# Pneumococcal vaccination incentive campaign in chronic heart failure patients in France: a cluster-randomized trial

Hugo Guillermou, Grégoire Mercier, Bernard Litovsky, Laurence Papinaud, François Roubille

► To cite this version:

Hugo Guillermou, Grégoire Mercier, Bernard Litovsky, Laurence Papinaud, François Roubille. Pneumococcal vaccination incentive campaign in chronic heart failure patients in France: a cluster-randomized trial. European Journal of Preventive Cardiology, 2025, pp.zwaf529. 10.1093/eur-jpc/zwaf529. hal-05229340

HAL Id: hal-05229340

https://hal.science/hal-05229340v1

Submitted on 4 Feb 2026

HAL is a multi-disciplinary open access
archive for the deposit and dissemination of sci-
entific research documents, whether they are pub-
lished or not. The documents may come from
teaching and research institutions in France or
abroad, or from public or private research centers.

L'archive ouverte pluridisciplinaire HAL, est
destinée au dépôt et à la diffusion de documents
scientifiques de niveau recherche, publiés ou non,
émanant des établissements d'enseignement et de
recherche français ou étrangers, des laboratoires
publics ou privés.

<figure><img src="image_1.png" /></figure>

Distributed under a Creative Commons CC BY 4.0 - Attribution - International License
# Pneumococcal vaccination incentive campaign in chronic heart failure patients in France: a cluster-randomized trial

Hugo Guillermou<fnref n="1" />, Grégoire Mercier<fnref n="1" />,<fnref n="2" />, Bernard Litovsky<fnref n="3" />, Laurence Papinaud<fnref n="3" />, François Roubille<fnref n="1" />,<fnref n="4" />

## Keywords:

Cardiology; Heart Failure; Pneumococcal Vaccines; Pragmatic Clinical Trials as Topic; Vaccination Coverage.

<footnote n="1">¹ University Hospital of Montpellier, Montpellier, France.</footnote>

<footnote n="2">² UMR IDESP, INSERM, Montpellier University, Montpellier, France.</footnote>

<footnote n="3">³ Information Systems Unit at the Regional Medical Office of the Assurance Maladie, Montpellier, France.</footnote>

<footnote n="4">⁴ PhyMedExp, Cardiology Department, University of Montpellier, INSERM U1046, CNRS UMR, 9214; INI-CRT, Montpellier, France.</footnote>
# Abstract

**Background:**

Pneumococcal vaccination rates are critically low among high-risk patients, particularly those with heart failure (HF), who face increased risk of severe infections and complications. There is limited evidence on the effectiveness of simple interventions to improve vaccination uptake in this population.

**Objectives:**

A prospective interventional trial was designed to evaluate the impact of an incentive campaign carried out by the French health insurance.

**Methods:**

This study was an open-label pragmatic cluster randomized controlled trial involving general practitioners (GPs) and their related patients with chronic HF in a French region, identified through the regional health insurance database. Data were routinely collected using this database to conduct the study. HF patients were assigned to intervention or control groups based on the randomized allocation of their treating GPs. In the intervention group, both GPs and their patients received two vaccination incentive letters, whereas the control group was only observed. Vaccines were provided at no cost under the French vaccination program. The primary endpoint was the pneumococcal vaccination rate at one year. The secondary objective aimed to identify factors associated with vaccine uptake.

**Results:**

3952 GPs and 12 285 corresponding patients with HF were included between Mar 23, 2023, and Mar 23, 2024. The pneumococcal vaccination rate at one year was 35% in the intervention group (n = 6140) versus 3.5% in the control group (n = 5937). Multivariable analyses showed a 15-fold higher rate of vaccination in patients receiving the intervention. The vaccination rate increased with male patients, female GP, and previous influenza vaccination but decreased in younger (<70 years) and older patient age (>85 years).

**Discussion:**

This randomized study demonstrated the effectiveness of a mail-in promotion to sustainably increase pneumococcal vaccination coverage in a frail high-risk population.
## WHAT IS ALREADY KNOWN ON THIS TOPIC

⇒ Improvement in foetal cardiac screening has contributed to improving prenatal diagnosis of aortic arch anomalies, and its prenatal diagnosis requires precise anatomical description to determine the risk of subsequent vascular rings.

