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Echocardiographic manifestations of annuloaortic ectasia: Its “paradoxical” motion of the aorta and premature systolic closure of the aortic valve
Echocardiographic manifestations of annuloaortic ectasia: Its "paradoxical" motion of the aorta and premature systolic closure of the aortic valve Yoshihiko Atsuchi, M.D. Yasuo Nagai, M.D. Yukio K o m a t s u , M.D. Kenji N a k a m u r a , M.D. Minoru Shibuya, M.D. Koshichiro Hirosawa, M.D. Tokyo, Japan
Annulo-aortic ectasia is a m o r p h o l o g i c a l t e r m which designates a lesion with a d i l a t a t i o n of the a n n u l u s of the aortic valve a n d fusiform a n e u r y s m of the ascending a o r t a t e r m i n a t i n g p r o x i m a l to the origin of the i n n o m i n a t e artery. 1 T h e basic histological lesion is cystic medial necrosis as previously described by E r d h e i m . ~ a n d is acc o m p a n i e d by o t h e r s o m a t i c s t i g m a s of M a r f a n ' s s y n d r o m e in m o s t cases. 3 T h e r e are only a few echocardiographic r e p o r t s on a n n u l o - a o r t i c ectasia a n d t h e y m e r e l y describe dilatation of the aortic roo~ as a m a n i f e s t a t i o n . 4-6 We p e r f o r m e d echocardiographic studies on patients with a n n u l o - a o r t i c ectasia a n d found not only dilatation of the aortic root b u t also u n u s u a l m o t i o n of the aortic wall a n d aortic valve. Subjects and methods T h e r e were 12 subjects. Eleven of t h e m exhibited skeletal a n d / o r o p h t h a l m i c findings of M a r f a n ' s syndrome. One lacked b o t h and was t h o u g h t to h a v e f o r m e fruste. 7 T e n were m a l e and two were female. T h e i r ages ranged from 10 to 55 years, the m e a n age being 33 years. T h e existence of a n n u l o - a o r t i c ectasia in all cases was proved by a n g i o c a r d i o g r a p h y a n d / o r surgery (Fig. 1). As control groups. 20 n o r m a l subjects (22 to 36 years. From the Section of Medical Cardiology, The Heart Institute Japan. Tokyo Women's Medical College, Tokyo, Japan. Received for publication Oct. 30. 1975. Reprint requests: Yoshihiko Atsuchi. M.D.. Medical Cardiology. The Heart Institute Japan, I0 Kawada-cho. Shinjuku-ku. Tokyo. Japan 162.
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m e a n age 31), a n d 16 p a t i e n t s w i t h r h e u m a t i c aortic regurgitation (18 to 44 years, m e a n age 36) were selected. Echocardiographic e q u i p m e n t was a c o m m e r cially available Ultrasonic Cardiograph, Model WM-09 m a d e by Sanei-Sokki, and a 2.25 M H z . t r a n s d u c e r 12 m m . in diameter. T h e t e c h n i q u e o f recording the e c h o c a r d i o g r a m of the aortic and mitral valve a n d the m e t h o d of M - m o d e scan have been described previously. 8' 9 T h e aortic root d i a m e t e r was m e a s u r e d f r o m the a n t e r i o r edge of the anterior aortic wall echo to the a n t e r i o r edge of the posterior aortic wall echo a t the end of ventricular diastole w h e n the aortic valve first appeared in the aortic root while scanning between the m i t r a l and aortic root. This m e t h o d was adopted because of variable m e a s u r e m e n t values of the aortic root diameter, especially in annulo-aortic ectasia, w h e n the t r a n s d u c e r is tilted in a cephalad direction. Results A o r t i c root d i a m e t e r . T h e aortic root d i a m e t e r at t h e end of diastole was 2.9 _+ 0.35 cm. (range, 2.5 to 3.5 cm.) in the n o r m a l subjects, 3.6 • 0.37 cm. (range, 3.0 to 4.3 cm.) in aortic regurgitation, and 4.2 • 0.52 cm. (range 3.2 to 5.0 cm.) in annulo-aortic ectasiao T h e values were statistically significant for each group (p < 0.005). M o t i o n of aortic root. In the control groups b o t h the a n t e r i o r and p o s t e r i o r walls showed almost parallel f o r w a r d m o t i o n a f t e r the maxim u m opening of the aortic valve and c o n t i n u e d to
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Fig. 1. Angiocardiograms show pear-shaped dilatation of the ascending aorta and aortic regurgitation jet. Left; anteroposterior view; right; lateral view.
