title: Surgical management of a hypoplastic distal aortic arch and coarctation of aorta in a patient with Klippel-Feil syndrome, ascending aortic aneurysm and bicuspid aortic valve.
abstract: Klippel-Feil syndrome has been associated with cardiovascular malformations, but only 3 cases have been reported to be associated with aortic coarctation and surgical management is not defined. A 51-year old woman with Klippel-Feil syndrome associated with an aneurysm of the ascending aorta, hypoplastic aortic arch and aortic coarctation at the level of the left subclavian artery presented with shortness of breath 2 years after diagnosis. Imaging identified interim development of a 7.2-cm aneurysm at the level of the aortic coarctation. She underwent surgical repair with a Dacron interposition graft under hypothermic circulatory arrest. She continues to do well 18 months following repair.
authors: Clinic of Cardiac Surgery, Eastern Slovak Institute for Cardiovascular Diseases, Kosice, Slovak Republic.
authors: Frantisek
authors: F
authors: Sabol
authors: Clinic of Cardiac Surgery, Eastern Slovak Institute for Cardiovascular Diseases, Kosice, Slovak Republic adriankolesar@seznam.cz.
authors: Adrián
authors: A
authors: Kolesar
authors: Clinic of Cardiac Surgery, Eastern Slovak Institute for Cardiovascular Diseases, Kosice, Slovak Republic.
authors: Tomás
authors: T
authors: Toporcer
authors: University of Massachusetts Medical Center, Worcester, USA.
authors: Milan
authors: M
authors: Bajmoczi
mesh_terms: D000015:Abnormalities, Multiple; D001013:Aorta, Thoracic / Q000002:abnormalities / Q000000981:diagnostic imaging / Q000601:surgery*; D001014:Aortic Aneurysm / Q000175:diagnosis / Q000209:etiology / Q000601:surgery*; D001017:Aortic Coarctation / Q000150:complications / Q000175:diagnosis / Q000601:surgery*; D001021:Aortic Valve / Q000002:abnormalities*; D001027:Aortography / Q000379:methods; D000082882:Bicuspid Aortic Valve Disease; D001807:Blood Vessel Prosthesis; D019917:Blood Vessel Prosthesis Implantation / Q000295:instrumentation; D005260:Female; D006324:Heart Arrest, Induced; D006349:Heart Valve Diseases / Q000150:complications* / Q000175:diagnosis; D006801:Humans; D007036:Hypothermia, Induced; D007714:Klippel-Feil Syndrome / Q000150:complications* / Q000175:diagnosis; D008875:Middle Aged; D011093:Polyethylene Terephthalates; D011474:Prosthesis Design; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
chemical_list: D011093:Polyethylene Terephthalates
keywords: Aortic coarctation; Hypoplastic distal arch; Klippel–Feil syndrome