## WHAT THIS STUDY ADDS

⇒ Prenatal diagnosis of the isolated right aortic arch is accurate, with a good concordance between prenatal and postnatal ultrasound regarding aortic arch position. The rare discrepancies between prenatal and postnatal diagnoses concerned the double aortic arch and the pattern of brachiocephalic vessel branching.

## HOW THIS STUDY MIGHT AFFECT RESEARCH, PRACTICE OR POLICY

⇒ The existence of asymptomatic forms of vascular ring anomalies with tracheal compression supports the value of systematic postnatal CT scan after prenatal diagnosis of an isolated right aortic arch.

# INTRODUCTION

Prenatal diagnosis of congenital variants and anomalies of the aortic arch has been given increasing attention, as these anomalies may be associated with vascular rings, congenital heart defects (CHD) and chromosomal abnormalities, with important implications for prognosis and management.¹ The additional role of the three-vessel and trachea view in foetal cardiac screening has contributed to improving prenatal diagnosis of aortic arch anomalies.²

Within the spectrum of aortic arch anomalies, right aortic arch (RAA) has been commonly related to conotruncal CHD. Nevertheless, the existence of isolated forms of RAA, that is, without any CHD, raises the question of whether it is an anatomical variant or a serious vascular defect.³ RAA prenatal diagnosis requires precise anatomical description to determine the risk of subsequent vascular rings. This RAA can be observed in a solitary aortic arch or in a double aortic arch (DAA) with a variable distribution of the supra-aortic vessels and a left or right ductus arteriosus. Certain forms lead to vascular rings which may be postnatally symptomatic, depending on the degree of tracheal or oesophageal compression.³ ⁴ Thus, symptoms appear earlier and more frequently in infants with DAA,⁵ are less predictable in RAA with left aberrant subclavian artery⁶ and usually absent in non-ring forms, such as RAA with right ductus arteriosus.⁷
The performance of prenatal screening of RAA has been previously evaluated in terms of anatomical diagnostic accuracy and prediction of postnatal outcome. 7–9 Classically, the evidence of an RAA in foetal ultrasound is the first step before diagnosing a vascular ring. The foetal sonographer will then need to specify the ductus arteriosus lateralisation, the distribution of brachiocephalic vessels and the possibility of an additional left arch. Then, after birth, the cardiac sonographer should validate the diagnosis of RAA and specify the vascular ring. Nevertheless, the role of postnatal gold standard cardiac imaging, such as CT scan or cardiac magnetic resonance (CMR), remains controversial. 1 6 8 9

From a large multicentric cohort, this study aimed to describe the implications of RAA prenatal diagnosis, in terms of prenatal and postnatal diagnostic concordance.

# METHODS

## Study design and patient population

This retrospective observational multicentric study was conducted in 14 French centres experts for prenatal diagnosis in foetal cardiology, from January 2010 to September 2019.

All cases of prenatal visualisation of a right-sided aortic arch identified from each centre's database and confirmed by a foetal cardiologist were screened, identified as an RAA and defined as an aortic arch running to the right and posterior to the trachea. Cases with complex intracardiac anomalies were excluded, as well as cases resulting in termination of pregnancy. Cases with minor associated cardiac lesions were not excluded if no significant haemodynamic consequence at birth was expected.

In clinical practice, the prenatal visualisation of a right-sided aortic arch may result in two main diagnoses, for example, the solitary right aortic arch (S-RAA) or the DAA. 10 11 The S-RAA is characterised by a single RAA and divided into two subtypes depending on the ductus arteriosus course (right or left). The DAA is characterised by an RAA and, commonly, a hypoplastic left arch. This classification has also been recently used by Bet *et al*. 12

A variety of patterns of brachiocephalic vessel branching have been described in S-RAA. 13 The most common pattern is the S-RAA with an aberrant left subclavian artery, with or without a Kommerell's diverticulum, and the second most common type is the S-RAA with mirror image branching.

For the DAA, the most common form is a dominant RAA with a hypoplastic left arch (whose distal extremity may be atretic) and a symmetrical distribution of brachiocephalic vessels.

