Supravalvular aortic arch stenosis: A report of progressive disease and endovascular hybrid repair in a child

Abstract

Objective: This case highlights the clinical consequences of elastin (ELN) arteriopathy, which affects the supravalvular aorta, the branches of the arch and pulmonary arterial branches. We present a seven-year-old male patient with complex, inherited, non-syndromic supravalvular aortic stenosis (SVAS) who underwent numerous surgical procedures since birth. We highlight his most recent hybrid operation, which included a stent-in-stent endovascular strategy to treat significant recurrent stenosis of the aortic arch. Method: This hybrid reconstruction included carotid and subclavian extra-anatomical bypasses, followed by an aortic arch stent-graft-in-stent-graft (SG-SG) insertion where a stainless-steel stent-graft was placed as an inlay within another nitinol stent-graft. Thereafter, a 16mm high-pressure balloon was used to adequately expand both stent-grafts. Results: Our findings revealed that the SG-SG technique of the arch improved and alleviated the patient's hypertension and lower claudication symptoms. Although 1-year follow up scans revealed that both stent grafts were patent, progressive arteriopathy affected other aortic branch vessels. Conclusion: Aortic arch stent graft insertion may be considered as an alternative to open surgery in aortic restenosis. A bypass procedure involving extra-anatomical right carotid-to-left carotid and left carotid-to-left subclavian may be considered to facilitate stent graft insertion. Endovascular interventionalists need to be aware of the progressive consequences of elastin arteriopathy.