Modified Aortic Uncrossing Procedure: A Novel Approach for Norwood Palliation of Complex Univentricular Congenital Heart Disease With a Circumflex Aorta

Abstract

The circumflex aorta is a rare type of true vascular ring anomaly. It consists of a retroesophageal right aortic arch, a left-sided descending thoracic aorta, and a left-sided ligamentum arteriosum. The "aortic uncrossing procedure" described by Planché and Lacour-Gayet is the procedure of choice for managing this aortic anomaly in patients with a biventricular heart. The presence of a circumflex aorta in a patient with heterotaxy syndrome and univentricular congenital heart disease requiring Norwood palliation is highly unusual. We report such a case and describe our approach to its surgical management.

Circumflex aorta with double aortic arch in an infant

“…The surgery could also be done via a median sternotomy without cardiopulmonary bypass using the side‐biting clamp on the ascending aorta . Some modifications have been made to the uncrossing procedure to address the unusual anatomy in heterotaxy single‐ventricle hearts by incorporating the arch into the Damus‐Kaye‐Stansel anastomosis . Dealing with the circumflex aorta in the adult population is challenging as mobilization of the aorta to perform an arch advancement is extremely difficult.…”

Norwood Procedure With Left Ventricle Exclusion in Complex Single Ventricle Patients: A Novel Technique

“…Our technique expands on previous methods of palliating severe atrioventricular valve regurgitation. 4,5 This small case series provides evidence that a Norwood operation coupled with LVE by a fenestrated mitral valve patch, and aortic valve oversewing, leaving a small washing jet at the commissure to prevent widening/tearing of the defect, has thus far proven to be an effective method in this unique patient population. Patient 1's success demonstrated that LVE via a fenestrated PTFE patch could be safely accomplished when severe mitral valve regurgitation in a single ventricle patient is present.…”

“…Patient 1 was originally described in 2017 as a complex case report; however, utilization of and expansion upon that original surgical technique in similar cases thereafter led to the more developed and reproducible palliative procedure described herein. 4…”

Congenital arch malformation: a review of morphology and surgical management of circumflex aortic arch

“…Other reported cardiac lesions include atrial septal defect and bicuspid aortic valve [14,33] . Less commonly, Tetralogy of Fallot or double outlet RV, a lesion with a higher frequency of right aortic arch than in the normal population, had also been reported in circumflex aorta [15] ; as well as left heart hypoplasia [5,34] .…”

“…Cardiopulmonary bypass with deep hypothermic total circulatory arrest can be used; but we preferred the strategy of selective antegrade cerebral perfusion via the innominate artery perfused via a 3 mm to 3.5 mm Goretex shunt [6] . Aortic uncrossing procedure has also been used in single ventricular physiology with aortic arch obstruction requiring a concomitant Norwood procedure [34] .…”