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

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.