Review Reports - Vascular Rings as a Cause of Respiratory Difficulties

Round 1

Reviewer 1 Report

Comments and Suggestions for Authors

This manuscript presents a clinically relevant retrospective case series of pediatric patients with vascular rings and slings, including an uncommon combination of right aortic arch with aberrant left subclavian artery and pulmonary artery sling. The topic is appropriate for the journal and may be useful to pediatricians, pediatric cardiologists, pulmonologists, radiologists, and cardiothoracic surgeons. The manuscript also benefits from a clinically oriented description of presentation, diagnostic imaging, and surgical management. However, several methodological, numerical, and reporting issues should be addressed before the manuscript is suitable for publication.

1. Study design and patient selection.
The Methods section should describe the cohort assembly more precisely. Please state whether the seven patients represent all consecutive eligible patients diagnosed or followed at the institution during 2021–2025, provide explicit inclusion and exclusion criteria, explain how cases were identified from the hospital information system, and clarify whether any potentially eligible cases were excluded. The duration and completeness of postoperative follow-up should also be reported. Given the very small sample size, the study should consistently be described as a retrospective descriptive case series rather than implying population-level estimates.

2. Statistical presentation and handling of “outliers”.
With only seven patients, the repeated exclusion of individual patients as “outliers” requires reconsideration. These older patients are clinically relevant observations rather than statistical anomalies and should not be removed solely because their ages differ substantially from those of the remaining patients. I recommend presenting the complete cohort using median, range and/or interquartile range as the primary descriptive measures, with any restricted pediatric/early-presentation analysis clearly identified as a sensitivity or descriptive subgroup analysis. The criteria used to define an outlier should otherwise be stated explicitly.

3. Numerical inconsistencies must be reconciled throughout the manuscript, tables, abstract, and supplementary dataset.
Several discrepancies are currently present. For example, the text reports a mean age at diagnosis of 56.75 months, whereas Table 1 reports 57.5 months. The supplementary patient-level table supports 56.75 months. In addition, the Results state that cyanosis occurred in 4 patients and vomiting in 4 patients, whereas Table 2 reports 5 patients for each variable. The supplementary dataset supports 4/7 for both cyanosis and vomiting. Please perform a complete audit of all counts, percentages, means, medians, and denominators against the source dataset.

4. CT angiography reporting is internally inconsistent.
Section 3.4 states that definitive diagnosis was obtained by CT angiography in all seven patients (100%). However, the Discussion subsequently states that CT angiography was used in 6 patients (71.4%). Moreover, 6/7 would correspond to 85.7%, not 71.4%. The supplementary patient-level dataset indicates CT angiography in all seven patients. This should be corrected consistently throughout the manuscript.

5. There is an important factual error concerning DiGeorge syndrome.
The Discussion refers to “21q11.2 deletion syndrome (DiGeorge syndrome).” This should be corrected to 22q11.2 deletion syndrome. Given the relevance of genetic abnormalities to vascular rings, the authors should carefully verify the genetics terminology throughout the manuscript.

6. Ethics reporting requires substantial clarification.
The manuscript currently provides only a generic statement that the procedures complied with ethical standards and that patient consent was waived because of the retrospective design. Please provide the full name of the approving Institutional Review Board/Ethics Committee, approval or waiver number, and approval date. The authors should also clarify whether the waiver specifically covered retrospective use of pediatric clinical and imaging data. Because Figure 1 contains patient-derived imaging and the cohort includes very rare phenotypes that may increase re-identification risk, the authors should clarify whether separate consent for publication was required or formally waived according to institutional policy.

7. Surgical complication denominators should be clarified.
Six of seven patients underwent surgery, whereas postoperative complications occurred in two patients. The manuscript reports this as 28.5%, apparently using the entire cohort as the denominator. When comparing postoperative complication rates with published surgical cohorts, the clinically appropriate denominator is the number of operated patients; therefore, this corresponds to 2/6 (33.3%). Please state denominators explicitly and ensure that like-for-like comparisons are made.

