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You are here: Contents > 2015 > Volume 24 Number 6 November 2015 > AORTIC VALVE DISEASE > Repair of Bicuspid Aortic Valve Syndrome with Anomalous Right Coronary Artery in Osteogenesis Imperfecta

Repair of Bicuspid Aortic Valve Syndrome with Anomalous Right Coronary Artery in Osteogenesis Imperfecta

Robert Patrick Davis1, Nicholas D. Andersen1, Asvin M. Ganapathi1, Louis R. DiBernardo2, G. Chad Hughes1

1Division of Cardiovascular & Thoracic Surgery, Department of Surgery, Duke University Medical Center, Durham, North Carolina, 2Department of Pathology, Duke University Medical Center, Durham, North Carolina, USA

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.

The Journal of Heart Valve Disease 2015;24:666-668

Repair of Bicuspid Aortic Valve Syndrome with Anomalous Right Coronary Artery in Osteogenesis Imperfecta

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