The various prenatal RAA ultrasound images (2D ultrasound, colour Doppler ultrasound and anatomical diagram) are illustrated in figure 1.
<figure><img src="image_2.png" /><figcaption>**Figure 1** Prenatal ultrasound diagnosis of RAA. This represents the various step 1 ultrasound images (2D ultrasound, colour Doppler ultrasound, and anatomical diagram). ALSA, aberrant left subclavian artery; DAA, double aortic arch; DAA-LD, double aortic arch with left ductus arteriosus; L, left; RAA, right aortic arch; RAA-LD, right aortic arch with left ductus arteriosus; RAA-mirror, right aortic arch with mirror-image branching; RAA-RD, right aortic arch with right ductus arteriosus; S-RAA, solitary right aortic arch.</figcaption></figure>

<figure><img src="image_3.png" /><figcaption>**Figure 2** Flowchart. RAA, right aortic arch. ARCADE, acronym of the study (prenatal ultrasound diagnosis of a right-sided aortic arch).</figcaption></figure>
**Data collection**

The data were collected and classified into three steps, according to the stage of the diagnostic process. Prenatal description of RAA type, subtype and pattern of brachiocephalic vessel distribution was performed using the foetal ultrasound (Us) data report (prenatal Us, step 1). Postnatal echocardiography reports were analysed to assess the description of RAA type (postnatal Us, step 2). In patients who underwent additional cardiac imaging with CT scan (CT scan, step 3), we collected the indication of the scan either on a systematic basis or based on symptoms, and the CT scan results: specific anatomy of RAA and the related degree of tracheal compression according to Myer's classification (mild<50%; moderate 51%-70%; severe>70%). The genetic analyses were collected.

Lastly, neonatal and 1-year outcomes, including the onset of compressive symptoms and
related management, were analysed.

**Statistical analysis**

The study population was described with means and SD for quantitative variables and with numbers and percentages for qualitative ones. The concordance between the different diagnostic stages (prenatal Us, step 1; postnatal Us, step 2; and CT scan, step 3) was evaluated by Gwet's AC1 coefficient, which provides better stability in case of substantial imbalance of the marginal totals of the table. Values less than 0.5, between 0.5 and 0.75, between 0.75 and 0.9 and greater than 0.90, were indicative of poor, moderate, good and excellent reliability, respectively.

The statistical significance was set at 0.05 and analyses were performed using the software
SAS Enterprise Guide, V.7.13.

RESULTS

**Population**

Over the 10-year study period, 312 prenatally diagnosed cases of isolated RAA were identified in the 14 investigating centres. We excluded three cases marked by a termination of pregnancy, all related to 22q11.1 microdeletion. Overall, a total of 309 cases with RAA were included in the study and analysed for step 1 (prenatal Us). The step 2 group (postnatal Us) included 286 paediatric subjects (93% of the cohort) and the step 3 group (postnatal CT scan) included 108 paediatric subjects (35% of the cohort). Figure 2 illustrates the flowchart. The table 1 summarises the population characteristics.

**Prenatal diagnosis reliability**

Description of step 1 group (prenatal Us) S-RAA was prenatally diagnosed for 281 foetuses (91%) and DAA for 28 foetuses. The ductus arteriosus was described in most cases (91%) and located on the left side for 84% of cases in the S-RAA group. Prenatal ultrasound described
the anatomy of the supra-aortic branch vessels in only 27% of cases. Step 1 results are described in figure 3.

<table><caption>**Table 1** Population characteristics</caption><thead><tr><th></th><th></th><th></th></tr></thead><tbody><tr><td>N</td><td></td><td>309</td></tr><tr><td>Sex ratio (male/female)</td><td></td><td>0.94</td></tr><tr><td>Birth term (weeks of gestation)</td><td></td><td>38.2±2.2</td></tr><tr><td>Genetic analyses</td><td></td><td>165 (53.4)</td></tr><tr><td rowspan="3">Genetic abnormalities</td><td>Microdeletion 22q11.1</td><td>6 (1.9)</td></tr><tr><td>Trisomy 21</td><td>1 (0.3)</td></tr><tr><td>Others</td><td>5 (1.6)</td></tr><tr><td>Minor cardiac defects found on postnatal Us</td><td></td><td>46 (14.9)</td></tr><tr><td rowspan="3">Symptomatic forms of RAA</td><td>In the overall RAA cohort</td><td>50 (16.2)</td></tr><tr><td>In the S-RAA group (N=281)</td><td>41 (12.9)</td></tr><tr><td>In the DAA group (N=28)</td><td>9 (2.9)</td></tr><tr><td rowspan="3">Type of symptoms</td><td>Respiratory</td><td>34 (11)</td></tr><tr><td>Digestive</td><td>7 (2.3)</td></tr><tr><td>Respiratory and digestive</td><td>9 (2.9)</td></tr><tr><td>Aortic arch surgery during first year of life</td><td></td><td>24 (7.8)</td></tr><tr><td>Mean age at surgery (months)</td><td></td><td>5.7±3.6</td></tr><tr><td rowspan="2">Indication for surgery</td><td>Prophylactic</td><td>1 (0.3)</td></tr><tr><td>Symptoms</td><td>23 (7.4)</td></tr><tr><td rowspan="3">Type of surgery</td><td>Double aortic arch repair</td><td>8 (2.6)</td></tr><tr><td>Resection of Kommerell's diverticulum</td><td>11 (3.6)</td></tr><tr><td>Ligamentum arteriosum section</td><td>5 (1.6)</td></tr><tr><td rowspan="2">Surgical complications</td><td>Chylothorax</td><td>3 (1)</td></tr><tr><td>Tracheomalacia</td><td>1 (0.3)</td></tr><tr><td colspan="3">Values were mean±SD or N (%). DAA, double aortic arch; RAA, right aortic arch; S-RAA, solitary right aortic arch; Us, ultrasound.</td></tr></tbody></table>