8. Conclusions regarding echocardiography should be moderated.
The present series demonstrates that echocardiography raised suspicion of a vascular ring in several patients, while CT angiography provided definitive anatomical confirmation. The conclusion that the study “confirmed” the importance of echocardiography is stronger than can be supported by a seven-patient retrospective case series without a diagnostic accuracy comparator. I suggest stating that, in this series, echocardiography was a useful non-invasive modality for raising suspicion and assessing associated cardiac abnormalities, whereas cross-sectional angiographic imaging was required to define vascular anatomy.

9. Terminology should be standardized.
Please carefully distinguish vascular rings from vascular slings and ensure that classifications of complete and incomplete rings are anatomically precise. The terminology surrounding RAA+ALSA should specify the structures completing the ring when relevant. Abbreviations should also be standardized throughout the text.

10. Tables require correction and editorial revision.
In addition to the numerical discrepancies noted above, several spelling and formatting problems should be corrected. Examples include “Reccurent”, “Dyspnoe”, “Apnoe”, inconsistent decimal separators, and percentages such as 28.7% for 2/7. Table 1 also requires correction of the mean age at diagnosis. Raw counts should remain prominent because percentages based on a denominator of seven can visually exaggerate precision.

11. Reference list.
References 13 and 17 appear to be duplicate citations of the same Fang et al. 2015 article with the same DOI and should be consolidated, followed by renumbering of subsequent references if necessary. The bibliographic information of all references should be checked for completeness and consistency.

12. Language and proofreading.
The manuscript would benefit from careful English-language editing. Examples include “outliner” instead of “outlier”, “subclavial” instead of “subclavian”, “magnetic resonance imagining” instead of “magnetic resonance imaging”, inconsistent singular/plural constructions, and several grammatical errors. Of particular importance, the Results state that the mean gestational age at birth was 37.2 “months”; this should be “weeks”.

Overall, the manuscript addresses a clinically interesting topic and the rare combined ring–sling case adds educational value. However, a careful revision of the methodology, ethics reporting, patient-level numerical consistency, terminology, tables, and interpretation is required before publication.

Author Response

Dear Reviewer,

Thank you very much for taking the time to review our manuscript and for your thorough and valuable comments. We have carefully considered all of them, and they have helped us to substantially revise the manuscript. Below, we respond to your comments point by point. Please see the attachment to read a new version of the manuscript, in which are all changes highlighted.

In addition to the changes described below, several other changes were made in response to the comments of the other reviewers. These include a revised Introduction with a clearly stated aim of the study, a restructured Results section and a new patient-level Supplementary Table 1.

1. Study design and patient selection: The Methods section should describe the cohort assembly more precisely. Please state whether the seven patients represent all consecutive eligible patients diagnosed or followed at the institution during 2021–2025, provide explicit inclusion and exclusion criteria, explain how cases were identified from the hospital information system, and clarify whether any potentially eligible cases were excluded. The duration and completeness of postoperative follow-up should also be reported. Given the very small sample size, the study should consistently be described as a retrospective descriptive case series rather than implying population-level estimates.

Thank you for this comment. We have revised Section 2.1 (Study Design and Patients) and Section 2.2 (Data Analysis). The Methods now state that all patients referred to our department for the differential diagnosis of respiratory symptoms of unclear etiology (and, in one case, with a prenatally raised suspicion of a vascular ring) in whom the diagnosis of a vascular ring was definitively confirmed between 2021 and 2025 were included, and that no potentially eligible patient was excluded. The exclusion criteria (diagnosis established before 2021 and unconfirmed suspicion of a vascular ring) are stated explicitly.

We have also explained how the patients were identified: the hospital information system NIS MEDEA was searched using the ICD-10 codes for individual types of vascular rings and slings (Q25.4, Q25.7, Q25.8 and Q27.8).

The duration of postoperative follow-up has been added to Section 3.6. All operated patients remain under regular follow-up, and individual follow-up data are now shown in Supplementary Table 1.

Finally, the study is now consistently described as a retrospective descriptive case series, including in the Abstract.

2. Statistical presentation and handling of "outliers": With only seven patients, the repeated exclusion of individual patients as “outliers” requires reconsideration. These older patients are clinically relevant observations rather than statistical anomalies and should not be removed solely because their ages differ substantially from those of the remaining patients. I recommend presenting the complete cohort using median, range and/or interquartile range as the primary descriptive measures, with any restricted pediatric/early-presentation analysis clearly identified as a sensitivity or descriptive subgroup analysis. The criteria used to define an outlier should otherwise be stated explicitly.

