title: Laubry-Pezzi syndrome with aortic root dilatation treated with a bentall and De Bono procedure.
abstract: Laubry-Pezzi syndrome is a clinical entity in which prolapse of an aortic valve cusp into a subjacent ventricular septal defect (VSD) due to Venturi effect results in progressive aortic valve insufficiency. Aortic valve prolapse is found in over 5% of children with VSDs, most commonly in association with supracristal VSDs, and the risk of development of aortic regurgitation increases during childhood, peaking at 5 to 10 years of age. The VSD closure eliminates the low-pressure zone that is the cause of ongoing aortic valve cusp deformity and, if performed early, prevents the development of aortic regurgitation. However, the management of this rare pathology is still a matter of some debate with respect to indications, operative techniques, and timing. We report the case of a patient with Laubry-Pezzi syndrome, originally operated on at six years of age for VSD closure and plasty between the left and the right aortic cusps. However, in the follow-up the patient developed severe aortic regurgitation and aortic root dilatation. Eventually, a Bentall and De Bono procedure was performed.
authors: Complejo Hospitalario Universitario Insular-Materno Infantil, Las Palmas de Gran Canaria, Spain.
authors: Efrén
authors: E
authors: Martínez-Quintana
authors: Fayna
authors: F
authors: Rodríguez-González
authors: María Jesús
authors: MJ
authors: López-Gude
mesh_terms: D001022:Aortic Valve Insufficiency / Q000209:etiology / Q000601:surgery*; D001023:Aortic Valve Prolapse / Q000150:complications / Q000601:surgery*; D019917:Blood Vessel Prosthesis Implantation / Q000379:methods*; D006345:Heart Septal Defects, Ventricular / Q000601:surgery*; D019918:Heart Valve Prosthesis Implantation / Q000379:methods*; D006801:Humans; D008297:Male; D024162:Second-Look Surgery; D013577:Syndrome; D055815:Young Adult
keywords: Laubry; aorta; aortic valve; regurgitation; valvuloplasty