A right aortic arch with a left descending aorta and an aberrant left innominate artery is a rare but recognized vascular anomaly that can result in compression of the trachea and the esophagus. This vascular anomaly has been diagnosed using cardiac catheterization and angiography. Recently, computed tomography and magnetic resonance imaging have been used for noninvasive diagnosis. The aim of this report is to highlight the possibility of echocardiographic diagnosis.
Case Report
A symptomatic 8-month-old boy was referred to our cardiology department for evaluation of inspiratory stridor, noted since birth but worse in the previous month. The patient was affected by biliary atresia and had undergone a Kassai portoenterostomy at 2 months of age. By 5 months of age, he had developed biliary cirrhosis and hepatic insufficiency. His weight was 6.7 kg. A cardiac examination revealed a heart rate of 110 beats/min, normal blood pressure (88-90/55-60 mm Hg) and pulse in all 4 extremities, and a grade 2 systolic murmur at the right sternal border. Electrocardiographic results were normal. Fiber optic endoscopy showed normal laryngeal morphology and tracheomalacia of the distal third of the trachea and proximal left main bronchus. A pulsatile mass was seen indenting the back wall of the carina and the origin of the left bronchus.
Two-dimensional echocardiography demonstrated normally related great arteries {S,D,S}. There was a bicommissural aortic valve with a mild systolic gradient and mild mitral valve dysplasia. A right aortic arch with a left descending aorta was diagnosed; an aberrant left innominate artery arose from the proximal descending aorta. The ductus arteriosus was not patent. The right aortic arch was imaged directly to the right of the airway shadow in a suprasternal axial view ( Figure 1 ). The left descending aorta and the left innominate artery arising from it were imaged directly in a left parasagittal plane view ( Figures 2 A and 2 B). Although the posterior arch segment could not be imaged directly because of shadowing by the airway, its presence was inferred by the curving of the distal arch to the left before disappearing behind the airway ( Figure 1 ) and the connection to the descending aorta, indicated by the diverging flow patterns seen in the innominate artery and descending aorta ( Figure 2 B).
Cardiac catheterization ( Figure 3 ) was performed to exclude vascular anomalies in the intracranial circulation in case the innominate artery had to be cross-clamped during surgical treatment.
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