We agree with the reviewer. All analyses excluding individual patients have been removed from the manuscript. Continuous variables are now presented for the complete cohort as medians with interquartile ranges and ranges, and the statistical approach is described in Section 2.2. Where the ranges are wide, we only describe which patients account for this (patients diagnosed in adolescence), without excluding them. The Results, Discussion, Abstract and Table 1 have been revised accordingly.

3. Numerical inconsistencies must be reconciled throughout the manuscript, tables, abstract, and supplementary dataset: Several discrepancies are currently present. For example, the text reports a mean age at diagnosis of 56.75 months, whereas Table 1 reports 57.5 months. The supplementary patient-level table supports 56.75 months. In addition, the Results state that cyanosis occurred in 4 patients and vomiting in 4 patients, whereas Table 2 reports 5 patients for each variable. The supplementary dataset supports 4/7 for both cyanosis and vomiting. Please perform a complete audit of all counts, percentages, means, medians, and denominators against the source dataset.

Thank you for pointing out these discrepancies. We have checked all counts, percentages, medians, means and denominators against the patient-level dataset. Table 1 has been revised and now presents the median (IQR), range and mean for each parameter. In Table 2, cyanosis and vomiting are now correctly reported in 4 of 7 patients (57.1%). Incorrectly rounded percentages have been corrected throughout the manuscript, the tables and the Abstract.

4. CT angiography reporting is internally inconsistent: Section 3.4 states that definitive diagnosis was obtained by CT angiography in all seven patients (100%). However, the Discussion subsequently states that CT angiography was used in 6 patients (71.4%). Moreover, 6/7 would correspond to 85.7%, not 71.4%. The supplementary patient-level dataset indicates CT angiography in all seven patients. This should be corrected consistently throughout the manuscript.

Thank you, this was our error. The Discussion has been corrected and now states, in agreement with the Results and the supplementary dataset, that CT angiography was used for definitive confirmation in all patients (n = 7, 100%).

5. There is an important factual error concerning DiGeorge syndrome: The Discussion refers to “21q11.2 deletion syndrome (DiGeorge syndrome).” This should be corrected to 22q11.2 deletion syndrome. Given the relevance of genetic abnormalities to vascular rings, the authors should carefully verify the genetics terminology throughout the manuscript.

We apologize for this error. It has been corrected to 22q11.2 deletion syndrome. We have also checked the genetics terminology throughout the manuscript and clarified the description of the cited population-based study, which concerned fetuses with a prenatal diagnosis of RAA or DAA.

6. Ethics reporting requires substantial clarification: The manuscript currently provides only a generic statement that the procedures complied with ethical standards and that patient consent was waived because of the retrospective design. Please provide the full name of the approving Institutional Review Board/Ethics Committee, approval or waiver number, and approval date. The authors should also clarify whether the waiver specifically covered retrospective use of pediatric clinical and imaging data. Because Figure 1 contains patient-derived imaging and the cohort includes very rare phenotypes that may increase re-identification risk, the authors should clarify whether separate consent for publication was required or formally waived according to institutional policy.

Thank you for this important comment. The ethics information has been completed in Section 2.3 and in the Institutional Review Board and Informed Consent Statements. The study was approved by the Ethics Committee of the Jessenius Faculty of Medicine in Martin, Comenius University in Bratislava (approval no. EK 10/2026; date of approval 29 January 2026). We now state that the waiver of patient consent covered the retrospective use of pediatric clinical and imaging data, that all images were anonymized, and that the requirement for separate consent for publication was waived by the Ethics Committee in accordance with institutional policy.

7. Surgical complication denominators: Six of seven patients underwent surgery, whereas postoperative complications occurred in two patients. The manuscript reports this as 28.5%, apparently using the entire cohort as the denominator. When comparing postoperative complication rates with published surgical cohorts, the clinically appropriate denominator is the number of operated patients; therefore, this corresponds to 2/6 (33.3%). Please state denominators explicitly and ensure that like-for-like comparisons are made.

We agree. Postoperative complications are now reported as 2 of 6 operated patients (33.3%) in both the Results and the Discussion.

