Segmental agenesia of the descending aorta treated by extra-anatomic bypass

Abstract

We present a 12-year-old girl with clinical manifestation of restenosis after coarctation repair in the neonatal period. However, MR-angiography revealed hypoplasia of the distal arch, complete absence of the descending aorta and severe hypoplasia of the thoraco-abdominal aorta. Treatment consisted of an extra-anatomic ascending–descending PTFE graft via sternotomy with normalization of upper-body hypertension and disappearance of leg claudication. It demonstrates how this very rare pathology of true segmental aortic agenesia may successfully be treated in older children with coexisting aortic arch or isthmus anomalies. Long-term follow-up is needed to assess the potential benefit of this approach.