PMC4717593_01
a Aortography showing a focal, hourglass-type supra-valvular aortic stenosis with normal morphology of aortic arch and descending thoracic aorta. b Three-dimensional reconstruction imaging of computed tomography showing the spatial and anatomical relationship of the stenotic aortic root and coronary orifices
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Fig1
Aortography showed a focal, hourglass-type supra-valvular aortic stenosis with normal morphology of aortic arch and descending thoracic aorta (Fig. 1a, Additional file 1). Electrocardiogram-gated computed tomographic aortography revealed the more accurate spatial and anatomical relationship of the stenotic ascending aorta and aortic root including the orifice of coronary arteries (Fig. 1b).
Intraoperative exploration showing a severe stenotic change with thickening of the ascending aortic wall (a, arrow) and the aneurysmal change (mural thrombus was not presented because of evacuation with suction device) of the left ventricular apex (b, arrow). Postoperative 1-year, a three-dimensional computed tomography reconstruction showed the successful surgical repair of stenotic ascending aorta using an aortic valve graft and newly perceived disease of the innominate artery (c)
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Fig2
Longitudinal aortotomy revealed severe stenotic wrinkling change of ascending aorta just above the dilated orifice of both coronary arteries without discrete sinus of Valsalva (Fig. 2a). Separate left ventricular apical incision revealed extremely thin walled transmural scar with an intraventricular clot (Fig. 2b). Postoperative 1-year follow-up computed tomographic aortography revealed good contour of prosthetic materials located on the ascending aorta (Fig. 2c).
Serial review of perioperative imaging data revealed a subtle change of the innominate artery orifice (a, b, arrow) with small caliber of descending aorta (c)
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Fig3
However, a slightly aggravated stenotic lesion on innominate artery orifice and shrinkage of the entire abdominal aorta were found (Fig. 3).