8. Conclusions regarding echocardiography should be moderated: The present series demonstrates that echocardiography raised suspicion of a vascular ring in several patients, while CT angiography provided definitive anatomical confirmation. The conclusion that the study “confirmed” the importance of echocardiography is stronger than can be supported by a seven-patient retrospective case series without a diagnostic accuracy comparator. I suggest stating that, in this series, echocardiography was a useful non-invasive modality for raising suspicion and assessing associated cardiac abnormalities, whereas cross-sectional angiographic imaging was required to define vascular anatomy.

The Conclusions now state that, in this series, echocardiography was a useful non-invasive modality for raising suspicion and assessing associated cardiac abnormalities, whereas cross-sectional angiographic imaging was required to define the vascular anatomy. The conclusion of the Abstract has been revised in the same way, and the Results of the Abstract now describe echocardiography as the modality that raised the initial suspicion rather than established the diagnosis. In the Discussion, we note that its diagnostic value would need formal validation.

9. Terminology: Please carefully distinguish vascular rings from vascular slings and ensure that classifications of complete and incomplete rings are anatomically precise. The terminology surrounding RAA+ALSA should specify the structures completing the ring when relevant. Abbreviations should also be standardized throughout the text.

Thank you. We have revised the terminology throughout the manuscript. The rare case is now consistently described as a combination of a vascular ring (RAA+ALSA) and a pulmonary artery sling, instead of “two vascular rings”, in the Abstract, Results, Figure 1 caption, Discussion and Conclusions. In the Introduction and Results, we now state that in RAA+ALSA the ring is completed by a left-sided ligamentum arteriosum, and we define the ring–sling complex as the combination of a pulmonary artery sling with complete cartilaginous tracheal rings. Abbreviations are now defined at first use and the list of abbreviations has been completed.

10. Tables: Several spelling and formatting problems should be corrected. Examples include “Reccurent”, “Dyspnoe”, “Apnoe”, inconsistent decimal separators, and percentages such as 28.7% for 2/7. Table 1 also requires correction of the mean age at diagnosis. Raw counts should remain prominent because percentages based on a denominator of seven can visually exaggerate precision.

Tables 1 and 2 have been revised. The spelling errors have been corrected, decimal points are used consistently, the percentages have been recalculated, and the terminology has been unified with the text.

11. Reference list: References 13 and 17 appear to be duplicate citations of the same Fang et al. 2015 article with the same DOI and should be consolidated, followed by renumbering of subsequent references if necessary. The bibliographic information of all references should be checked for completeness and consistency.

Thank you. The duplicate reference has been consolidated and the subsequent references have been renumbered. We have also reviewed the bibliographic information of the remaining references.

12. Language and proofreading: The manuscript would benefit from careful English-language editing. Examples include “outliner” instead of “outlier”, “subclavial” instead of “subclavian”, “magnetic resonance imagining” instead of “magnetic resonance imaging”, inconsistent singular/plural constructions, and several grammatical errors. Of particular importance, the Results state that the mean gestational age at birth was 37.2 “months”; this should be “weeks”.

Thank you for the careful reading of our manuscript. All of the listed errors have been corrected, including the unit of gestational age, which is now correctly given in weeks. We have also corrected other grammatical errors and unified the spelling throughout the text. We agree with the suggestion regarding English language editing and will arrange the proposed language correction of the manuscript.

Once again, we would like to thank you for your valuable comments and suggestions, which have helped us to improve our manuscript.

Yours sincerely,

On behalf of all authors

Author Response File: Author Response.docx

Reviewer 2 Report

Comments and Suggestions for Authors

This manuscript presents a retrospective analysis of seven patients with vascular rings. While the topic is clinically relevant, several major issues must be addressed before the manuscript can be considered for publication.

First, the introduction devotes substantial space to general background information, yet the study’s objective remains unclear. The structure should be revised so that the rationale and purpose of the study are explicitly stated. Additionally, anatomical diagrams are essential; without them, readers cannot visualize the vascular ring configurations described in the text.

Second, although vascular rings are rare, a case series of n=7 is extremely small. The authors must clarify what novel findings emerge from this cohort. One possibility is to reframe the manuscript so that the rare coexistence of RAA, ALSA, and PAS becomes the central message, supported by detailed clinical interpretation. As currently written, the results section largely lists symptoms and anatomical findings without extracting clinical insights or contributing new understanding to the field.

