Innominate Artery Steal Syndrome After Stage I Palliation for Hypoplastic Left Heart Syndrome

Abstract

Four neonates with hypoplastic left heart syndrome (HLHS) were found to have innominate artery steal syndrome (IASS) following stage I palliation with a modified Blalock-Taussig (B-T) shunt. All patients presented in the early postoperative period with a persistent supplemental oxygen requirement. Two-dimensional echocardiography with Doppler flow analysis and cardiac catheterization with angiography demonstrated obstruction at the origin of the innominate artery. This obstruction was associated with intermittent retrograde flow through the right vertebral and common carotid arteries into the distal innominate artery and modified B-T shunt. An ascending neoaorta to right pulmonary artery shunt, with takedown of the B-T shunt, was performed in two of the patients. The other two patients were treated with percutaneous balloon dilatation of the obstructed area. Both procedures improved arterial blood oxygen saturations and eliminated the need for supplemental oxygen. In three of four patients, the left common carotid artery was found to be larger than the right. All four patients developed microcephaly, and one patient had encephalomalacia in the distribution of the right anterior and middle cerebral arteries. The development of IASS following stage I palliation for HLHS and the association of neurologic sequelae from this complication are discussed.

Significant Stenosis of the Brachiocephalic Trunk and Moderate Stenosis of the Left Circumflex Artery in Computed Tomography Angiography Images

“…Most often, the steal syndrome affects the left subclavian artery and results from the narrowing of its proximal part [ 2 ]. In the articles published so far there are few case reports of the brachiocephalic steal syndrome [ 3 , 4 , 5 , 6 ]. In summary, they note that, in contrast to the subclavian steal, brachiocephalic trunk stenosis induces distinct and much more significant hemodynamic alterations in extracranial arterial flow and is rarely asymptomatic.…”

“…In summary, they note that, in contrast to the subclavian steal, brachiocephalic trunk stenosis induces distinct and much more significant hemodynamic alterations in extracranial arterial flow and is rarely asymptomatic. Symptoms of significant right-sided cerebrovascular insufficiency as well as progressive pain and weakness of the right upper extremity were the most common causes of diagnosis when brachiocephalic steal syndrome was diagnosed [ 3 , 4 , 5 , 6 ].…”