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Clinical, radiological and functional follow-up after surgical decompression of double aortic arch
Maartje ten Berge1, Johan van der Laag, Cornelis K van der Ent
1Department of Respiratory Diseases, Wilhelmina Children's Hospital, Lundlaan 6, 3584 EA Utrecht, The Netherlands.
Insights
Surgical relief of double aortic arch (DAA) significantly improved symptoms. However, persistent tracheal and esophageal narrowing, along with abnormal lung function, were observed in long-term follow-up of these congenital vascular anomalies.
Area of Science:
- Cardiovascular Surgery
- Pediatric Surgery
- Thoracic Surgery
Background:
- Double aortic arch (DAA) is a congenital vascular anomaly causing significant tracheal and esophageal compression.
- Surgical intervention is indicated for severe symptomatic cases.
Purpose of the Study:
- To assess clinical, radiological, and functional outcomes after surgical correction of DAA.
- Evaluate long-term results in pediatric patients.
Main Methods:
- Ten children with DAA underwent surgical decompression.
- Follow-up included clinical assessment, radiological imaging, and pulmonary function tests (Maximal Expiratory Flow Volume curves, peak expiratory flow).
- Methacholine challenge was used to assess bronchial hyper-reactivity.
Main Results:
- Marked improvement in clinical symptoms was reported post-surgery.
- Radiological evaluation showed persistent narrowing of the trachea and esophagus.
- Pulmonary function tests revealed reduced peak expiratory flow and signs of upper airway obstruction, with two patients exhibiting bronchial hyper-reactivity.
Conclusions:
- Surgical decompression of DAA effectively alleviates clinical symptoms.
- Despite surgical success, residual radiological narrowing and abnormal long-term lung function persist.
- Ongoing monitoring is crucial for patients with DAA post-surgery.
Background:
Double aortic arch (DAA) is a congenital vascular anomaly that causes tracheal and oesophageal compression. It requires surgical intervention in patients with severe symptoms.
Objective:
To evaluate the clinical, radiological and functional follow-up after surgical relief of the compression.
Materials And Methods:
Ten children (seven boys) with DAA were operated on at a mean age of 1.3 years (range 0.2-7.5). At a mean age of 10.1 years (range 5-18 years), a follow-up study was performed that included clinical, radiological and functional parameters.
Results:
Seven children reported only mild respiratory symptoms and some trouble with swallowing. Preoperative fluoroscopy with spot images showed the mean tracheal diameter at the level of stenosis to be 37+/-23% of the maximal diameter. At the time of follow-up, this was 70+/-13%. The mean of the oesophageal diameter was 39+/-20% preoperatively and 47+/-16% postoperatively. Maximal expiratory flow volume (MEFV) curves of seven children showed typical characteristics of intrathoracic upper airway obstruction. Mean peak expiratory flow was significantly reduced (77+/-10% of predicted, P<0.0001). Bronchial hyper-reactivity, tested by methacholine challenge, was found in two patients.
Conclusions:
There was marked relief of clinical symptoms after surgical decompression of DAA in all patients. In spite of this, radiological narrowing of trachea and oesophagus persisted and lung function results were abnormal at long-term follow-up.