Norwood Procedure in a Patient With Hypoplastic Left Heart Syndrome, Right Aortic Arch, and Right Descending Aorta

Abstract

We describe the surgical treatment of a patient with hypoplastic left heart syndrome and right aortic arch.

Hybrid Norwood for Hypoplastic Left Heart Syndrome With Rare Aortic Arch Anatomy

“…Palacios-Macedo and colleagues isolated the left subclavian artery, which was ligated at the time of the Norwood. 8 Ligating the innominate artery without reimplanting it would leave a patient with single ventricle physiology. This could potentially place the patient at an unnecessarily high risk for ischemic stroke following first-stage palliation.…”

“…However, HLHS with an RAA and RDA is an extremely rare association with very little information in the literature regarding optimal surgical management. [1][2][3][4][5][6][7][8] Traditional Norwood-style reconstruction is not possible with a right arch and normally related great vessels because the arch reconstruction and APA would be on opposite sides. Therefore, alternative surgical strategies must be used.…”

“…Most recently, a novel surgical approach was described by Palacios-Macedo and colleagues. 8 Their technique involved dividing the ascending aorta, reconstructing the aortic arch with a generous patch of pericardium, and sewing this end-to-end to the proximal pulmonary root. The divided small ascending aorta was then reimplanted into the pulmonary root end-to-side.…”

“…There are few reports regarding alternative surgical techniques in this rare subset of patients. [1][2][3][4][5][6][7][8] However, to our knowledge, no reports of a hybrid approach for this anatomic substrate in patients with HLHS exist. We describe a patient with HLHS and unusual aortic arch anatomy who underwent a hybrid procedure as first-stage palliation.…”