[Double aortic arch: clinical aspects, diagnosis and therapy in children and adults]
Insights
Double aortic arch, a rare vascular anomaly, often causes airway compression in infants. Surgical resection of the smaller arch effectively resolves symptoms, even in oligosymptomatic adults.
Area of Science:
- Cardiovascular Surgery
- Pediatric Surgery
- Medical Imaging
Background:
- Double aortic arch is a rare congenital vascular anomaly.
- It frequently leads to tracheal and esophageal compression in infants, causing significant symptoms.
- Delayed diagnosis can occur, even into adulthood, despite early childhood indicators.
Purpose of the Study:
- To evaluate symptoms, diagnostic methods, and treatment outcomes for double aortic arch.
- To emphasize the importance of early diagnosis and surgical intervention.
- To assess the utility of advanced imaging techniques in diagnosis.
Main Methods:
- Retrospective review of 8 patients (7 children, 1 adult) treated over 30 years.
- Analysis of presenting symptoms, diagnostic imaging (esophagography, bronchography, angiography, MRI, CT), and surgical procedures.
- Evaluation of surgical outcomes following resection of the smaller aortic arch.
Main Results:
- Common symptoms included dyspnea, stridor, recurrent pulmonary infections, feeding difficulties, and failure to thrive.
- Diagnostic imaging confirmed tracheal and esophageal compression in most patients.
- Surgical resection of the smaller aortic arch, performed via posterolateral thoracotomy, was successful in all patients, resolving symptoms completely.
- Magnetic resonance angiography and computed tomography proved effective alternatives to conventional angiography.
Conclusions:
- Prompt diagnosis and surgical treatment of double aortic arch in early childhood are crucial.
- Surgical resection of the smaller aortic arch is recommended even for oligosymptomatic patients to prevent future complications.
- Less invasive imaging modalities like MRI and CT can replace preoperative angiography.
Abstract:
Double aortic arch is a rare vascular anomaly which usually causes tracheal and esophageal compression in the first few months of life. During the last 30 years, 7 children, 2 to 24 months old, and one 29-year-old woman with double aortic arches have been treatedatour institution. Symptoms, diagnosis and treatment of these patients were evaluated. Dyspnoe, stridor, recurrent pulmonary infections, feeding problems and failure to thrive were the leading symptoms. Despite typical symptoms from early childhood, the diagnosis was missed in our adult patient. Typical compression of the esophageus and the trachea was visualized by esophagography by 7 and bronchoscopy/-graphy by 6 patients. Angiography was performed in all children, whereas magnetic resonance angiography and computed tomography were done in the adult patient. Resection of the smaller aortic arch, left in 3 and right in 5, through a right or a left posterolateral thoracotomy was uncomplicated and fully resolved the symptoms in all patients. Typical symptoms in early childhood should lead to prompt diagnosis and surgical treatment of double aortic arch. Surgical resection of the smaller aortic arch should also be performed in oligosymptomatic patients to prevent complications later. Preoperative angiography can be replaced by the less invasive magnetic resonance imaging and computed tomography.
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