Fig. 2. Rheumatic aortic regurgitation. Both anterior (AW) and posterior (PW) walls show a parallel forward motion after the maximum opening of the aortic valve leaflets (Am). The onset of the opening (Ao) is coincident on the R wave of the ECG ("early opening"). Diastolic cusp separation (S) is observed. The distance between two consecutive vertical and horizontal dots equals 1 cm. and 1 second, respectively.
do so for a w h i l e a f t e r t h e a o r t i c v a l v e c l o s e d (Fig. 2). H o w e v e r , in a n n u l o - a o r t i c e c t a s i a , v e r y u n u s u a l m o t i o n o f t h e w a l l w a s o b s e r v e d ( F i g . 3). The posterior wall exhibited backward motion even a f t e r t h e m a x i m u m o p e n i n g o f t h e a o r t i c valve, a n d t h i s c o n t i n u e d u n t i l m i d d l e e j e c t i o n . T h i s m o t i o ~ is a p p a r e n t l y o p p o s i t e t o t h a t o f t h e
American Heart Journal
control groups and appears "paradoxical." This t y p e of " p a r a d o x i c a l " m o t i o n w a s o b s e r v e d in e i g h t cases. T h e r e m a i n i n g c a s e s s h o w e d a l m o s t " p a r a l l e l " m o t i o n , a l t h o u g h in t h r e e o f f o u r c a s e s the posterior wall appeared somewhat flat when c o m p a r e d w i t h t h e a n t e r i o r wall. Fig. 4 is a n e c h o c a r d i o g r a m of t h i s s a m e p a t i e n t a f t e r sur-
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Fig. 3. Annulo-aortic ectasia. The posterior wall of the aortic root shows backward ("paradoxical") motion during early to middle ejection period. The aortic valve leaflets show premature systolic closure (C), "early opening," and diastolic cusp separation. See Fig. 2 for abbreviations.
Fig. 4. Postoperative echogram. Abnormal motion is no longer present. Both anterior and posterior walls show a parallel forward motion. Prosthetic valve opens appreciably after the QRS of the ECG. See Fig. 2 for abbreviations.
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gical correction by the m e t h o d of Singh and Bentall2 ~ T h e a b n o r m a l systolic m o t i o n is no longer present and b o t h anterior and posterior walls show parallel forward motion. T h e aortic root diameter revealed more dilatation in the cases of " p a r a d o x i c a l " types t h a n "parallel" types, and values were statistically significant (p < 0.005) (Fig. 5). Aortic valve. T h e aortic valve leaflets opened rapidly with the onset of systole, and t h e n exhibited a b r u p t p r e m a t u r e partial closure immediately after ventricular ejection (Fig. 3). T h e closure occurred consistently right after the cusps opening in all cases and was never seen during middle or late systole. T h e onset of the opening of the a b n o r m a l valve is almost coincident on the R waves of the electrocardiogram and reached a fully open position at the J point of the QRS. A similar opening was also observed in r h e u m a t i c aortic regurgitation (Fig. 2). Usually, the aortic valve opens at an average of 112 msec. after the Q wave~1; this is a p p a r e n t l y "early opening." T h e postoperative echocardiogram (Fig. 4) shows t h a t the Bj6rk-Shiley prosthetic valves reached a fully open position appreciably after the QRS. T h e "early opening" was in all b u t one instance with a comparatively small aortic valve a n n u l u s diameter and where aortic regurgitation and diastolic cusp separation have n o t been recognized yet. Atrioventricular valves. T h e r e were no specific findings for annulo-aortic ectasia. Diastolic fluttering of the mitral valve was observed in four cases, and systolic prolapse of the m i t r a l valve in one case. T h e r e was no a b n o r m a l m o t i o n in the tricuspid valve.
