Ease of Sutureless aortic valve replacement in a patient with unexpected Ochronosis -A Case Report

Abstract

Abstract Background: Alkaptonuria is a rare congenital metabolic disorder that results in homogenetic acid accumulation in body cartilage and connective tissues due lack of homogenetic acid dioxygenase enzyme. It has different clinical manifestations, including: spondyloarthropathy, eye and skin pigmentation, obstruction of the genitourinary tract by ochronosis stones, and involvement of the cardiovascular system. Cardiac ochronosis is a rare presentation of alkaptonuria that the patient has aortic stenosis with or without other cardiovascular involvement. Case presentation: We present an unexpected case of ochronosis that diagnosed during cardiac surgery.Sutureless aortic valve replacement was done for her. Because of fragile, thin and atheromatous ascending aorta in patients with ochronosis, it seems that this sutureless valve replacement method is more suitable in these sorts of patients. Conclusions: Although cardiac ochronosis is very rare, but the surgeons should keep it in mind and pay attention to the clinical manifestations of alkaptonuria when examining a patient with aortic valve stenosis.