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The multiform sonographic spectrum of arterial duct in right aortic arch.

ABSTRACT: To study the different characteristics of arterial duct (AD) in a series of prenatally detected right aortic arch (RAA). Out of 832 congenital heart diseases (CHD) referred to a tertiary center, 98 cases had RAA. Based on anatomical landmarks we identified 7 types of AD: type 1 left-sided, transverse; type 2 left-sided, vertical; type 3 from the underside of aortic arch (AA), vertical; type 4 right-sided, mirror-image "V", transverse; type 5 right-sided, "H" shaped, transverse; type 6 bilateral; type 7 absent or unidentifiable. For each type of AD the incidence of associated major CHD was calculated and chi-square test was applied to verify the null hypothesis with significance level of p < 0.05. Type 1 occurred in 43% of cases including 4 with CHD and no cases with pulmonary outflow obstruction (POO). Symptoms of vascular ring were present in 41% of survivors. Type 2, 3 and 7 AD were associated with tetralogy of Fallot (TOF) or equivalents. No type 5 AD with CHD had POO and 3 isolated cases had asymptomatic hypoplasia of left pulmonary artery (LPA). Two type 6 AD had disconnection of LPA. Type 1 occurred more often as an isolated finding (p < 0.001), whereas types 2 (p = 0.0026), 3 (p = 0.0045), 4 (p = 0.0325) and 7 (p = 0.0001) were frequently associated with major CHD. In RAA, type 1 (U-shaped) is usually an isolated finding (p < 0.001) which includes all symptomatic vascular rings. POO is always present when the AD is vertical or absent but not when it lies on a transverse plane. Bilateral AD is rare and brings the risk of functionary loss of left lung if not identified.

SUBMITTER: Chiappa E

PROVIDER: S-EPMC8604842 | biostudies-literature |

REPOSITORIES: biostudies-literature

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Project description: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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The multiform sonographic spectrum of arterial duct in right aortic arch.

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