<figure><img src="image_4.png" /><figcaption>**Figure 3** Prenatal ultrasound diagnosis of RAA (step 1). *aortic vessels description without ductus lateralisation. ALSA, aberrant left subclavian artery; DAA, double aortic arch; DAA-LD, double aortic arch with left ductus arteriosus; MD, missing data; RAA, right aortic arch; RAA-LD, right aortic arch with left ductus arteriosus; RAA-mirror, right aortic arch with mirror-image branching; RAA-RD, right aortic arch with right ductus arteriosus; S-RAA, solitary right aortic arch.</figcaption></figure>
Concordance between step 1 (prenatal Us) and step 2 (postnatal Us) diagnoses

The concordance between prenatal and postnatal ultrasound diagnoses (step 1 vs step 2) was excellent regarding the RAA type (AC1=0.97, 95% CI=(0.94 to 0.99)). Diagnoses were discordant in nine cases: two patients had a postnatal echocardiographic diagnosis of left aortic arch; four patients with a prenatal diagnosis of S-RAA had a postnatal echocardiographic diagnosis of DAA and inversely, three patients with a prenatal diagnosis of DAA had a postnatal echocardiographic diagnosis of S-RAA.

Concordance between step 1 (prenatal Us) and step 3 (postnatal CT scan) diagnoses

In the step 3 group (n=108), the CT scan was performed on 57 patients (53%) presenting with
symptoms and on a systematic basis to other patients. The concordance between prenatal Us
and postnatal CT scan (step 1 vs step 3) was good regarding the RAA type (AC1=0.80, 95%
CI=(0.69 to 0.90)). CT scan corrected 15 misclassified prenatal diagnoses of RAA: 5 children
with a prenatal diagnosis of S-RAA had a postnatal CT scan diagnosis of DAA and 10
patients with a prenatal diagnosis of DAA had a postnatal CT scan diagnosis of S-RAA.
Figure 4 illustrates the concordance between all steps. The concordance between step 1
(prenatal Us) and step 3 (postnatal CT scan) was poor regarding the distribution of
brachiocephalic vessels (AC1=0.21, 95% CI=(0.06 to 0.36)). CT scan revealed tracheal
compression in 60 (61%) patients, of which 26 cases of moderate-to- severe compression, and
found additional vascular complications in 9 patients (10%), such as subclavian artery
stenosis.

<figure><img src="image_5.png" /><figcaption>**Figure 4** Diagnostic concordance between step 1 (prenatal ultrasound), step 2 (postnatal ultrasound) and step 3 (CT scan). The blue arrow represents the diagnostic stages in the RAA population between step 1 (prenatal ultrasound), step 2 (postnatal ultrasound) and step 3 (CT scan). The red arrow represents the diagnostic stages in the DAA population between step 1 (prenatal ultrasound), step 2 (postnatal ultrasound) and Step 3 (CT scan). The colour changes of the arrows represent the diagnostic changes at each step, between RAA and DAA (blue to red) and between DAA and RAA (red to blue). AC1, concordance coefficient; DAA, double aortic arch; S-RAA, solitary right aortic arch; Step 1, prenatal ultrasound; Step 2, postnatal ultrasound; Step 3, CT scan.</figcaption></figure>
## Genetic disorders associated with prenatal isolated RAA

Over half of the cohort (n=165, 53%) underwent genetic testing, of which 154 cases were tested prenatally. Overall, genetic anomalies were identified for 12 cases, of which 6 had a 22q11.1 microdeletion. Among prenatal tests, genetic anomalies were identified in seven cases, of which four had an S-RAA with no other extracardiac defect. Interestingly, we found one case combining S-RAA, normal thymus gland and 22q11.1 microdeletion. The rate of genetic anomalies in the tested cohort was 7% (4% of the general cohort). The incidence of 22q11.1 microdeletion was 4% in the tested cohort (2% of the general cohort).

