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11/13. Early aortic valve closure in combined idiopathic hypertrophic subaortic stenosis and discrete subaortic stenosis.

    A patient with idiopathic hypertrophic subaortic stenosis (90 mm Hg resting intraventricular gradient) and discrete subaortic stenosis was found to have two separate systolic closing movements of the aortic valve on M mode echocardiography, each movement being associated with a separate systolic ejection murmur. The first closing movement occurred early in systole and was attributed to alterations in flow across the discrete stenosis. The second closing movement coincided with a later systolic murmur and systolic anterior motion of the mitral valve. The case supports the concept that timing of early closing movements of the aortic valve is useful in the diagnosis of subvalvular obstruction.
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12/13. Aortic regurgitation due to non-traumatic rupture of the aortic valve commissures: report of two cases.

    Two cases of severe aortic regurgitation due to non-traumatic rupture of the aortic valve commissures are reported. The cause of rupture was hypertension in one patient, but it could not be identified in the other, where microscopic examinations of the aortic wall and the aortic cusps showed no particular pathologic changes. M-mode echocardiography revealed enlargement of the left ventricle, diastolic flutter of the anterior mitral leaflet and diastolic separation of the closure line of the aortic cusps in both patients. Two-dimensional echocardiography showed a downward displacement of the prolapsing motion of the aortic valve cusp during diastole toward the left ventricular outflow tract in one patient, and eccentricity of the coaptation point of the aortic valve without thickening of the cusps in the other. In addition to clinical features of progressive heart failure and characteristic cardiac murmur, echocardiographic studies provided correct diagnosis of aortic valve prolapse resulting from rupture of the aortic valve commissures. Both patients underwent aortic valve replacement successfully.
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13/13. Aortic root replacement for takayasu arteritis associated with ulcerative colitis and ankylosing spondylitis--report of a case.

    A 26-year-old man was admitted for treatment of congestive heart failure resulting from aortic regurgitation. The patient had been on medical treatment for ulcerative colitis (UC) since he was 14 years old and for ankylosing spondylitis (AS) since he was 20 years old. On admission, gradients of blood pressure among the extremities were observed. echocardiography revealed marked dilation of the left ventricle (LV), hypokinetic wall motion of the LV, slightly prolapsed aortic cusps with annular dilatation, and severe aortic regurgitation. Computed tomographic scans demonstrated an aneurysmal dilation of the ascending aorta and thickening of the descending and abdominal aortic wall. Digital subtraction angiography demonstrated an aneurysmal dilation of the ascending aorta; however, there was no clear evidence of steno-occlusive lesions in the brachiocephalic vessels. Blood studies showed positive inflammatory signs and negative rheumatoid factor. HLA typing showed A2, 24(9), B27, 67, Cw1, 7, and DR1, 2. Based on these data, the diagnosis of takayasu arteritis associated with UC and AS was made. Aortic root replacement was performed. Steroid therapy was restarted immediately after surgery. Histologic studies of the aortic wall showed findings compatible with takayasu arteritis. The combination of these rare diseases suggests that they have a common pathophysiologic background.
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