Aberrant right subclavian artery(ARSCA)is believed to have a benign natural history with no significant compression of the trachea and esophagus, in contrast to complete vascular ring(CVR). However, our clinical observation is that ARSCA can cause significant swallowing difficulties. In this study, we prospectively performed esophageal manometry to evaluate the severity of esophageal function in subjects with ARSCA and CVR and efficacy of surgical intervention.

From January, 2002 to December, 2007, 46 children of ARSCA(8.5+/-2.9 years)and 40 with CRV(6.9+/-4.1 years,p>0.05)were enrolled after confirmation of diagnosis by a history of dysphagia, barium swallow, echocardiography, and computed tomography. Patients with developmental delay or mental retardation were excluded. Esophageal manometry was performed preoperatively and postoperatively to measure the severity of esophageal compression.

Esophageal manometric measurement was successfully obtained for all subjects. All had symptomatic relief of dysphagia. All subjects had a high-pressure zone at the site of vascular compression. The maximal and mean preoperative esophageal pressures were not statistically different between ARSCA(77.4+/-36.9 mmHg and 45.8+/-25.8 mmHg)and CVR (98.6+/-37.1 mmHg,p>0.05 and 53.1+/-28.9 mmHg,p>0.05),respectively. Postoperatively, both maximal and mean pressures were significantly reduced for ARSCA(41.8+/-25.8 mmHg and 23.8+/-15.3 mmHg)and for CVR(53.1+/-37.1 mmHg and 28.9+/-15.1 mmHg),respectively.

This is the first study to demonstrate that ARSCA has a significant impact on esophageal function as CVR has. Esophageal manometry revealed a typical high pressure zone that is useful to quantitatively evaluate the severity of obstruction and the efficacy of surgical intervention. Left thoractomy with aortopexy may provide an effective surgical approach for ARSCA with restoration of esophageal function.