## Vascular ring compressive symptoms and management

At birth, 11 neonates (4%) presented with clinical symptoms suggestive of a vascular ring, such as stridor. Neonatal respiratory distress was never reported, and none required any respiratory assistance in relation to the vascular ring. During the first year of life, 50 patients (16%) were symptomatic, of which 43 presented with respiratory symptoms. Symptoms were noted in 32% of DAA and 15% of S-RAA. Aortic arch surgery was performed in 24 infants (8%) during the first year of life. Among them, eight patients (33%) were in the DAA group, of which one asymptomatic patient underwent prophylactic surgery. Surgery was performed with no mortality.

Postsurgical complications were observed in four patients (17%). However, no infant with a CT scan diagnosis of additional vascular complications, such as subclavian artery stenosis, underwent surgery during the first year of life.

Among the 51 patients who underwent systematic CT scan, 25 showed moderate-to- Severe tracheal compression on CT scan, of which 3 cases experienced symptoms related to airway compression and 1 case underwent aortic arch surgery during the first year of life.

## DISCUSSION

This multicentric retrospective cohort of 309 foetal cases confirmed the reliability of prenatal diagnosis regarding isolated RAA, highlighted the rare cases of discrepancies between prenatal and postnatal diagnosis and underlined the value of CT scan to improve the postnatal follow-up.

This study showed that prenatal diagnosis of an isolated RAA was accurate, with a good-to- excellent concordance between prenatal and postnatal ultrasound regarding aortic arch position.

Lateralisation of the ductus arteriosus was most frequently notified (>90%) with a high incidence of the left ductus (>80%), ensuring the ring character. These results are in line with previous studies,¹⁶⁻²⁰ but provide a higher level of evidence by providing statistical concordance analyses between prenatal and postnatal ultrasound diagnoses. As parental stress induced by prenatal diagnosis is commonly increased by prognostic uncertainties, ²¹ optimal prenatal counselling after diagnosing an RAA should address several aspects tending to better
predict the postnatal outcome and involve genetic counselling. Indeed, in this cohort, the isolated RAA was associated with genetic disorders in 7% of tested cases, and with 22q11.1 microdeletion in 4% of tested cases. These rates align with similar studies on the tested population as shown in the recent meta-analysis from Luo et al (7.5% and 4.3%, respectively).22 Moreover, in our study, these rates are diminished by half in the absence of additional extracardiac anomaly, which is in line with the two existing meta-analyses. 22 23 Therefore, based on our results and supported by the recent literature, invasive laboratory diagnostic testing should be offered in prenatally diagnosed RAA with a low risk of genetic anomaly.

In this study, the rare discrepancies between prenatal and postnatal diagnoses mainly concerned the DAA, strengthening what had been highlighted in previous reports.7 24 Despite the use of certain key ultrasound techniques such as ‘9 or Z shapes’, or the visualisation of different arch orientations, prenatal diagnosis of DAA sometimes remains difficult.7 10 25 Misdiagnosing a DAA by an S-RAA or inversely may have serious consequences on prenatal counselling and postnatal management, as DAA is more often and earlier symptomatic than other vascular ring anomalies. 5 10 26 To limit this risk, postnatal cross-sectional imaging, such as CT scan, appears necessary for an accurate diagnosis of DAA.26–28 The limits of ultrasound diagnosis were even more relevant for supra-aortic trunk description in S-RAA. Indeed, in this study, even for the aberrant left subclavian artery, that is, the most frequent and probably least difficult-to- diagnose anomaly of the aortic arch, accurate prenatal ultrasound description was made in less than 50% of cases. Therefore, CT scan and CMR are helpful additional exams to investigate patients with vascular ring anomalies, by providing an accurate description of aortic arch anatomy based on surgical observations29 and enabling the detection of associated vascular anomalies.30 Moreover, CT scan has a higher density resolution than CMR for tracheal airway assessment with limited radiation and no general anaesthesia. 31 Although the practice of a systematic CT scan is still disputed,4 10 we, like others,16 30 32 consider it necessary for an accurate anatomical diagnosis.

