Vascular rings are congenital anomalies occurring early in the development of the aortic arch and great vessels. The aorta originally develops as a series of arches with bilateral symmetry. By the end of the second month of foetal life, parts of the arch complex have regressed leaving the 'typical' anatomy of a left aortic arch. The two most common types of complete vascular rings are double aortic arch (Figure 1) and right aortic arch with left ligamentum arteriosum, making up 85–90% of cases. Vascular rings make up less than 1% of congenital cardiac defects. Some rings are isolated defects while others occur with heart defects.
Primary symptoms associated with vascular rings relate to structures encircled by the ring such as the trachea and oesophagus. Occasionally presenting with acute life threating emergencies (ALTE), they usually present with respiratory/deglutition abnormalities but can occasionally be asymptomatic, incidental findings. Traditionally identified by barium swallow, cross-sectional modalities (CT/MRI) are the chosen modality; diagnosis can be missed by echocardiography alone.
Method A retrospective study identifying all patients with vascular rings using the cardiac database presenting between 2001–2015. We excluded aberrant right subclavian artery with left arch as this does not form a vascular ring.
Results 69 patients were diagnosed with a vascular ring. There was a male preponderance (45/69). 4 patients died. Other cardiac lesions identified in 28 patients. 58 had surgery.
The most common diagnosis was double aortic arch (40 patients), followed by right aortic arch with aberrant left subclavian (26 patients).
Stridor was the commonest symptom (34 patients). 12 patients identified following imaging for other cardiac abnormalities. 10 were identified through respiratory symptoms (persistent cough, chest infections). 6 were identified through PICU and 2 had acute life threatening events. 5 patients had diagnosis missed on echocardiogram or barium swallow.
Conclusion Vascular rings can present in a variety of different ways. The majority of cases require surgery. Cross sectional studies should be undertaken to prevent missed diagnosis.
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