Long term outcomes of vascular ring correction: single centre experience

Vascular rings are a rare congenital anomaly of the aortic arch that can lead to significant clinical symptoms due to compression of the airways and esophagus, although they can also be asymptomatic. This condition can be challenging to address, due to its variability in symptoms and rarity; however, focusing on the appropriate management strategies is crucial, as patients treated within the optimal time frame tend to have favorable outcomes, whereas delays in treatment can lead to sequelae. The primary objective is to compare the clinical course, perioperative outcomes (<30 days), and long-term outcomes (>30 days) between patients with a prenatal diagnosis and those with a postnatal diagnosis, to identify the benefits of early diagnosis and timely surgical intervention on patient outcomes. A retrospective cohort study was conducted on 109 pediatric patients (aged <18 years) who had diagnosis of vascular ring at “Ospedale del Cuore G. Pasquinucci” in Massa, Italy, from January 2005 to July 2024. Of these, 73 patients who underwent corrective surgery exclusively for vascular ring were considered. Patient records, including baseline characteristics, clinical presentation, diagnostic test reports, operative notes, and follow-up data, were thoroughly reviewed. Patients were classified based on their symptomatic status and timing of diagnosis (prenatal vs. postnatal). Perioperative and long-term outcomes were analyzed, comparing prenatal and postnatal diagnosis groups and examining trends across the first and second decades of the patient cohort. The statistical analysis of the long-term follow-up was performed only on patients who continued their follow-up at the center. Surgical correction of vascular rings is perioperatively safe, as no deaths have been reported, and is effective in relieving symptoms in symptomatic patients. Prenatal diagnosis is essential for determining the optimal timing of surgery, allowing the procedure to be performed when the patient is most capable of tolerating it, thus preventing delays that could negatively impact long-term outcomes. Early detection enables ongoing patient monitoring and timely surgical intervention, which enhances the management of associated conditions, alleviates airway compression, and significantly improves overall clinical outcomes.

Publication Details

Journal
Electronic Theses and Dissertations Repository (University of Pisa)
Published
2024-09-19
Primary Topic
Tracheal and airway disorders
Type
article
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article

Long term outcomes of vascular ring correction: single centre experience

Electronic Theses and Dissertations Repository (University of Pisa)
Tracheal and airway disorders
article

Long term outcomes of vascular ring correction: single centre experience

article en

Abstract

Vascular rings are a rare congenital anomaly of the aortic arch that can lead to significant clinical symptoms due to compression of the airways and esophagus, although they can also be asymptomatic. This condition can be challenging to address, due to its variability in symptoms and rarity; however, focusing on the appropriate management strategies is crucial, as patients treated within the optimal time frame tend to have favorable outcomes, whereas delays in treatment can lead to sequelae. The primary objective is to compare the clinical course, perioperative outcomes (<30 days), and long-term outcomes (>30 days) between patients with a prenatal diagnosis and those with a postnatal diagnosis, to identify the benefits of early diagnosis and timely surgical intervention on patient outcomes. A retrospective cohort study was conducted on 109 pediatric patients (aged <18 years) who had diagnosis of vascular ring at “Ospedale del Cuore G. Pasquinucci” in Massa, Italy, from January 2005 to July 2024. Of these, 73 patients who underwent corrective surgery exclusively for vascular ring were considered. Patient records, including baseline characteristics, clinical presentation, diagnostic test reports, operative notes, and follow-up data, were thoroughly reviewed. Patients were classified based on their symptomatic status and timing of diagnosis (prenatal vs. postnatal). Perioperative and long-term outcomes were analyzed, comparing prenatal and postnatal diagnosis groups and examining trends across the first and second decades of the patient cohort. The statistical analysis of the long-term follow-up was performed only on patients who continued their follow-up at the center. Surgical correction of vascular rings is perioperatively safe, as no deaths have been reported, and is effective in relieving symptoms in symptomatic patients. Prenatal diagnosis is essential for determining the optimal timing of surgery, allowing the procedure to be performed when the patient is most capable of tolerating it, thus preventing delays that could negatively impact long-term outcomes. Early detection enables ongoing patient monitoring and timely surgical intervention, which enhances the management of associated conditions, alleviates airway compression, and significantly improves overall clinical outcomes.

Electronic Theses and Dissertations Repository (University of Pisa)
Good health and well-being
Openalex Percentile: Top 42%
Tracheal and airway disorders
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