title: Very Long Segment Congenital Thoracoabdominal Aortic Coarctation (Diffuse Aortic Dysplasia) with Infrarenal Aortobi-Iliac and Cavobi-Iliac Aplasia in a 30-Year-Old Patient.
abstract: Congenital dysplastic aortic syndromes range from coarctation at the aortic isthmus to more extended aortic disease (midaortic syndrome). The latter is usually restricted to dysplastic aortic segments of up to 15 cm. Long segment dysplasia of the entire abdominal or thoracic aorta is extremely rare. This case of a 30-year-old patient with a very long segment congenital thoracoabdominal aortic coarctation and infrarenal aortobi-iliac and cavobi-iliac aplasia represents to our knowledge the most extended congenital vascular malformation in a surviving adult patient. The developed extensive collateral pathways ensured the survival of the patient, so that the main clinical manifestation was a refractory hypertension. Because of the extent of the disease, open surgery represented the only viable option. Interestingly, after 30 years of uncontrollable hypertension, the patient's blood pressure promptly responded to surgical treatment. A concomitant infrarenal aplasia of both the aorta and cava vein is also very unusual and points to a major developmental deficit during vascular embryogenesis.
authors.affiliation: Department of Vascular and Endovascular Surgery, HELIOS Klinikum Krefeld, Krefeld, Germany. Electronic address: a.mamopoulos@web.de.
authors.forename: Apostolos
authors.initials: A
authors.lastname: Mamopoulos
authors.affiliation: Department of Vascular and Endovascular Surgery, HELIOS Klinikum Krefeld, Krefeld, Germany.
authors.forename: Bernd
authors.initials: B
authors.lastname: Luther
mesh_terms: D000328:Adult; D001012:Aorta, Abdominal / Q000002:abnormalities* / Q000503:physiopathology / Q000601:surgery; D001013:Aorta, Thoracic / Q000002:abnormalities* / Q000503:physiopathology / Q000601:surgery; D001017:Aortic Coarctation / Q000150:complications / Q000175:diagnosis / Q000503:physiopathology / Q000601:surgery; D001027:Aortography / Q000379:methods; D001794:Blood Pressure; D019917:Blood Vessel Prosthesis Implantation; D003097:Collateral Circulation; D005260:Female; D006801:Humans; D006973:Hypertension / Q000209:etiology / Q000503:physiopathology; D007083:Iliac Artery / Q000002:abnormalities* / Q000503:physiopathology; D018810:Magnetic Resonance Angiography; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D014682:Vena Cava, Inferior / Q000002:abnormalities* / Q000503:physiopathology