Overall, the scientific contribution of the manuscript is limited in its current form. A substantial restructuring—both conceptually and analytically—is required.

Author Response

Dear Reviewer,

In addition to the changes described below, several other changes were made in response to the comments of the other reviewers. These include a revised statistical presentation, more detailed ethics reporting, standardized terminology and a shortened Introduction.

1. Introduction and objective of the study: First, the introduction devotes substantial space to general background information, yet the study’s objective remains unclear. The structure should be revised so that the rationale and purpose of the study are explicitly stated.

Thank you for this important comment. We agree that the objective of the study was not stated clearly enough in the original version. The Introduction has therefore been substantially revised. We shortened the general background (history, embryology and repeated general descriptions of symptoms and diagnostics), while keeping the key information from the cited literature.

We added a new paragraph explaining the rationale of the study. Vascular rings are frequently overlooked because their symptoms mimic far more common conditions, which may lead to a diagnostic delay. Most published cohorts are surgical series, whereas data describing these patients from the perspective of a pediatric department, to which children usually present first with unexplained respiratory symptoms, are limited. In Slovakia, aortic arch anomalies have previously been characterized mainly from a morphological perspective, and the role of echocardiography as an initial examination in symptomatic children has not been clearly established.

The aim of the study is now stated explicitly at the end of the Introduction. Specifically, we aimed to characterize the timing and spectrum of respiratory and gastrointestinal symptoms, associated cardiac and genetic abnormalities, and the age at definitive diagnosis; to evaluate the contribution of individual imaging modalities, particularly echocardiography, to raising the initial suspicion of a vascular ring; and to describe surgical management, its timing and outcomes. The aim in the Abstract has been revised accordingly.

2. Anatomical diagrams: Additionally, anatomical diagrams are essential; without them, readers cannot visualize the vascular ring configurations described in the text.

Thank you for this suggestion. We fully agree that anatomical diagrams are always helpful for understanding vascular ring configurations. After careful consideration, however, we decided not to include them in this manuscript. Schematic diagrams of the individual types of vascular rings are well established and are available in the review articles and classification papers cited in our manuscript, so they would not add new information to our study (example Licari et al.). In addition, the manuscript is primarily focused on the clinical presentation and the diagnostic pathway rather than on the anatomy of individual ring types. Instead of schematic drawings, Figure 1 presents CT angiography images of the actual anatomy in our patient with the rare combination of a vascular ring and a pulmonary artery sling, and the anatomical diagnosis of each patient is given in Supplementary Table 1. We have also revised the terminology so that the anatomical configurations, including the structures completing the ring in RAA+ALSA, are described more precisely in the text.

3. Size of the cohort and novel findings: Second, although vascular rings are rare, a case series of n=7 is extremely small. The authors must clarify what novel findings emerge from this cohort. One possibility is to reframe the manuscript so that the rare coexistence of RAA, ALSA, and PAS becomes the central message, supported by detailed clinical interpretation. As currently written, the results section largely lists symptoms and anatomical findings without extracting clinical insights or contributing new understanding to the field.

We fully acknowledge that our cohort is small, and this is stated as a limitation of the study. We carefully considered the reviewer’s suggestion to reframe the manuscript around the rare coexistence of RAA+ALSA and a pulmonary artery sling. However, we did not want to focus the study on a single patient, as our intention was to describe the whole group of children referred to our department for unexplained respiratory symptoms, which reflects the situation that pediatricians face in everyday practice. The rare combined case remains an important part of the manuscript and is described in the Results, in Figure 1 and in the Discussion.

To clarify what our study adds, the revised manuscript now states the aims explicitly and focuses on the pediatric perspective, which differs from most published surgical series. To extract more clinical insights, we have made the following changes compared with the original version:

- The Results have been restructured and are presented in the order of diagnosis, management and outcome, including a new section on the diagnostic work-up and the sequence of examinations (Section 3.3) and a section on outcome and follow-up (Section 3.6).

-A patient-level table (Supplementary Table 1) has been added, presenting the clinical course of each patient, including the first examination that raised the suspicion of a vascular ring, the order of all examinations, the surgical procedure, complications, outcome and duration of follow-up.

