Papillary Fibroelastoma of the Aortic Root Causing Intermittent Coronary Ostial Obstruction: The Diagnostic Power of 3D Transesophageal Echocardiography

Round 1

Reviewer 1 Report

Comments and Suggestions for Authors

The manuscript presents a rare and educational case of an aortic root papillary fibroelastoma causing intermittent right coronary ostial obstruction. The images are of high quality, and the added value of three-dimensional transesophageal echocardiography (3D TEE) for anatomical definition and surgical planning is clearly demonstrated. The case is appropriate for the Interesting Images section.

However, a minor revision is recommended to improve clarity and completeness:

1. Clinical decision-making
   Please briefly clarify the clinical rationale for performing coronary CT angiography in this patient (e.g., exclusion of coronary artery disease vs. preoperative assessment).

2. Pathophysiological explanation
   The manuscript would benefit from a short clarification on the proposed mechanism of intermittent right coronary ostial obstruction, particularly the dynamic relationship between tumor mobility and the coronary ostium.

3. Surgical indication
   Please add a brief justification for the decision to proceed with surgical excision in this patient, supported by guideline recommendations or relevant literature.

4. Language and minor corrections
   A careful language revision is advised to correct minor grammatical and typographical issues (e.g., spelling errors in keywords and minor phrasing inconsistencies: “image sees by the surgeon” → “image seen by the surgeon”  , “echocardiograpy” → “echocardiography” , “Non of the data” → “None of the data”).

Author Response

We are deeply grateful for your very detailed and useful comments. We have thoroughly revised the paper to take into account every observation and suggestion of the reviewers.

Below is a point-by-point response detailing the changes made to the revised manuscript.

Clinical decision-making

Comment 1: Please briefly clarify the clinical rationale for performing coronary CT angiography in this patient (e.g., exclusion of coronary artery disease vs. preoperative assessment).

Response: We thank the reviewer for this comment. The clinical rationale for coronary CT angiography has been clarified in the revised manuscript. We now explicitly state that CT angiography was performed to exclude occult coronary artery disease as an alternative cause of chest discomfort and as part of the preoperative assessment. This clarification has been added to the legend of Figure 3, lines 53-55.

 

Pathophysiological explanation

Comment 2: The manuscript would benefit from a short clarification on the proposed mechanism of intermittent right coronary ostial obstruction, particularly the dynamic relationship between tumor mobility and the coronary ostium.

Response: We agree with the reviewer and have expanded the pathophysiological explanation. The revised manuscript now explicitly describes the dynamic interaction between the highly mobile papillary fibroelastoma and the right coronary artery ostium, emphasizing intermittent and non-complete obstruction as the most likely mechanism underlying the patient’s transient symptoms. This explanation has been incorporated into the legend of Figure 4, lines 74-89.

 

Surgical indication

Comment 3: Please add a brief justification for the decision to proceed with surgical excision in this patient, supported by guideline recommendations or relevant literature.

Response: This point has been addressed. A clear justification for surgical excision has been added, highlighting the patient’s symptoms, the marked mobility of the lesion, its proximity to the right coronary artery ostium, and the associated risk of dynamic obstruction and embolization. This information is now included in the Figure 3 legend, lines 48-53.

 

Language and minor corrections

Comment 4: A careful language revision is advised to correct minor grammatical and typographical issues (e.g., spelling errors in keywords and minor phrasing inconsistencies: “image sees by the surgeon” → “image seen by the surgeon”  , “echocardiograpy” → “echocardiography” , “Non of the data” → “None of the data”).

Response: The manuscript has undergone careful language revision. All noted grammatical and typographical errors have been corrected, including spelling inconsistencies in keywords and minor phrasing issues throughout the text.

Reviewer 2 Report

Comments and Suggestions for Authors

Dear Authors and Editors!

This is a very interesting case report of a rare finding. I suggest only to mention a word on the CMR utility in such tumors.

Author Response

We are deeply grateful for your very detailed and useful comments. We have thoroughly revised the paper to take into account every observation and suggestion of the reviewers.

Below is a point-by-point response detailing the changes made to the revised manuscript.

Comment: I suggest only to mention a word on the CMR utility in such tumors.

Response: We thank the reviewer for this suggestion. A statement regarding the potential role of cardiac magnetic resonance for tissue characterization has been added. We also clarify that, in this case, three-dimensional transesophageal echocardiography provided sufficient anatomical information for mass characterization, making additional imaging unnecessary. This clarification has been included in the Figure 3 legend, lines 55-57.

