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.