- In the Discussion, our findings are interpreted in comparison with published cohorts. For example, the median age at symptom onset in our cohort (30 days) was earlier than reported in comparable studies; the initial suspicion was raised by echocardiography in most patients, whereas chest X-ray was not sufficient to raise suspicion; and none of our patients was asymptomatic at the time of diagnosis, which reflects the selection of children referred for respiratory symptoms.

-The interpretation has been moderated so that it does not go beyond what a small retrospective case series can support

4. Overall scientific contribution: Overall, the scientific contribution of the manuscript is limited in its current form. A substantial restructuring—both conceptually and analytically—is required.

We are grateful for this honest assessment. Based on the comments of all reviewers, the manuscript has been substantially restructured. Conceptually, the rationale and aims of the study are now clearly defined. Analytically, the data of the complete cohort are presented as medians with interquartile ranges and ranges, all numerical data have been checked against the patient-level dataset, and the Results follow the order of diagnosis, management and outcome. We hope that the revised manuscript is now clearer and that its contribution, although limited by the size of the cohort, is better defined.

Once again, we would like to thank you for your valuable comments and suggestions, which have helped us to improve our manuscript.

Yours sincerely,

On behalf of all authors

Reviewer 3 Report

Comments and Suggestions for Authors

The manuscript entitled Vascular rings as a cause of respiratory difficulties is a retrospective study. The authors presented a cohort of patients diagnosed with vascular rings and compared the management of these patients with other studies in the context of the continuously evolving understanding of this diagnosis.

The manuscript is relatively well-written. The introduction is too long comparing with the results.

This retrospective study presenting a congenital pathology is very small. The data regarding the presented cohort offer nothing new compared to current literature.

However, I think it would be interesting to detail the imaging methods used in these seven cases and the sequence in which they were applied to confirm the diagnosis.

It is essential to identify a diagnostic or screening algorithm for medical practice that goes beyond computed tomography.

Can a diagnostic or screening algorithm be developed using multimodal techniques, starting from the most specific symptoms?

To enhance the scientific quality of this manuscript, the results should be presented in the order of diagnosis, management, and prognosis.

Author Response

Dear Reviewer,

Thank you very much for taking the time to review our manuscript and for your helpful comments. Your comments have helped us to improve the manuscript considerably. Below, we respond to your comments point by point All changes in the revised manuscript are highlighted in yellow.

In addition to the changes described below, several other changes were made in response to the comments of the other reviewers. These include a revised statistical presentation, a check of all numerical data against the patient-level dataset, more detailed ethics reporting and standardized terminology.

1. Length of the Introduction: The manuscript is relatively well-written. The introduction is too long comparing with the results.

Thank you for your kind words and for this comment. We have tried to shorten the Introduction. We removed redundant general background, such as several sentences on the history and embryology of vascular rings and repeated general descriptions of symptoms and diagnostics, while keeping the key information from the cited literature. At the same time, we replaced part of this text with a more specific description of the rationale and aims of the study, in order to explain more clearly how our work differs from previously published studies.

2. Size of the cohort and novelty: This retrospective study presenting a congenital pathology is very small. The data regarding the presented cohort offer nothing new compared to current literature.

We fully acknowledge that our cohort is small, and this is stated as a limitation of the study. In the revised Introduction, we have tried to define more clearly what our study aims to add. Most published cohorts are surgical series, whereas our study describes children from the perspective of a pediatric department, to which these patients are usually first referred for unexplained respiratory symptoms. We focus on the clinical presentation, the diagnostic pathway and the role of individual imaging modalities in raising the initial suspicion. Our cohort also includes a rare combination of a vascular ring and a pulmonary artery sling in a single patient. We hope that, despite its size, the study may be of practical value to pediatricians in everyday clinical practice.

3. Imaging methods and their sequence: However, I think it would be interesting to detail the imaging methods used in these seven cases and the sequence in which they were applied to confirm the diagnosis.

Thank you for this valuable suggestion. We have added a patient-level Supplementary Table 1, which shows for each patient the first diagnostic examination that raised the suspicion of a vascular ring and all examinations in the order in which they were performed. This information is also summarized in the Results (Section 3.3, Diagnostic Work-Up): echocardiography was performed in all patients, chest X-ray in five patients and bronchofibroscopy in six patients, in most cases after CT angiography, and the diagnosis was definitively confirmed by CT angiography in all patients.