Reviewer 3 Report

Comments and Suggestions for Authors

Dear Authors, I have analyzed the document "The Key Role of 3D Transesophageal Echocardiography in the Diagnosis and Treatment of Patient with Recurrent Chest Discomfort Caused by a Mobile Aortic Root Tumor" and identified several critical points that could improve to enhance the clarity and scientific rigor of the presentation.

1. Title and Initial Clarity

• Critique: The title is very long and could be more concise. Furthermore, the current phrasing "a mobile Aortic Root Tumor" is generic.
• Suggestion: Since the definitive diagnosis is Papillary Fibroelastoma, and the key feature was intermittent obstruction, I would suggest a title that reflects the specificity of the diagnosis and the mechanism. For example:
• Example of Improved Title: "Papillary Fibroelastoma of the Aortic Root Causing Intermittent Coronary Ostial Obstruction: The Diagnostic Power of 3D Transesophageal Echocardiography."

2. Clinical Details and Symptoms

• Critique: The description of chest pain is very brief: "mild pain localized along the left sternal border, that was not associated with physical activity and lasted less than one minute"¹. This type of pain is highly atypical for coronary ischemia.
• Suggestion: The authors should strengthen the connection between the atypical clinical presentation and the proposed pathophysiological mechanism (intermittent obstruction of the right coronary ostium).
• Add a brief discussion on why a mobile PFE might cause such mild and transient symptoms, perhaps resulting from a dynamic and non-complete obstruction.
• It is crucial to specify whether the atypical symptomatology was, in fact, the main reason for requesting echocardiography, following the exclusion of other causes.

3. Imaging Evidence and the Role of 3DTEE

• Critique: The most important part of the manuscript is the role of 3DTEE, but the findings are described separately across the imaging methods (2DTTE/3DTTE vs. 2DTEE/3DTEE).
• Suggestion:
• Organization of Results: Combine the description of all echocardiographic findings into one clear, sequential section.
• Emphasis on the Cusp: Highlight with greater emphasis the anatomical re-referencing provided by 3DTEE. While 2DTEE located the mass in the projection of the right coronary sinus, 3DTEE clearly showed the attachment site of the pedicle to the aortic wall between the bases of the left and right coronary cusps of the aortic valve, which is crucial.
• Visualization: The figures are good, but a schematic diagram of the aorta and the right coronary artery ostium with the PFE in two positions (obstructing and non-obstructing) would greatly help visualize the pathogenic mechanism .

4. Discussion and Literature

• Critique: The discussion is very short (less than 30 lines) and does not sufficiently expand on the context. A thorough comparison with the limitations of other techniques is missing^666666666.
• Suggestion:
• Technological Comparison: Expand the discussion on the limitations of 2DTTE and 2DTEE and the unique advantages of 3DTEE (anatomical reconstruction, visualization of the pedicle, relationship to the coronary ostium). Although mentioned, this point deserves more detail.
• Mechanism Reference: Delve deeper into the literature references concerning intermittent coronary obstruction by PFE. If the atypical symptom was caused by a dynamic mechanism, citing works that support this hypothesis is essential.

5. Terminological Details and Abbreviations

• Critique: The abbreviation for the tumor is "Tm" in the figure captions, but "PFE" is the specific term for the pathology.
• Suggestion: Replace the generic use of "Tm" with the confirmed histological diagnosis "PFE" (or Papillary Fibroelastoma) in all captions for Figures 1, 2, and 3, where the diagnosis is clear from the context.

6. Relevance of the 3DTEE Role (Conclusion)

• Critique: The conclusion states that 3DTEE helped plan the optimal surgical approach "without the need for more complex and expensive imaging modalities". However, Figure 3 mentions that Computed Tomography Coronary Angiography was performed (although to rule out atherosclerotic lesions).
• Suggestion: Modify the statement to reflect that 3DTEE allowed for surgical planning without further complex investigations for mass characterization (e.g., Cardiac Magnetic Resonance), but specify that CTCA was nonetheless used in the diagnostic phase to rule out atherosclerosis as an alternative cause of chest pain.

Reduce the level of duplicates, the rate is 31% and it is very high

In summary, the article presents a compelling clinical case on the superior role of 3DTEE, but would benefit from a more in-depth discussion on the pathogenic mechanism and greater integration and clarity in the presentation of the imaging data.

Author Response

We are deeply grateful for your very detailed and useful comments. We have thoroughly revised the paper to take into account every observation and suggestion of the reviewers.

Below is a point-by-point response detailing the changes made to the revised manuscript.

