Jacques A. M. van Son, MD, PhD, William D. Edwards, MD, Gordon K

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Presentation transcript:

Pathology of coronary arteries, myocardium, and great arteries in supravalvular aortic stenosis Jacques A.M. van Son, MD, PhD, William D. Edwards, MD, Gordon K. Danielson, MD The Journal of Thoracic and Cardiovascular Surgery Volume 108, Issue 1, Pages 21-28 (July 1994) DOI: 10.5555/uri:pii:S0022522394702136 Copyright © 1994 Mosby, Inc. Terms and Conditions

Fig. 1 Schematic diagram of discrete and diffuse forms of supravalvular aortic stenosis. The Journal of Thoracic and Cardiovascular Surgery 1994 108, 21-28DOI: (10.5555/uri:pii:S0022522394702136) Copyright © 1994 Mosby, Inc. Terms and Conditions

Fig. 2 Gross pathology of supravalvular aortic stenosis. A and B, Discrete form shows external constriction (A, arrows) and internal thickening at level of aortics inotubular junction (B, *). Note focal fusion of aortic cusp to junctional ridge (arrow). C. Diffuse form exhibits marked thickening of aortic wall and walls of brachiocephalic branches. Asc Ao, Ascending aorta; AS, aortic sinus; LV, left ventricle; PT, pulmonary trunk; SVC, superior vena cava. The Journal of Thoracic and Cardiovascular Surgery 1994 108, 21-28DOI: (10.5555/uri:pii:S0022522394702136) Copyright © 1994 Mosby, Inc. Terms and Conditions

Fig. 3 Photomicrographs of coronary arteries in supravalvular aortic stenosis. A. Normal coronary artery, for comparison. B. Distinct intima (Int), media (Med), and adventitia (Adv), with thickening of all three layers. C. Severe luminal obstruction caused by wall thickening, with loss of internal elastic membrane, loss of distinction between intima and media, and disorganized (dysplastic) intimal-medial proliferation. D. Severe stenosis caused by dysplasia and atherosclerosis in 39-year-old man. E. Transition from three distinct arterial layers (right) into thickened and disorganized wall with loss of distinction between intima and media (left). F. Arterial dysplasia associated with focal acute intimal tear (*) and minor intramedial dissection (arrow). (Elastic-van Gieson; A, ×35; B and C,×30; D, ×15; E, ×60; F, ×20.) The Journal of Thoracic and Cardiovascular Surgery 1994 108, 21-28DOI: (10.5555/uri:pii:S0022522394702136) Copyright © 1994 Mosby, Inc. Terms and Conditions

Fig. 3 Photomicrographs of coronary arteries in supravalvular aortic stenosis. A. Normal coronary artery, for comparison. B. Distinct intima (Int), media (Med), and adventitia (Adv), with thickening of all three layers. C. Severe luminal obstruction caused by wall thickening, with loss of internal elastic membrane, loss of distinction between intima and media, and disorganized (dysplastic) intimal-medial proliferation. D. Severe stenosis caused by dysplasia and atherosclerosis in 39-year-old man. E. Transition from three distinct arterial layers (right) into thickened and disorganized wall with loss of distinction between intima and media (left). F. Arterial dysplasia associated with focal acute intimal tear (*) and minor intramedial dissection (arrow). (Elastic-van Gieson; A, ×35; B and C,×30; D, ×15; E, ×60; F, ×20.) The Journal of Thoracic and Cardiovascular Surgery 1994 108, 21-28DOI: (10.5555/uri:pii:S0022522394702136) Copyright © 1994 Mosby, Inc. Terms and Conditions

Fig. 3 Photomicrographs of coronary arteries in supravalvular aortic stenosis. A. Normal coronary artery, for comparison. B. Distinct intima (Int), media (Med), and adventitia (Adv), with thickening of all three layers. C. Severe luminal obstruction caused by wall thickening, with loss of internal elastic membrane, loss of distinction between intima and media, and disorganized (dysplastic) intimal-medial proliferation. D. Severe stenosis caused by dysplasia and atherosclerosis in 39-year-old man. E. Transition from three distinct arterial layers (right) into thickened and disorganized wall with loss of distinction between intima and media (left). F. Arterial dysplasia associated with focal acute intimal tear (*) and minor intramedial dissection (arrow). (Elastic-van Gieson; A, ×35; B and C,×30; D, ×15; E, ×60; F, ×20.) The Journal of Thoracic and Cardiovascular Surgery 1994 108, 21-28DOI: (10.5555/uri:pii:S0022522394702136) Copyright © 1994 Mosby, Inc. Terms and Conditions