4. Diagnostic or screening algorithm: It is essential to identify a diagnostic or screening algorithm for medical practice that goes beyond computed tomography. Can a diagnostic or screening algorithm be developed using multimodal techniques, starting from the most specific symptoms?

Thank you for this important question. We agree that a practical diagnostic or screening algorithm based on multimodal techniques would be very valuable for clinical practice. However, we believe that developing or proposing a new diagnostic algorithm is beyond the scope of this study. With only seven patients in a retrospective descriptive case series, our data are not sufficient to develop or validate such an algorithm, and we did not want to draw conclusions that our results cannot support. In the Discussion, we compare our diagnostic approach with previously proposed algorithms and note that the diagnostic value of echocardiography would need to be formally validated. We believe that this question should be addressed in larger, ideally prospective and multicenter studies.

5. Order of the Results: To enhance the scientific quality of this manuscript, the results should be presented in the order of diagnosis, management, and prognosis.

Thank you for this suggestion, which we have followed. The Results section has been restructured and is now presented in the following order: demographics (Section 3.1), symptomatology (Section 3.2), diagnostic work-up (Section 3.3), vascular rings, slings and other cardiac findings (Section 3.4), management and timing of surgery (Section 3.5), and outcome and follow-up (Section 3.6), which now also includes the duration of postoperative follow-up.

Once again, we would like to thank you for your valuable comments and suggestions, which have helped us to improve our manuscript.

Yours sincerely,

On behalf of all authors

Reviewer 4 Report

Comments and Suggestions for Authors

The manuscript presents a retrospective single-center cohort of seven pediatric patients with vascular rings diagnosed between 2021 and 2025. The manuscript provides potentially useful clinical information about symptomatology, abnormalities, imaging modalities, and diagnosis. However, several issues need to be addressed. I would recommend the following corrections:

All numerical variables should be checked again. Units should be standardized. Check the terminology for gestational age, age at symptom onset, age at diagnosis, time between diagnosis and surgery, etc.
The manuscript would be strengthened by a table containing, for each patient sex; gestational age; age at symptom onset; presenting symptoms; age at suspected diagnosis; imaging modalities; anatomical diagnosis; associated cardiac abnormalities; genetic diagnosis; age at surgery; surgical procedure; postoperative complications; clinical outcome; duration of follow-up.
The Methods state that the authors analyzed patients with a confirmed vascular ring “or patients who were followed up for this diagnosis.” The authors should clearly state what were the inclusion criteria; were all patients with suspected vascular rings included; were patients included only after anatomical confirmation; were asymptomatic patients included; were patients referred from other hospitals?
The manuscript should avoid implying that this small retrospective cohort provides sufficient evidence to establish echocardiography as a screening or diagnostic strategy.
The surgical section provides description of procedures, but additional information would improve its clinical value.
The manuscript states that “100% of patients were symptomatic”, but later describes one patient who was asymptomatic for most of their life and developed symptoms at age 14 years.
The abstract contains several numerical inconsistencies and should be revised after the correction of the Results.
Decimal notation should be standardized.

Author Response

Dear Reviewer,

Thank you very much for taking the time to review our manuscript and for your detailed and constructive comments. Your comments have helped us to improve the manuscript considerably. Below, we respond to each comment point by point. All changes in the revised manuscript are highlighted in yellow.

In addition to the changes described below, several other changes were made in response to the comments of the other reviewers. These include a revised Introduction with a clearly stated aim of the study, a restructured Results section presented in the order of diagnosis, management and outcome, a revised statistical presentation, more detailed ethics reporting, and standardized terminology.

1. Numerical variables, units and terminology: All numerical variables should be checked again. Units should be standardized. Check the terminology for gestational age, age at symptom onset, age at diagnosis, time between diagnosis and surgery, etc.

Thank you for this comment. We have checked all numerical variables again against the patient-level dataset and corrected the inconsistencies we found. The units are now stated for each parameter: gestational age in weeks, birth weight in grams, age at symptom onset in days, age at definitive diagnosis and age at surgery in months, the diagnosis-to-surgery interval in days, and the duration of follow-up in months.