Title and initial clarity

Comment 1:

• Critique:The title is very long and could be more concise. Furthermore, the current phrasing "a mobile Aortic Root Tumor" is generic.
• Suggestion:Since the definitive diagnosis is Papillary Fibroelastoma, and the key feature was intermittent obstruction, I would suggest a title that reflects the specificity of the diagnosis and the mechanism. For example:

• Example of Improved Title:"Papillary Fibroelastoma of the Aortic Root Causing Intermittent Coronary Ostial Obstruction: The Diagnostic Power of 3D Transesophageal Echocardiography."

 Response: We thank the reviewer for this helpful suggestion. In line with this comment, the title has been revised to improve clarity and specificity. The updated title now explicitly reflects the definitive diagnosis of papillary fibroelastoma as well as the underlying mechanism of intermittent coronary ostial obstruction, while emphasizing the diagnostic role of three-dimensional transesophageal echocardiography. We believe that the revised title more accurately conveys the key clinical and imaging message of the manuscript. Lines 2-4.

 

Clinical details and symptoms

Comment 2:

 

• Critique:The description of chest pain is very brief: "mild pain localized along the left sternal  border, that was not associated with physical activity and lasted less than one minute"¹. This type of pain is highly atypical for coronary ischemia.
• Suggestion:The authors should strengthen the connection between the atypical clinical presentation and the proposed pathophysiological mechanism (intermittent obstruction of the right coronary ostium).

• Add a brief discussion on why a mobile PFE might cause such mild and transient symptoms, perhaps resulting from a dynamic and non-complete obstruction.
• It is crucial to specify whether the atypical symptomatology was, in fact, the main reason for requesting echocardiography, following the exclusion of other causes.

Response: We thank the reviewer for this insightful comment. We agree that the described chest discomfort is atypical for classical coronary ischemia and therefore required clearer pathophysiological interpretation. In the revised manuscript, we have strengthened the link between the mild, short-lasting symptoms and the proposed mechanism of intermittent, non-complete obstruction of the right coronary ostium by a highly mobile papillary fibroelastoma. We now explicitly state that the dynamic nature of the lesion likely resulted in transient contact with the coronary ostium, producing brief and self-limited symptoms rather than sustained ischemia. In addition, we clarify that the atypical symptomatology, after exclusion of other potential causes, was indeed the primary reason for performing echocardiographic evaluation. These clarifications have been incorporated into the Figure 4 legends, lines 74-89.

Imaging evidence and role of 3DTEE

Comment 3:

• Critique:The most important part of the manuscript is the role of 3DTEE, but the findings are described separately across the imaging methods (2DTTE/3DTTE vs. 2DTEE/3DTEE).
• Suggestion:

• Organization of Results:Combine the description of all echocardiographic findings into one clear, sequential section.
• Emphasis on the Cusp:Highlight with greater emphasis the anatomical re-referencing provided by 3DTEE. While 2DTEE located the mass in the projection of the right coronary sinus, 3DTEE clearly showed the attachment site of the pedicle to the aortic wall between the bases of the left and right coronary cusps of the aortic valve, which is crucial.
• Visualization:The figures are good, but a schematic diagram of the aorta and the right coronary artery ostium with the PFE in two positions (obstructing and non-obstructing) would greatly help visualize the pathogenic mechanism .

Response: We thank the reviewer for this important and constructive comment. We agree that the central message of the manuscript is the added value of three-dimensional transesophageal echocardiography and that this should be conveyed in a clear and coherent manner. In the revised version, the echocardiographic findings have been reorganized into a more sequential and integrated description across the figures, moving from initial two-dimensional assessment to definitive three-dimensional characterization. Greater emphasis is now placed on the anatomical re-referencing achieved with 3DTEE. While two-dimensional transesophageal echocardiography localized the mass to the projection of the right coronary sinus, three-dimensional transesophageal echocardiography clearly identified the precise attachment of the pedicle to the aortic wall between the bases of the left and right coronary cusps, which was critical for understanding the mechanism of intermittent coronary ostial obstruction and for surgical planning.

In response to the reviewer’s suggestion, we have added a schematic illustration (Figure 4) depicting the dynamic behavior of the papillary fibroelastoma in obstructing and non-obstructing positions relative to the right coronary artery ostium, in order to further clarify the proposed pathophysiological mechanism.

Discussion and literature

Comment 4:

• Critique:The discussion is very short (less than 30 lines) and does not sufficiently expand on the context. A thorough comparison with the limitations of other techniques is missing^666666666.
• Suggestion:

• Technological Comparison:Expand the discussion on the limitations of 2DTTE and 2DTEE and the unique advantages of 3DTEE (anatomical reconstruction, visualization of the pedicle, relationship to the coronary ostium). Although mentioned, this point deserves more detail.
• Mechanism Reference:Delve deeper into the literature references concerning intermittent coronary obstruction by PFE. If the atypical symptom was caused by a dynamic mechanism, citing works that support this hypothesis is essential.

