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Variations in the Evaluation and Management of Vascular Rings: A Survey of American Clinicians

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Abstract

Vascular rings are arterial malformations that lead to the compression of the trachea and/or esophagus. While “tight” rings often produce symptoms and require surgery, “loose” rings rarely produce symptoms. Given advances in fetal echocardiography, this diagnosis is now more often made prenatally. This poses a new conundrum in the management of asymptomatic patients, leading to practice variation and creating a target for clinical system improvement. Hence, we conducted this survey aiming to demonstrate the practice variation existing in current evaluation and management of these patients. An anonymous web-based survey was distributed to several listservs for pediatric cardiologists and pediatric cardiothoracic surgeons. Survey questions targeted respondent practice characteristics, testing obtained, and indications for testing or surgical referral. In total 61 responses were received, predominantly from pediatric cardiologists (95%) in the United States (97%). About 60% of clinicians reported frequently diagnosing patients with vascular rings by fetal echocardiogram, with only about 20% diagnosing them frequently on evaluation of symptoms. Computed tomography angiography and echocardiogram were the most common imaging modalities employed. Most clinicians obtained cross-sectional imaging at the time of diagnosis and referred to surgery once patients had at least occasional symptoms. Respondents demonstrated a low degree of agreement (Krippendorf’s alpha 0.48). Few statistically significant patterns were identified between respondents based on their practice characteristics. This study identified significant variation between clinicians regarding the evaluation and management of vascular rings. Further research or expert opinions may help to standardize practice, saving costs and improving the quality of care for affected patients.

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All authors contributed to the study conception and design. Material preparation, data collection and analysis were performed by DP. The first draft of the manuscript was written by DP, and all authors commented on previous versions of the manuscript. All authors read and approved the final manuscript.

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Correspondence to Daniel M. Pasternack.

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The authors have no competing interests to declare that are relevant to the content of this article. The authors did not receive support from any organization for the submitted work.

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This survey involving human participants was in accordance with the ethical standards of the 1964 Helsinki Declaration and its later amendments or comparable ethical standards. The Institutional Review Board of the New York University Grossman School of Medicine approved this study.

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Pasternack, D.M., Ludomirsky, A., Tan, R.B. et al. Variations in the Evaluation and Management of Vascular Rings: A Survey of American Clinicians. Pediatr Cardiol 45, 959–966 (2024). https://doi.org/10.1007/s00246-024-03442-8

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