Management of Prenatally Diagnosed Isolated Right Aortic Arch

NCT04029064 | Completed

• 1 other identifier: RECHMPL19_0146
• Study Type: observational
• Target: 300
• Locations: 1 country
• Sites: 1

Brief Summary

The existence of a right aortic arch integrates nosologically into the group of aortic vascular arch anomalies. In the majority of cases, a right aortic arch is associated with cono-truncal congenital heart diseases (CHD), such as tetralogy of Fallot, pulmonary atresia with ventricular septal defect, and truncus arteriosus. Sometimes, a right aortic arch can be isolated, with an incidence of 0.05% to 0.1 %. Therefore, an assessment of the anatomy is necessary because an isolated right aortic arch can be associated with an encircling aortic arch anomaly and may subsequently have a functional repercussion, creating a digestive obstacle (oesophageal compression) and/or a respiratory obstacle (tracheal compression). Furthermore, indications for surgery in this type of abnormalities are not well-established. The diagnosis of a vascular artery anomaly is currently mostly made prenatally, with increasing diagnostic accuracy. Indeed, the application of the "3VT view" (e.g. three vessels and tracheae view) in routine prenatal sonographic screening for CHD has contributed to a better identification of such anomalies. Prenatal diagnosis of an isolated right aortic arch involves the identification of the following aspects: the existence of a left ductus arteriosus may relate to an increased risk of an encircling aortic arch anomaly at birth; the analysis of the different vessels of the aortic arch, which can be difficult because of the fetal circulation and the maternal-fetal conditions of the examination; and the absence of a thymus, which suggests the existence of a genetic anomaly, such as DiGeorge syndrome. After birth, the evaluation of the functional repercussion of the anomaly remains complex. The incidence of secondary symptoms to encircling abnormalities is estimated at 25%, but varies according to the anomaly: it is very frequent in case of a double aortic arch, variable in case of a right aortic arch with left ductus arteriosus (from 9% to 47% in the literature), absent in case of right aortic arch with right ductus arteriosus. Postnatal echocardiography can only assess the existence of a right aortic arch and check ductus arteriosus closure. The complete anatomical diagnosis of a vascular artery anomaly can only be made by slice cardiac imaging (CT scan or MRI), but in practice these examinations are not routinely performed in the absence of neonatal symptoms. The aim of this study is to evaluate the accuracy of prenatally diagnosed isolated right aortic arch in terms of anatomy and functional prognosis. The secondary aims are :

• to specify the association of an isolated right aortic arch with a genetic anomaly,
• to define the role of CT scan in children prenatally diagnosed with right aortic arch, in terms of diagnosis, prognosis and follow-up,
• to evaluate the incidence of respiratory and digestive complications in the first year of life,
• to evaluate the indications for surgical management during the first year of life.

Conditions

Isolated Right Aortic Arch

Keywords

right aortic arch; congenital heart disease; pediatrics; cardiology; prenatal diagnosis

Outcome Measures

Primary Outcomes (1)

• Correlation between the antenatal description of isolated right aortic arch and the postnatal description and functional prognosis

  Correlation between the antenatal description of isolated right aortic arch and the postnatal description and functional prognosis

  1 day

Secondary Outcomes (4)

• Rate of genetic anomaly with an isolated right aortic arch

  1 day

• Rate of realization of chest CT angiography before the first year of life with an isolated right aortic arch

  1 day

• Rate of surgical repair during the first year of life after prenatal diagnosis of an isolated right aortic arch.

  1 day

• Rate of respiratory and digestive complications in the first year of life.

  1 day

Eligibility Criteria

• Age: Up to 1 Year
• Sex: all
• Healthy Volunteers: No
• Age Groups: Child (0-17)
• Sampling Method: Non-Probability Sample

Study Population

Children with antenatal diagnosis of isolated right aortic arch

You may qualify if:

• Prenatal diagnosis of an isolated right aortic arch

You may not qualify if:

• Association with any congenital heart disease according to the ACC-CHD (Orphanet) classification
• Postnatal diagnosis of an isolated right aortic arch
• Refusal of parents to use medical data

Sponsors & Collaborators

• University Hospital, Montpellier: lead
• Congeital heart disease explorations unit - UE3C Lowendal - Paris: collaborator
• University Hospital, Rouen: collaborator
• Nantes University Hospital: collaborator
• University Hospital, Grenoble: collaborator
• University Hospital, Bordeaux: collaborator
• Clinique La louvère - Lille - France: collaborator
• University Hospital, Tours: collaborator
• University Hospital, Caen: collaborator
• University Hospital, Toulouse: collaborator
• Hospices Civils de Lyon: collaborator
• University Hospital, Marseille: collaborator
• Amiens University Hospital: collaborator
• Martinique University Hospital: collaborator
• Centre Hospitalier Universitaire Dijon: collaborator
• Private cardiology practice, Massy: collaborator
• Private cardiology practice, Marseille: collaborator
• Private cardiology practice, Pontoise: collaborator
• Private cardiology practice, Strasbourg: collaborator
• Private cardiology practice, Brest: collaborator
• Private cardiology practice, Rennes: collaborator

Study Sites (1)

Uh Montpellier

Montpellier, 34295, France

Location

Related Publications (1)

• Guillaumont S, Vincenti M, Thomas F, Huguet H, Picot MC, Abassi H, Huby AC, Laux D, Thomas-Chabaneix J, Cohen L, Gavotto A, Amedro P; ARCADE study group. Implications of right aortic arch prenatal diagnosis: the multicentric nationwide ARCADE cohort. Arch Dis Child Fetal Neonatal Ed. 2025 Feb 21;110(2):138-144. doi: 10.1136/archdischild-2024-327242.

  PMID: 38964845 DERIVED

MeSH Terms

Conditions

Heart Defects, Congenital

Condition Hierarchy (Ancestors)

Cardiovascular Abnormalities; Cardiovascular Diseases; Heart Diseases; Congenital Abnormalities; Congenital, Hereditary, and Neonatal Diseases and Abnormalities

Study Officials

• Sophie GUILLAUMONT, MD

  University Hospital, Montpellier

  PRINCIPAL INVESTIGATOR

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: RETROSPECTIVE
• Sponsor Type: OTHER
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: April 25, 2019
• First Posted: July 23, 2019
• Study Start: April 1, 2019
• Primary Completion: December 1, 2019
• Study Completion: December 31, 2019
• Last Updated: March 10, 2020

Record last verified: 2019-03

Locations

FR (1)