Discussion In annulo-aortic ectasia the aortic root diameters showed a r e m a r k a b l e dilatation and t h e y exceeded t h a t of the r h e u m a t i c regurgitation group. When the aortic root d i a m e t e r measures more t h a n 4.0 cm., it is necessary to p a y a t t e n t i o n to the motion of the aortic wall and aortic valve leaflets and to suspect annulo-aortic ectasia. We assume t h a t histological changes of the aortic wall greatly c o n t r i b u t e to the cause of abnormal motion of the aortic wall. It is a wellknown fact t h a t cystic medial necrosis of the aortic wall is f r e q u e n t l y associated with Marfan's syndrome, 3 resulting in a decreasing of the elasticity of the wall. Consequently, the aortic wall expansion in annulo-aortic ectasia appears to be
American Heart Journal
cm
P< 0.005
6 L
.%
O
-0"0-
2
Parallel Paradoxical n=4
n=8
Fig. 5. Aortic root diameter vs. aortic wall motion. "Paradoxical" type shows a more dilated diameter than "parallel" type. AO = aortic root. more influenced by the blood flow a n d / o r t u r b u lence a r o u n d the markedly dilated sinus of Valsalva t h a n a normal aortic wall. Yuste and associates 12 reported an echocardiogram with dissecting a n e u r y s m of the ascending a o r t a in which the posterior wall exhibited p o s t e r i o r motion during early to middle systole. E v e n though the etiology is different, this p h e n o m e n o n is similar to t h a t of annulo-aortic ectasia from the viewpoint of a decreasing of elasticity of the aortic wall. Assuming t h a t the a b n o r m a l m o t i o n of aortic wall is caused by the above-mentioned factor, the cases t h a t now present "parallel" type have the possibility of showing "paradoxical" type with the progression of the histological changes and a decreasing of elasticity of t h e aortic wall. T h e p r e m a t u r e systolic partial closure of the aortic valve is t h o u g h t to be caused b y a completely different h e m o d y n a m i c change as seen in idiopathic h y p e r t r o p h i c subaortic stenosis 13 or discrete subaortic stenosis 14 which has a dynamic obstruction in the left ventricular outflow tract. Recently, C h a n d r a r a t n a and associates 1~ reported a similar closing m o t i o n in a
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p a t i e n t w i t h a "floppy" aortic valve. T h i s f a c t m a y suggest t h a t the closure is due to the redund a n t aortic valve leaflets f r e q u e n t l y associated with M a r f a n ' s syndrome. However, we a n d o t h e r a u t h o r s observed such a closure in the p a t i e n t s with r u p t u r e d 16 a n d even u n r u p t u r e d sinus of Valsalva a n e u r y s m ; a d e f o r m i t y of a n e u r y s m a l dilatation of the sinus of V a l s a l v a also seems to be c o m p o u n d e d b y either the lower pressure s y s t e m or a m o r e a u g m e n t e d " p u l l - b a c k " m e c h a n i s m . T h e m e c h a n i s m of "early opening" of the aortic valve is far conclusive. Since the electrical systolic events precede the m e c h a n i c a l systolic events, the aortic valve n o r m a l l y opens a f t e r t h e QRS. H o w ever, in the p a t i e n t s with a n n u l o - a o r t i c ectasia and in some cases of r h e u m a t i c aortic regurgitation this u n u s u a l m o t i o n was observed. V e r y similar m o t i o n is also observed in the echocardiog r a m r e p o r t e d b y D e M a r i a a n d associates ~ a n d Feigenbaum2 The common echocardiographic findings in all cases is the presence of the diastolic cusp s e p a r a t i o n which has been described as one of the findings of aortic r e g u r g i t a t i o n " ; an incomplete c o a p t a t i o n of the aortic valve leaflets d u r i n g diastole m a y be one of the c o n t r i b u t i n g factors. This is only speculation t h a t awaits definitive explanation. We could n o t obtain findings specific to annulo-aortic ectasia f r o m the m o t i o n o f t h e a t r i o v e n t r i c u l a r valves. An interesting point is t h e incidence of systolic prolapse of the m i t r a l valve. Brown a n d associates is r e p o r t e d t h a t a m o n g 35 cases with M a r f a n ' s s y n d r o m e , t h e y f o u n d a leafl e t prolapse in 32 cases. On the c o n t r a r y , we recorded only one case a m o n g 12. This discrepancy is p r o b a b l y due to a different left v e n t r i c u l a r volume. F o n t a n a and associates I~ s p e c u l a t e d t h a t the degree of m i t r a l valve prolapse is closely related to the left v e n t r i c u l a r end-diastolic volume, a n d an increase of v e n t r i c u l a r v o l u m e would p r o d u c e m o r e tension on the m i t r a l v a l v e leaflets and chordae by increasing the distance between the v e n t r i c u l a r wall a n d p a p i l l a r y muscles and t h e valve ring, and l a t e r a n d less leaflet prolapse would t h e n result during systole. W h e n this h e m o d y n a m i c m e c h a n i s m is applied to a n n u l o - a o r t i c ectasia with a m a r k e d l y dilated left ventricle due to severe aortic regurgitation, t h e end-diastolic v o l u m e is increased and, therefore, the degree of m i t r a l valve prolapse decreases a n d possibly disappears as a n n u l o - a o r t i c ectasia progresses. T h e only case with a prolapsing m i t r a l
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valve is the one with a c o m p a r a t i v e l y s m a l l aortic valve a n n u l u s where aortic r e g u r g i t a t i o n has n o t been recognized yet, a n d in addition, we h a v e found a m i t r a l valve prolapse in f o u r out of five o t h e r cases of M a r f a n ' s s y n d r o m e which were n o t included in this s t u d y because of t h e absence of a n n u l o - a o r t i c ectasia. In conclusion, our d a t a indicate t h a t echocardio g r a p h y provides a reliable, sensitive, and practical noninvasive m e a n s of a c c u r a t e l y diagnosing annulo-aortic ectasia w i t h a b n o r m a l m o t i o n of the aortic wall and aortic valve.