Response: Given the Interesting Images format, we avoided a separate extended discussion section. Instead, we integrated the key comparative and pathophysiological insights directly into the figure legends, particularly Figure 3 and 4, in accordance with journal style and format requirements.

Terminology and abbreviations

Comment 5:

 

• Critique:The abbreviation for the tumor is "Tm" in the figure captions, but "PFE" is the specific term for the pathology.
• Suggestion:Replace the generic use of "Tm" with the confirmed histological diagnosis "PFE" (or Papillary Fibroelastoma) in all captions for Figures 1, 2, and 3, where the diagnosis is clear from the context.

 Response: We thank the reviewer for this comment. The generic abbreviation “Tm” has been removed from the figure captions and replaced with the specific histologically confirmed diagnosis, papillary fibroelastoma (PFE), in the legends of Figures 1, 2, and 3, where the diagnosis is clear from the context.

 

Relevance of 3DTEE and imaging strategy

Comment 6:

 

• Critique:The conclusion states that 3DTEE helped plan the optimal surgical approach "without the need for more complex and expensive imaging modalities". However, Figure 3 mentions that Computed Tomography Coronary Angiography was performed (although to rule out atherosclerotic lesions).
• Suggestion:Modify the statement to reflect that 3DTEE allowed for surgical planning without further complex investigations for mass characterization (e.g., Cardiac Magnetic Resonance), but specify that CTCA was nonetheless used in the diagnostic phase to rule out atherosclerosis as an alternative cause of chest pain.

 Response: We thank the reviewer for this important clarification. We agree that the original wording could be interpreted as ambiguous. In the revised manuscript, we have modified the statement to specify that three-dimensional transesophageal echocardiography provided sufficient information for mass characterization and surgical planning, without the need for additional complex imaging modalities such as cardiac magnetic resonance. At the same time, we explicitly clarify that coronary computed tomography angiography was performed during the diagnostic work-up to exclude atherosclerotic coronary artery disease as an alternative cause of chest pain. This distinction has been incorporated into the revised Figure 3 legend, lines 53-55.

Similarity / duplication

Comment 7:
Reduce the level of duplicates, the rate is 31% and it is very high

Response: We thank the reviewer for drawing our attention to the similarity report. To address this concern, we carefully revised the manuscript with a focus on reducing duplicated and generic formulations. In particular, the Abstract and the concluding parts of the figure legends were rephrased to replace standard, literature-derived expressions with case-specific descriptions emphasizing the unique clinical presentation, imaging findings, and pathophysiological mechanism observed in this patient. These changes were made without altering the scientific content, and we believe they have substantially reduced the overall similarity level of the manuscript.

 

Reviewer 4 Report

Comments and Suggestions for Authors

Comments to the Authors (Minor revisions)

1. Language and minor typos (editorial polish):
• Abstract: “almost identical to the image sees by the surgeon” → “almost identical to the image seen by the surgeon.”

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• Page 4: “in the real time” → “in real time.”

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• Page 4: “Non of the data…” → “None of the data…”

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• Abbreviations: “2DTEE Two. dimensional” → “Two-dimensional” (remove punctuation typo).

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• Keywords: “echocardiograpy” → “echocardiography.”

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1. Abbreviations consistency (important for clarity):
   In the abbreviations list, “RCC” is reported as “Right coronary artery,” but in standard aortic-valve terminology RCC usually indicates right coronary cusp (while RCA is right coronary artery and is already used in Figure 2). Please revise abbreviations to avoid ambiguity (e.g., RCC = right coronary cusp; RCA = right coronary artery).

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1. Ethics / consent statement (wording adjustment):
   Since this is a human clinical case, consider replacing “Not applicable” with a brief case-report statement consistent with journal policy (e.g., written informed consent obtained; patient identity protected). Current wording may be interpreted as inconsistent for a single-patient report.

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1. Figure legends (small additions to strengthen reproducibility):
• Figure 2: you provide mass dimensions and pedicle length; please specify how these were measured (3DTEE dataset measurement? intraoperative measurement?) and ensure units are explicit (mm).

diagnostics-4059488-peer-review…

• Consider adding one short sentence clarifying the key anatomical point: attachment between the bases of the left and right coronary cusps/aortic wall adjacent to the RCA ostium, to mirror the text and guide non-expert readers.

diagnostics-4059488-peer-review…

1. References formatting (minor cleanup):
   Please correct small formatting/spacing issues (e.g., “comprehensiveanalysis” spacing) to match journal style.

