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Published on: October 6, 2022
Pediatric airway compression in aortic arch malformations: a multidisciplinary approach
Francesca Petreschi1, Antonella Coretti1, Federica Porcaro1
1Pediatric Pulmonology and Cystic Fibrosis Unit, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.
Insights
Aortic arch malformations (AAMs) in children often present with respiratory issues. Management strategies vary, with surgery indicated for severe cases, while conservative approaches suit milder conditions.
Area of Science:
- Pediatric Cardiology
- Thoracic Surgery
- Medical Diagnostics
Background:
- Aortic arch malformations (AAMs) require clinical suspicion in pediatric patients with persistent respiratory symptoms or feeding difficulties.
- Early identification is crucial for timely intervention and improved outcomes.
Purpose of the Study:
- To describe a cohort of patients diagnosed with AAMs.
- To evaluate the effectiveness of a local management protocol for AAMs.
- To analyze the different types of AAMs and their associated symptoms.
Main Methods:
- Retrospective review of 59 patients diagnosed with AAMs.
- Classification into three groups: double aortic arch (DAA), complete vascular ring (non-DAA), and anomalous origin of the innominate artery (IA).
- Analysis of clinical presentation, diagnostic methods, and treatment outcomes.
Main Results:
- Prenatal diagnosis was achieved in 62.7% of cases.
- Nearly half (49.2%) of the children presented with symptoms.
- Respiratory symptoms varied significantly across groups (73.7% in DAA, 24.2% in non-DAA, 100% in IA).
- Surgical repair was performed in 52.5% of patients, with symptom improvement observed post-operatively.
Conclusions:
- Current management protocols for AAMs lack standardization.
- Conservative management is viable for asymptomatic patients or those with less than 50% airway stenosis.
- Close patient follow-up is essential for early detection of symptom progression.
Background:
Aortic arch malformations (AAMs) should be suspected in the presence of persistent respiratory symptoms despite medical treatment or feeding problems at the pediatric age.
Aim:
We report a descriptive cohort of patients with AAMs and the local management protocol applied.
Methods:
A total of 59 patients with AAM were retrospectively reviewed. Three groups were identified: double aortic arch (DAA), group 1; complete vascular ring (non-DAA), group 2; and anomalous origin of the innominate artery (IA), group 3.
Results:
Prenatal diagnosis was available for 62.7% of the patients. In all, 49.2% of children were symptomatic. There was a significantly different prevalence of respiratory symptoms within the three groups: 73.7% in group 1, 24.2% in group 2, and 100% in group 3 (p-value: <0.001). Surgery was considered in the presence of symptoms in patients with DAA and in those with reduction of the tracheal section area greater than 50%. A total of 52.5% of the patients underwent surgical repair (median age 6 months). The median follow-up interval was 21.9 months. Respiratory symptoms improved in most symptomatic patients.
Conclusions:
No specific protocols are available for the management of patients with AAMs. Conservative treatment seems to be reasonable for asymptomatic patients or those with airway stenosis less than 50%. A close follow-up is necessary to identify early patients who become symptomatic.
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