Vascular rings are rare congenital anomalies caused by an anomalous configuration of the aortic arch or associated vessels surrounding the trachea and esophagus to form a complete or incomplete compressing ring around them. They make up less than 1% of all congenital cardiac defects. The most common forms are double aortic arch, right aortic arch, pulmonary artery sling, vascular rings associated with left aortic arch, and cervical aorta.
Patients often present with symptoms in the first few months of life and require surgery early in life. The classic sign is the “seal-bark” cough. In addition noisy breathing may be heard both during inspiration and expiration (biphasic stridor). A common finding in all forms of vascular rings is the increased occurrence of respiratory infections.
Diagnostic tests for visualization of the relevant pathology and the exact location of the obstruction include computerized tomography scan, magnetic resonance imaging, and digital subtraction angiography. Echocardiography is useful in the diagnostic workup of associated congenital cardiac defects.
In patients with pulmonary artery sling, repair is undertaken via median sternotomy utilizing cardiopulmonary bypass and reimplanting the left pulmonary artery onto the left side of the main pulmonary artery. If necessary, tracheal repair can be performed concurrently. All other lesions are usually approached via a left-sided thoracotomy. The goal of surgical therapy in patients with a double aortic arch is to divide the smaller of the two arches at a site that does not compromise the blood flow to the head vessels. In patients with right-sided aortic arch, the ligamentum arteriosum is divided. Division of the left subclavian artery is not generally necessary for relieving tracheal compression. Patients with an anomalous left subclavian artery and Kommerell’s diverticulum may additionally have resection of the diverticulum and reimplantation of the left subclavian artery to the left carotid artery as a primary operation.
Excellent results have been achieved in recent years without operative mortality in high-volume centers. Most of the patients are essentially free of symptoms in the long term, and only a very small number need further interventions or supportive treatment.
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Republished with permission of Taylor and Franciss Group LLC books, from Newborn Surgery, PD. Dr.med. Benjamin Bierbach and Professor Mark Redmond, Vascular rings, chapter 39, fourth edition, 2017; permission conveyed through Copyright Clearance Center, Inc.
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