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Intermediate to late results of surgical relief of vascular tracheobronchial compression
Insights
Surgical repair of congenital aortic arch anomalies in 69 children significantly improved outcomes. Most infants with these rare vascular ring anomalies are now well long-term after surgery.
Area of Science:
- Cardiovascular Surgery
- Pediatric Cardiology
- Thoracic Surgery
Background:
- Congenital aortic arch anomalies are rare but can cause significant morbidity in infants.
- Surgical intervention is often necessary for conditions like double aortic arch and anomalous innominate artery origin.
Purpose of the Study:
- To evaluate the surgical outcomes and long-term results for infants and children undergoing repair of various congenital aortic arch anomalies.
Main Methods:
- Retrospective review of 69 pediatric patients who underwent surgical correction for aortic arch anomalies between 1979 and an unspecified end date.
- Surgical approaches included left thoracotomy; postoperative evaluation utilized tracheobronchoscopy, MRI, and pulmonary function testing.
Main Results:
- The most common anomalies were double aortic arch and anomalous innominate artery origin.
- Preoperative morbidity was high, with 84% symptomatic in the first trimester and 24 requiring mechanical ventilation.
- There were 4 early/late deaths and 5 reoperations; however, all 65 survivors are well long-term (mean 3.9 years post-surgery).
Conclusions:
- Surgical repair of congenital aortic arch anomalies in children leads to favorable long-term outcomes.
- Despite good clinical recovery, minor airway compression may persist postoperatively, necessitating careful evaluation.
Abstract:
Since 1979, a total of 69 infants and children aged 0.1 to 11.9 (median 0.7) years required surgical intervention for: double aortic arch (26), anomalous origin of innominate artery (26), right aortic arch with left ligamentum arteriosum (9), pulmonary artery sling (5), retroesophageal right subclavian artery (3). Before operation, the morbidity was high: 84% of children were symptomatic in the first trimester of life with 24 patients requiring mechanical ventilation. Seven children had an accompanying heart defect. Left thoracotomy was the preferred approach. There were 2 early (asphyxic brain damage, postoperative pneumonia) and 2 late (tracheomalacia, complex heart defect) deaths. Five patients (2 originally operated elsewhere) needed reoperation for persistent symptoms. All 65 survivors are well 1 month to 11.9 (mean 3.9 +/- 3.62) years after surgery. Tracheobronchoscopy, magnetic resonance imaging, and lung function testing were helpful for postoperative evaluation. Minor tracheal compression was revealed in 4 patients despite their good clinical condition.