We have also harmonized the terminology in the text and tables, using the terms “gestational age at birth”, “age at symptom onset”, “age at definitive diagnosis” and “diagnosis-to-surgery interval”

2. Patient-level table: The manuscript would be strengthened by a table containing, for each patient sex; gestational age; age at symptom onset; presenting symptoms; age at suspected diagnosis; imaging modalities; anatomical diagnosis; associated cardiac abnormalities; genetic diagnosis; age at surgery; surgical procedure; postoperative complications; clinical outcome; duration of follow-up.

We have added a patient-level table (Supplementary Table 1), which is referred to at the beginning of the Results. For each patient, it presents sex, gestational age at birth, birth weight, age at symptom onset, presenting symptoms, the first diagnostic examination that raised the suspicion of a vascular ring, all examinations in the order in which they were performed, age at definitive diagnosis, anatomical diagnosis, associated cardiac abnormalities, genetic diagnosis, surgical procedure, age at surgery, diagnosis-to-surgery interval, postoperative complications, clinical outcome and duration of postoperative follow-up. Because of its size, we have included it as a supplementary table 1.

3. Inclusion criteria: The Methods state that the authors analyzed patients with a confirmed vascular ring “or patients who were followed up for this diagnosis.” The authors should clearly state what were the inclusion criteria; were all patients with suspected vascular rings included; were patients included only after anatomical confirmation; were asymptomatic patients included; were patients referred from other hospitals?

Thank you for pointing out this unclear wording. The phrase has been removed, and Section 2.1 has been rewritten to state the inclusion and exclusion criteria explicitly. In answer to the individual questions:

Patients with a suspected vascular ring were not included unless the diagnosis was confirmed, patients in whom the diagnosis of a vascular ring was not confirmed were excluded.

Patients were included only after anatomical confirmation of the diagnosis, which was obtained by CT angiography in all patients (n = 7).

Asymptomatic patients were not included. All patients were referred for the differential diagnosis of respiratory symptoms of unclear etiology (in one case with a prenatally raised suspicion of a vascular ring), and all of them were symptomatic at the time of definitive diagnosis.

All patients were referred to our department from regional hospitals. Patients diagnosed before 2021 were not included, and no potentially eligible patient was excluded. We also describe how the patients were identified in the hospital information system using ICD-10 codes (Section 2.2).

4. Echocardiography: The manuscript should avoid implying that this small retrospective cohort provides sufficient evidence to establish echocardiography as a screening or diagnostic strategy.

We agree with the reviewer. We have revised the Abstract, Discussion and Conclusions accordingly. Echocardiography is now described only as a useful non-invasive modality that raised the initial suspicion of a vascular ring in our series, whereas CT angiography was required to define the vascular anatomy. In the Discussion, we state that its diagnostic value would need to be formally validated before it could be incorporated into clinical guidelines, and the Introduction notes that the role of echocardiography as an initial screening tool has not been clearly established.

5. Surgical section: The surgical section provides description of procedures, but additional information would improve its clinical value.

Thank you for this suggestion. Supplementary Table 1 now lists the surgical procedure for each patient, together with the age at surgery, the diagnosis-to-surgery interval, postoperative complications, the clinical outcome and the duration of follow-up.

6. Symptomatic and asymptomatic patients: The manuscript states that “100% of patients were symptomatic”, but later describes one patient who was asymptomatic for most of their life and developed symptoms at age 14 years.

Thank you for noticing this inconsistency. We have clarified that all patients were symptomatic at the time of definitive diagnosis. The patient in question (the only case of ARSA) had been asymptomatic for most of their life and had only been followed up for a suspected, unspecified anomaly of the aortic arch; the first symptoms appeared at the age of 14 years, before the definitive diagnosis. This is now explained in the Results (Section 3.2) and the Discussion, and a note has been added below Table 2.

7. Numerical inconsistencies in the Abstract: The abstract contains several numerical inconsistencies and should be revised after the correction of the Results.

Thank you. After the correction of the Results, the Abstract has been revised. Ages are now reported as medians with interquartile ranges, the percentages have been recalculated, the number of patients is given before the percentage, and all values are consistent with the Results and the supplementary dataset.

8. Decimal notation: Decimal notation should be standardized.

Decimal points are now used consistently throughout the manuscript and tables instead of decimal commas, and percentages are reported to one decimal place.

Once again, we would like to thank you for your valuable comments and suggestions, which have helped us to improve our manuscript.