DAA are favorable, even if surgery is performed due to symptoms. Determining whether other malformations or chromosomal anomalies exist is crucial for prognosis evaluation and prenatal decision-making.

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