Fig. 3 Photomicrographs of coronary arteries in supravalvular aortic stenosis. A. Normal coronary artery, for comparison. B. Distinct intima (Int), media (Med), and adventitia (Adv), with thickening of all three layers. C. Severe luminal obstruction caused by wall thickening, with loss of internal elastic membrane, loss of distinction between intima and media, and disorganized (dysplastic) intimal-medial proliferation. D. Severe stenosis caused by dysplasia and atherosclerosis in 39-year-old man. E. Transition from three distinct arterial layers (right) into thickened and disorganized wall with loss of distinction between intima and media (left). F. Arterial dysplasia associated with focal acute intimal tear (*) and minor intramedial dissection (arrow). (Elastic-van Gieson; A, ×35; B and C,×30; D, ×15; E, ×60; F, ×20.) The Journal of Thoracic and Cardiovascular Surgery 1994 108, 21-28DOI: (10.5555/uri:pii:S0022522394702136) Copyright © 1994 Mosby, Inc. Terms and Conditions

Fig. 3 Photomicrographs of coronary arteries in supravalvular aortic stenosis. A. Normal coronary artery, for comparison. B. Distinct intima (Int), media (Med), and adventitia (Adv), with thickening of all three layers. C. Severe luminal obstruction caused by wall thickening, with loss of internal elastic membrane, loss of distinction between intima and media, and disorganized (dysplastic) intimal-medial proliferation. D. Severe stenosis caused by dysplasia and atherosclerosis in 39-year-old man. E. Transition from three distinct arterial layers (right) into thickened and disorganized wall with loss of distinction between intima and media (left). F. Arterial dysplasia associated with focal acute intimal tear (*) and minor intramedial dissection (arrow). (Elastic-van Gieson; A, ×35; B and C,×30; D, ×15; E, ×60; F, ×20.) The Journal of Thoracic and Cardiovascular Surgery 1994 108, 21-28DOI: (10.5555/uri:pii:S0022522394702136) Copyright © 1994 Mosby, Inc. Terms and Conditions

Fig. 3 Photomicrographs of coronary arteries in supravalvular aortic stenosis. A. Normal coronary artery, for comparison. B. Distinct intima (Int), media (Med), and adventitia (Adv), with thickening of all three layers. C. Severe luminal obstruction caused by wall thickening, with loss of internal elastic membrane, loss of distinction between intima and media, and disorganized (dysplastic) intimal-medial proliferation. D. Severe stenosis caused by dysplasia and atherosclerosis in 39-year-old man. E. Transition from three distinct arterial layers (right) into thickened and disorganized wall with loss of distinction between intima and media (left). F. Arterial dysplasia associated with focal acute intimal tear (*) and minor intramedial dissection (arrow). (Elastic-van Gieson; A, ×35; B and C,×30; D, ×15; E, ×60; F, ×20.) The Journal of Thoracic and Cardiovascular Surgery 1994 108, 21-28DOI: (10.5555/uri:pii:S0022522394702136) Copyright © 1994 Mosby, Inc. Terms and Conditions

Fig. 4 Photomicrograph of myocardium in supravalvular aortic stenosis with coronary artery obstruction. Chronic ischemic changes include replacement fibrosis (left) and sarcoplasmic vacuolization of myocytes (right) from mitral papillary muscle. (Hematoxylin and eosin; ×200.) The Journal of Thoracic and Cardiovascular Surgery 1994 108, 21-28DOI: (10.5555/uri:pii:S0022522394702136) Copyright © 1994 Mosby, Inc. Terms and Conditions

Fig. 5 Photomicrograph of ascending aorta in supravalvular aortic stenosis. A, Marked disorganization and loss of elastic elements. B, Normal tissue, from distal portion of descending thoracic aorta in same patient, for comparison (Elastic-van Gieson; ×260.) The Journal of Thoracic and Cardiovascular Surgery 1994 108, 21-28DOI: (10.5555/uri:pii:S0022522394702136) Copyright © 1994 Mosby, Inc. Terms and Conditions