Case report
A 64-year-old male with a known history of hypertension, dyslipidemia, ankylosing spondylitis, alkaptonuria, right bundle branch block, and known mild to moderate aortic valve stenosis presented with progressive dyspnea on exertion and a two-month history of 20-pound weight loss. On physical examination the patient was noted to have bony enlargement of both knees, marked thoracic and cervical kyphosis, and blue-black discoloration of the sclera and pinna (Figure 1 ). A transthoracic echocardiogram (TTE) demonstrated a well-preserved left ventricular function with an ejection fraction of 65%, left ventricular hypertrophy with a maximum septal diameter of 2.20 cm, and severe aortic valve stenosis. Aortic valve stenosis was characterized by diffuse sclerosis and calcification with reduced leaflet excursion. An estimated aortic valve area of 1 cm2 was noted with a peak aortic valve gradient of 69 mmHg and mean gradient of 41 mmHg. After CT angiography of the chest demonstrated evidence of diffuse coronary calcification, he underwent cardiac catheterization which revealed severe three vessel coronary artery disease. The patient was referred to the Cardiac Surgery service for surgical evaluation and was recommended three vessel coronary artery bypass grafting (CABG), septal myectomy and aortic valve replacement with a bovine pericardial valve.
During arteriotomy of the native diseased coronary vessels and harvesting of both internal thoracic arteries, a diffuse bluish discoloration of the endothelial layer was noted (Figure 2 ). Upon septal myectomy, blue pigment was noted across the myocardium (Figure 2 ). Similarly, during the transverse aortotomy the tri-leaflet aortic valve had diffuse macular discoloration with bluish and blackish areas, especially in correspondence of the annulus. Transection of the aortic valve leaflets revealed a diffusely calcified aortic valve annulus with deposits of black carbonaceous material (Figure 3 ). The aortic valve was subsequently sent for pathological evaluation, which was remarkable for multiple black calcified lesions up to 0.5 cm in dimension, and involved approximately 15% of the leaflet surface (Figure 4 ).
Due to his pre-existing significant mobility limitation, the patient was discharged to an acute rehabilitation facility on postoperative day 11. The patient returned for routine 1 and 6-month follow-up with no complaints, and echocardiographic assessment revealed adequate contractility and bioprosthetic valve function. Follow-up 20 months after surgery revealed adequate ejection fraction (65%) and normal bioprosthetic valve function and gradients.