Aortic Arch Bare Metal Stenting in a 10-Years-Old Boy with DiGeorge Syndrome Using Overdrive External Pacing

Abstract

Introduction: In the last decade, percutaneous stent placement has become the standard for treating CoA after childhood. However, stent placement as a treatment for aortic arch hypoplasia has only been described in small series and case reports. Case Report: A nine-year-old boy with DiGeorge syndrome (BW 50 kg, BL 140 cm, BSA 1.4 m2) was admitted to our hospital due to planned heart catheterisation with aortic arch stenting. In the neonatal period, he underwent complete surgical correction of the IAA type B with perimembranous VSD. During the follow-up period, the progressive narrowing of the aortic arch was observed with gradually increasing systolic pressure gradient (PG) on the echocardiography examination (PG 55 mmHg). The cardiac catheterization was performed under general anaesthesia. The peak-to-peak gradient was estimated at 32 mmHg (AoAsc 128/78 mmHg; AoDsc 96/68 mmHg). Aortography pointed out tubular hypoplasia of the aortic arch between branches (17 mm in length) with localized narrowing right behind the first aortic branch, 6 mm in diameter, and post-stenotic dilatation. The stenosis was resolved using a 22mm long uncovered Cheatham Platinum (CP) stent crimped on a 12 mm Balloon-in-Balloon (BiB) catheter with additional flaring of the distal stent part (Figure 1B and C). During the stent implantation, an overdrive external pacemaker (wire was placed into the right ventricle anterograde across the right femoral vein) was used. Control angiography relieved satisfying anterograde flow across the aorta and aortic branches. The peak-to-peak gradient after stenting was 10 mmHg. Conclusion: Although technically challenging, carefully analyzing the arch and vessel morphology allows the dilation of the transverse aortic arch hypoplasia and stenosis using a bare metal stent with good angiographic and hemodynamic results and without complications.