Green aortic valve: alcaptonuria (ochronosis) with severe aortic stenosis

Abstract

A 67-year-old woman with known alcaptonuria presented herself with a 4-week history of dyspnhea (NYHA II) and edema of the lower limbx. Upon presentation, electrocardiography showed negative T-waves in the chest leads; she had a slight elevation of myoglobin and troponin I. Transthoracic echocardiography showed a severe aortic stenosis with a systolic pressure gradient of 69 mm Hg and mild mitral regurgitation with thickening of both mitral valve leaflets. Cardiac catheterization revealed secondary pulmonary hypertension, diffuse narrowing of the left anterior descending artery, obliteration of the circumflex branch, and marked stenosis of the right coronary artery. The patient underwent surgery using cardiopulmonary bypass. Oblique aortotomy revealed a tricuspid aortic valve with thickened, dark green aortic valves. The aortic wall and the left ventricular outflow tract had a similar appearance, as shown in Figure 1. Aortic valve replacement, using a 21-mm “Top-Hat” valve (Carbomedics, Austin, TX) and coronary artery bypass grafting using both internal thoracic arteries was performed. The patient’s postoperative course was complicated by her preexisting pulmonary hypertension, requiring prolonged mechanical ventilation. However, the patient was discharged from the intensive care unit 2 weeks after surgery and remains well. Address reprint requests to Dr. Zund, Clinic for Cardiovascular Surgery, University Hospital Zurich, Ramistrasse 100, D-Lab 32, CH-8091 Zurich Switzerland; e-mail: gregor.zundchi.usz.ch.