In terms of postnatal management, this study confirmed the low incidence and poor specificity of clinical symptoms in vascular ring anomalies, as previously reported.18 33 Aortic arch surgery was performed in only 8% of infants during their first year of life. The surgical indication is often difficult to establish, supporting systematic CT scan to provide relevant information on the existence of tracheal compression and its importance.34 35 Vigneswaran et al have also suggested routine bronchoscopy,35 but this examination requires general anaesthesia and, in our study, was only carried out in the event of significant CT compression and preoperatively. Overall, the management of asymptomatic patients with vascular ring anomalies remains controversial.9 Systematic surgery is usually recommended for DAA,5 and prophylactic surgery for all forms of vascular ring anomaly has also been supported to limit complications related to prolonged tracheal or oesophageal compression in adulthood. 36 37 This study suggests that a systematic surgical approach in vascular rings may not be relevant. Considering the potential surgical complications38 and the usual good evolution of RAA in children,39 a close clinical follow-up appears as appropriate management, especially when guided by CT scan anatomical and functional assessment.
**Study limitation**

This retrospective study was carried out in expert centres in foetal cardiology, and therefore
might underestimate non-diagnosed cases of RAA from general prenatal diagnosis settings.
Moreover, the study was based on data collection from electronic medical records. Results on
genetic disorders may have been underestimated, as three cases of termination of pregnancy
were excluded. Using a classification involving DAA in a study on RAA may seem
confusing, as they are not related to the same embryological mechanism. However, this
assumption was motivated by the fact that a prenatal RAA could be reclassified as DAA and
vice versa. Postnatal CT scan was performed in only 35% of cases, therefore, prospective
cohort studies with a prolonged patient follow-up remain necessary to confirm the value of
systematic CT scan after prenatal diagnosis of RAA.

CONCLUSION

This multicentric cohort of 309 foetal cases showed that prenatal diagnosis of the isolated RAA was accurate, with an excellent concordance between prenatal and postnatal ultrasound diagnoses, in terms of aortic arch position. The rare discrepancies between prenatal and postnatal diagnoses concerned the DAA and the pattern of brachiocephalic vessel branching, the latter remaining difficult to assess by ultrasound. In addition to misdiagnoses, the existence of asymptomatic forms of vascular ring anomalies with tracheal compression supported the value of systematic postnatal CT scan after prenatal diagnosis of an isolated RAA.

Acknowledgements

The authors would like to thank the patients and their families for agreeing to participate in
this study. We thank Sebastien Mounier for his work on the graphic design.

Collaborators

The ARCADE study group: Marianne Peyre, MD (Foetal, Paediatric and Adult Congenital Cardiology Clinic, Marseille, France); Isabelle Durand, MD (Paediatric and Congenital Cardiology Department, M3C Regional Reference Centre, Rouen, University Hospital, Rouen, France); Tristan Hazelzet, MD (Paediatric and Congenital Cardiology Department, M3C Regional Reference Centre, Rouen, University Hospital, Rouen, France); Pauline Gras, MD (Paediatric and Congenital Cardiology Department, M3C Regional Reference Centre, Lille University Hospital, Lille, France); Matthias Lachaud, MD (Paediatric and Congenital Cardiology Department, M3C Regional Reference Centre, Grenoble University Hospital, Grenoble, France); Herve Joly, MD (Paediatric and Congenital Cardiology Department, Val d'Ouest Clinic, Lyon, France); Guy Vaksmann, MD (Paediatric and Congenital Cardiology Department, La Louviere Clinic, Lille, France); Florent Paoli, MD (Paediatric and Congenital Cardiology Department, M3C Regional Reference Centre, La Timone Marseille, University Hospital, Marseille, France); Claire Bertail, MD (Paediatric and Congenital Cardiology Department, M3C Regional Reference Centre, Louis Pradel
Lyon University Hospital, Lyon, France); Yvan Bouzguenda, MD (Paediatric and Congenital Cardiology Department, La Louviere Clinic, Lille, France); Sylvie Falcon Eicher, MD (Paediatric and Congenital Cardiology Department, M3C Regional Reference Centre, Dijon University Hospital, Dijon, France).

