title: Repair of Bicuspid Aortic Valve Syndrome with Anomalous Right Coronary Artery in Osteogenesis Imperfecta.
abstract: Osteogenesis imperfecta (OI) is an inherited connective tissue disorder caused by the defective synthesis of type I collagen. The clinical phenotype is dominated by bone fragility, but cardiovascular tissue involvement has also been reported. Here, the case is described of a 37-year-old man with OI who presented with aortic insufficiency, bicuspid aortic valve, dilated aortic root, and anomalous right coronary artery. The patient was treated successfully with a mechanical valved conduit aortic root replacement and anomalous coronary artery unroofing and reimplantation. This case highlights the feasibility of complex surgical repairs in this population, as well as challenges surrounding the choice of valve prosthesis given the congenital bone fragility and predilection for fractures.
authors.affiliation: Division of Cardiovascular & Thoracic Surgery, Department of Surgery, Duke University Medical Center, Durham, North Carolina.
authors.forename: Robert Patrick
authors.initials: RP
authors.lastname: Davis
authors.affiliation: Division of Cardiovascular & Thoracic Surgery, Department of Surgery, Duke University Medical Center, Durham, North Carolina.
authors.forename: Nicholas D
authors.initials: ND
authors.lastname: Andersen
authors.affiliation: Division of Cardiovascular & Thoracic Surgery, Department of Surgery, Duke University Medical Center, Durham, North Carolina.
authors.forename: Asvin M
authors.initials: AM
authors.lastname: Ganapathi
authors.affiliation: Department of Pathology, Duke University Medical Center, Durham, North Carolina, USA.
authors.forename: Louis R
authors.initials: LR
authors.lastname: DiBernardo
authors.affiliation: Division of Cardiovascular & Thoracic Surgery, Department of Surgery, Duke University Medical Center, Durham, North Carolina.
authors.forename: G Chad
authors.initials: GC
authors.lastname: Hughes