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Recurrent coarctation in a patient with Alagille syndrome

Department of Paediatric Cardiothoracic Surgery, Alder Hey Children's Hospital, Eaton Rd, Liverpool, L12 2AP, UK

*Corresponding author. Tel.: +44-151-2525635; fax: +44-151-2525643.

E-mail address: tanishq26{at}yahoo.com (V. Agarwal).

A boy presented with Alagille syndrome and recurrent coarctation of the descending thoracic aorta. Initially, he underwent resection of the coarctation segment and end-to-end anastomosis. Following recurrent coarctation an extra-anatomic bypass procedure (a conduit was interposed between the ascending and descending thoracic aorta) was performed. He recently presented with stenosis at the anastomosic site between the extra-anatomic conduit and ascending aorta. Tissue overgrowth at the anastomotic site was responsible for the ‘recoarctation’. This was surgically relieved. Patients with Alagille syndrome have vascular lesions affecting multiple organs. The management of these patients is developing. We highlight the need to tailor-make treatment to the requirements of the individual, the need for close follow-up, and the importance of long-term management of these patients.

Key Words: Aortic coarctation; Cardiac surgery; Alagille syndrome