**Contributors**

SG, MV, GA and PA directed, conceptualised and designed the study, enrolled patients, drafted the initial manuscript, reviewed and revised the manuscript. FT, DL, JT and LC enrolled patients and revised the manuscript. HH, M-CP, MD, HA and A-CH supervised the data management, performed the analyses and revised the manuscript. PA is the guarantor responsible for the overall content. All authors approved the final manuscript as submitted and agree to be accountable for all aspects of the work.

**Funding**

The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

**Competing interests**

None declared.

**Patient consent for publication**

Not applicable.

**Ethics approval**

This study involves human participants. The study was conducted in compliance with the Good Clinical Practices protocol and Declaration of Helsinki principles, was approved by institutional review board of Montpellier University Hospital (2019_IRB-MTP_03-31) and registered on ClinicalTrials. Gov (NCT04029064). Participants gave informed consent to participate in the study before taking part.

**Provenance and peer review**

Not commissioned; externally peer reviewed.

**Data availability statement**

Data are available on reasonable request.
REFERENCES

1 Hanneman K, Newman B, Chan F. Congenital variants and anomalies of the aortic arch. *Radiographics* 2017;37:32–51.

2 McGahan JP, James G, Hedriana H, et al. Key features on the 3-vessel view and 3-vessel tracheal view of isolated right aortic arch anomalies. *Ultrasound Q* 2020;36:235–9.

3 Evans WN, Acherman RJ, Ciccolo ML, et al. Vascular ring diagnosis and management: notable trends over 25 years. *World J Pediatr Congenit Heart Surg* 2016;7:717–20.

4 Evans WN, Acherman RJ, Ciccolo ML, et al. Right aortic arch with situs solitus frequently heralds a vascular ring. *Congenit Heart Dis* 2017;12:583–7.

5 Alsenaidi K, Gurofsky R, Karamlou T, et al. Management and outcomes of double aortic arch in 81 patients. *Pediatrics* 2006;118:e1336–41.

6 Backer CL, Mongé MC, Popescu AR, et al. Vascular rings. *Semin Pediatr Surg* 2016;25:165–75.

7 Razon Y, Berant M, Fogelman R, et al. Prenatal diagnosis and outcome of right aortic arch without significant intracardiac anomaly. *J Am Soc Echocardiogr* 2014;27:1352–8.

8 Aly S, Papneja K, Mawad W, et al. Prenatal diagnosis of vascular ring: evaluation of fetal diagnosis and postnatal outcomes. *J Am Soc Echocardiogr* 2022;35:312–21.

9 Stephens EH, Eltayeb O, Kennedy C, et al. Influence of fetal diagnosis on management of vascular rings. *Ann Thorac Surg* 2022;113:630–6.

10 Van Poppel MPM, Zidere V, Simpson JM, et al. Fetal echocardiographic markers to differentiate between a right and double aortic arch. *Prenat Diagn* 2022;42:419–27.

11 Yoo S -J., Min J -Y., Lee Y -H., et al. Fetal sonographic diagnosis of aortic arch anomalies. *Ultrasound Obstet Gynecol* 2003;22:535–46.

12 Bet BB, Snoep MC, van Leeuwen E, et al. Short-term outcome after the prenatal diagnosis of right aortic arch. *Prenat Diagn* 2023;43:629–38.

13 Edwards JE. Anomalies of the derivatives of the aortic arch system. *Med Clin North Am* 1948;32:925–49.

14 Myer CM 3rd, O'Connor DM, Cotton RT. Proposed grading system for subglottic stenosis based on endotracheal tube sizes. *Ann Otol Rhinol Laryngol* 1994;103:319–23.

15 Gwet KL. Handbook of inter-rater reliability: the definitive guide to measuring the extent of agreement among raters. Fourth Edition. *Advances Analytics, LLC*, 2014.

16 Bakhru S, Koneti NR, Patil S, et al. Prenatal diagnosis of vascular rings and outcome. *Ann Pediatr Cardiol* 2021;14:359–65.
17 Galindo A, Nieto O, Nieto MT, et al. Prenatal diagnosis of right aortic arch: associated findings, pregnancy outcome, and clinical significance of vascular rings. *Prenat Diagn* 2009;29:975-81.

18 Miranda JO, Callaghan N, Miller O, et al. Right aortic arch diagnosed antenatally: associations and outcome in 98 fetuses. *Heart* 2014;100:54-9.

