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Diagnosis and management of congenital vascular rings: a 22-year experience
K Chun1, P M Colombani, D L Dudgeon
1Division of Pediatric Surgery, Johns Hopkins University School of Medicine, Baltimore, MD 21205.
Insights
Early surgical repair of congenital aortic vascular rings is safe and effective. Treatment of associated aortic diverticula improves outcomes, allowing normal tracheal growth in most patients.
Area of Science:
- Cardiovascular Surgery
- Pediatric Surgery
- Thoracic Surgery
Background:
- Congenital aortic arch anomalies, or vascular rings, can cause significant respiratory symptoms in infants and children.
- These anomalies include right arch with aberrant left subclavian artery and double aortic arch, often associated with aortic diverticula.
Purpose of the Study:
- To evaluate the safety and efficacy of operative treatment for congenital aortic arch anomalies.
- To assess the outcomes of managing associated aortic diverticula in conjunction with vascular ring repair.
Main Methods:
- Retrospective review of 39 patients treated between 1968 and 1990 for congenital aortic arch anomalies.
- Surgical interventions included excision or aortopexy for aortic diverticula.
- Barium swallow was utilized for diagnosis, and outcomes were assessed based on symptom resolution and complications.
Main Results:
- Respiratory symptoms were present in 95% of patients, with right arch and double aortic arch being most common.
- Surgical repair, including management of aortic diverticula, resulted in rapid recovery and short hospital stays.
- Two deaths occurred due to specific complications; however, 97% of survivors were symptom-free post-operatively.
Conclusions:
- Early surgical repair of congenital aortic vascular rings is safe and effective.
- Addressing associated aortic diverticula through fixation or excision is crucial for optimal outcomes.
- Surgical intervention facilitates normal tracheal growth and long-term symptom relief.
Abstract:
Between 1968 and 1990, we operatively treated 39 patients (19 boys, 20 girls) with congenital aortic arch anomalies. Median age was 7 months (range, 1.5 months to 23 years). Thirty-seven patients (95%) had respiratory symptoms. Barium swallow was diagnostic in 95%. Right arch with aberrant left subclavian artery and double aortic arch were the most common types (11 each). Treatment of an aortic diverticulum was documented in 19 patients; the aortic diverticulum was excised (9), managed by aortopexy (7), or left in situ (3). Postoperative recovery was rapid, with a median intensive care unit stay of 2 days, time to oral feeding of 1 day, and postoperative time to discharge of 7 days. Two deaths occurred: 1 infant had undergone emergent operation for control of hemorrhage from an aortotracheal fistula due to tracheostomy tube erosion, and the other had multiple associated congenital heart defects. Postoperative complications included bleeding (1), pneumonia (5), and chylothorax (4). One boy had persistent severe symptoms due to an untreated aortic diverticulum and underwent subsequent excision of the aortic diverticulum with complete relief of symptoms. Median length of follow-up was 12.5 months, with at least 97% of survivors completely or nearly completely free of symptoms from the vascular ring. These results suggest that early repair of congenital aortic vascular rings, including fixating or excising an associated serious aortic diverticulum, is safe and effective and allows for normal tracheal growth.