In the Discussion (where you emphasize the value of non-invasive cardiovascular imaging/functional assessment), you may consider adding one reference on aortic stiffness assessment by echography-based/vascular methodology in systemic disease, as a broader example of complementary non-invasive cardiovascular evaluation. For instance: Colaci M et al. Aortic pulse wave velocity measurement in systemic sclerosis patients. Reumatismo. 2012;64(6):360–367.

Author Response

We are deeply grateful for your very detailed and useful comments. We have thoroughly revised the paper to take into account every observation and suggestion of the reviewers.

Below is a point-by-point response detailing the changes made to the revised manuscript.

Language and typographical corrections

Comment 1: Language and minor typos (editorial polish):

• Abstract: “almost identical to the image seesby the surgeon” → “almost identical to the image seen by the surgeon.”

diagnostics-4059488-peer-review…

• Page 4: “in the real time” →“in real time.”

diagnostics-4059488-peer-review…

• Page 4: “Nonof the data…” → “None of the data…”

diagnostics-4059488-peer-review…

• Abbreviations: “2DTEE Two. dimensional” →“Two-dimensional” (remove punctuation typo).

diagnostics-4059488-peer-review…

• Keywords: “echocardiograpy” →“”

diagnostics-4059488-peer-review…

Response: All listed language and typographical issues have been corrected.

Comment 2: Abbreviations consistency (important for clarity) - In the abbreviations list, “RCC” is reported as “Right coronary artery,” but in standard aortic-valve terminology RCC usually indicates right coronary cusp (while RCA is right coronary artery and is already used in Figure 2). Please revise abbreviations to avoid ambiguity (e.g., RCC = right coronary cusp; lines 53-55.).

Response: We thank the reviewer for pointing out this important issue. In the revised manuscript, the abbreviations were carefully revised to eliminate any ambiguity. The abbreviation “RCC” is now consistently used to denote the right coronary cusp, and ‘’RCA’’ to denote right coronary artery, in accordance with standard terminology.

Comment 3: Ethics / consent statement (wording adjustment) - Since this is a human clinical case, consider replacing “Not applicable” with a brief case-report statement consistent with journal policy (e.g., written informed consent obtained; patient identity protected). Current wording may be interpreted as inconsistent for a single-patient report.

Response: Thank you for this important warning. The institutional ethics committee has approved the presentation of the patient, which we have stated in the appropriate section, lines 98-103.

Comments 4: Figure legends (small additions to strengthen reproducibility):

• Figure 2: you provide mass dimensions and pedicle length; please specify how these were measured (3DTEE dataset measurement? intraoperative measurement?) and ensure units are explicit (mm).
• Consider adding one short sentence clarifying the key anatomical point: attachment between the bases of the left and right coronary cusps/aortic wall adjacent to the RCA ostium, to mirror the text and guide non-expert readers.

Response: We thank the reviewer for these helpful suggestions. In the revised Figure 2 legend, we have specified that tumor dimensions and pedicle length were obtained from the three-dimensional transesophageal echocardiography dataset, with units explicitly stated in millimeters. In addition, we added a short sentence highlighting the key anatomical feature, namely the attachment of the pedicle to the aortic wall between the bases of the left and right coronary cusps, adjacent to the coronary ostium, in order to improve clarity and reproducibility, particularly for non-expert readers. Also, in the Figure 4 legend we present a more comprehensive interpretation of the pathophysiological mechanism of the patient's symptoms, lines 74-89.

Comment 5: References formatting (minor cleanup) -  Please correct small formatting/spacing issues (e.g., “comprehensiveanalysis” spacing) to match journal style.

Response: We thank the reviewer for noting these minor issues. All identified formatting and spacing errors in the reference list have been corrected, and the references have been revised to fully comply with the journal’s formatting style.

Comment 6: In the Discussion (where you emphasize the value of non-invasive cardiovascular imaging/functional assessment), you may consider adding one reference on aortic stiffness assessment by echography-based/vascular methodology in systemic disease, as a broader example of complementary non-invasive cardiovascular evaluation. For instance: Colaci M et al. Aortic pulse wave velocity measurement in systemic sclerosis patients. Reumatismo. 2012;64(6):360–367.

Response: We thank the reviewer for this suggestion. Due to requests from other reviewers, we have significantly reduced and modified the content of the discussion. Therefore, unfortunately, we do not find it possible to include the suggested reference.

 

Round 2

Reviewer 3 Report

Comments and Suggestions for Authors

While several improvements have been made, the authors claim to have reduced the plagiarism level; however, it has actually increased to 35%. Please ensure the plagiarism rate is reduced before the manuscript can proceed to publication.