19 Li S, Luo G, Norwitz ER, et al. Prenatal diagnosis of congenital vascular rings and slings: sonographic features and perinatal outcome in 81 consecutive cases: prenatal diagnosis of vascular rings. *Prenat Diagn* 2011;31:334-46.

20 Vigneswaran TV, Allan L, Charakida M, et al. Prenatal diagnosis and clinical implications of an apparently isolated right aortic arch. *Prenat Diagn* 2018;38:1055-61.

21 Bratt EL, Järvholm S, Ekman-Joelsson BM, et al. Parent's experiences of counselling and their need for support following a prenatal diagnosis of congenital heart disease - a qualitative study in a Swedish context. *BMC Pregnancy Childbirth* 2015;15:171.

22 Luo Q, Chen J, Zhang Y, et al. Incidence of chromosomal anomalies in fetuses with isolated right aortic arch: a meta-analysis. *Prenat Diagn* 2020;40:294-300.

23 D'Antonio F, Khalil A, Zidere V, et al. Fetuses with right aortic arch: a multicenter cohort study and meta-analysis. *Ultrasound Obstet Gynecol* 2016;47:423-32.

24 Zidere V, Tsapakis EG, Huggon IC, et al. Right aortic arch in the fetus. *Ultrasound Obstet Gynecol* 2006;28:876-81.

25 Achiron R, Rotstein Z, Heggesh J, et al. Anomalies of the fetal aortic arch: a novel sonographic approach to in-utero diagnosis. *Ultrasound Obstet Gynecol* 2002;20:553-7.

26 Biermann D, Holst T, Hüners I, et al. Right aortic arch forming a true vascular ring: a clinical review. *Eur J Cardiothorac Surg* 2021;60:1014-21.

27 Chen X, Qu Y, Peng ZY, et al. Clinical value of multi-slice spiral computed tomography angiography and three-dimensional reconstruction in the diagnosis of double aortic arch. *Exp Ther Med* 2014;8:623-7.

28 Bartsota M, Jowett V, Manuel D, et al. Double aortic arch: implications of antenatal diagnosis, differential growth of arches during pregnancy, associated abnormalities and postnatal outcome. *Ultrasound Obstet Gynecol* 2023;62:69-74.

29 Leonardi B, Secinaro A, Cutrera R, et al. Imaging modalities in children with vascular ring and pulmonary artery sling: the evaluation of aortic arch anomalies in children. *Pediatr Pulmonol* 2015;40:781-8.

30 Arazińska A, Polguj M, Szymczyk K, et al. Right aortic arch analysis – anatomical variant or serious vascular defect? *BMC Cardiovasc Disord* 2017;17:102.
31 Xu R, Shi K, Yang Z, et al. Quantified evaluation of tracheal compression in pediatric complex congenital vascular ring by computed tomography. *Sci Rep* 2018;8:11183.

32 Campanale CM, Pasquini L, Santangelo TP, et al. Prenatal echocardiographic assessment of right aortic arch. *Ultrasound Obstet Gynecol* 2019;54:96-102.

33 Licari A, Manca E, Rispoli GA, et al. Congenital vascular rings: a clinical challenge for the pediatrician. *Pediatr Pulmonol* 2015;50:511-24.

34 Etesami M, Ashwath R, Kanne J, et al. Computed tomography in the evaluation of vascular rings and slings. *Insights Imaging* 2014;5:507-21.

35 Vigneswaran TV, Kapravelou E, Bell AJ, et al. Correlation of symptoms with bronchoscopic findings in children with a prenatal diagnosis of a right aortic arch and left arterial duct. *Pediatr Cardiol* 2018;39:665-73.

36 Worhunsky DJ, Levy BE, Stephens EH, et al. Vascular rings. *Semin Pediatr Surg* 2021;30:151128.

37 Said SM, Marey G, Knutson S, et al. Outcomes of surgical repair of vascular rings and slings in children: a word for the asymptomatic. *Semin Thorac Cardiovasc Surg* 2021;33:492-500.

38 Naimo PS, Fricke TA, Donald JS, et al. Long-term outcomes of complete vascular ring division in children: a 36-year experience from a single institution. *Interact Cardiovasc Thorac Surg* 2017;24:234-9.

39 Loomba RS. Natural history of asymptomatic and unrepaired vascular rings: is watchful waiting a viable option? A new case and review of previously reported cases. *Children (Basel)* 2016;3:44.
<figure><img src="image_6.png" /></figure>