Summary T h e echocardiographic f e a t u r e s of annuloaortic ectasia were studied in 12 patients. Eleven of t h e m exhibited skeletal a n d / o r o p h t h a l m i c findings of M a r f a n ' s s y n d r o m e a n d one was considered as having forme fruste. Echocardiog r a m s revealed not only m a r k e d d i l a t a t i o n of the aortic root b u t also unique m o t i o n of the aortic wall a n d aortic valve. Posterior m o t i o n of the posterior aortic wall during early to middle ejection period, i.e., " p a r a d o x i c a l " m o t i o n , was n o t e d in eight cases, a n d p r e m a t u r e systolic p a r t i a l closure of the aortic valve was seen in all cases. The authors express their gratitude to Mrs. Fumiko Adachi for her skillful technical assistance.
REFERENCES 1. Ellis, P. R., Cooley, D. A., and De Bakey, M. E.: Clinical considerations and surgical treatment of annulo-aortic ectasia. Report of successful operation, J. Thorac. Cardiovasc. Surg. 42:363, 1961. 2. Erdheim, J.: Medionecrosis aortae idiopathica cystica, Virchows Arch. Pathol. Anat. 276:187,1930. 3. Chapman, D. W., Beazley, H. L., Peterson, P. K., Webb, J. A., and Colley, D. A.: Annulo-aortic ectasia with cystic medial necrosis. Diagnosis and surgical treatment, Am. J. Cardiol. 16:679, 1965. 4. Kronzon, I., Weisinger, B., and Glassman, E.: Cystic medial necrosis with severe aortic root dilatation, Chest 66:79, 1974. 5. DeMaria, A. N., King, J. F., Salel, A. F., Caudill, C. C,, Miller, R. R., and Mason, D. T.: Echography and phonography of acute aortic regurgitation in bacterial endocarditis, Ann: Intern. Med. 82:329, 1975. 6. Winsberg, F.: Aortic valve, in Gramiak, R., and Waag, R. C., Editors: Cardiac ultrasound, St Louis, 1975, The C. V. Mosby Company, p. 84. 7. Golden, R. L., and Lochin, H.: The forme fruste in Marfan's syndrome, N. Engl. J. Med. 260:797, 1957. 8. Gramick, R., and Shah, P. M.: Echocardiography of the normal and diseased aortic valve, Radiology 96:1, 1970. 9. Feigenbaum, H.: Echocardiography, Philadelphia, 1972, Lea & Febiger, Publishers, pp. 25, 86. 10. Shlgh, M. P., and Bentall, H. H.: Complete replacement of the ascending aorta and the aortic valve for the
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11.
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13. 14.
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treatment of aortic aneurysm, J. Thorac. Cardiovasc. Surg. 63:218, 1972. Sakamoto, T., Matsuhisa, M., and Hayashi, T.: Echocardiographic and phonocardiographic measurement of systolic time intervals, Cardiovasc. Sound Bull. 5:63, 1975. Yuste, P., Aza, V., Minguez, I., Cerezo, L., and MartinezBordiu, C.: Dissecting aortic aneurysm diagnosed by echocardiography. A pre- and postoperative study, Br. Heart J. 36:111, 1974. Tajik, A. J., and Giulliani, E. R.: Echocardiographic observations in idiopathic hypertrophic subaortic stenosis, Mayo Ciin. Proc. 49:89, 1974. Davis, R. H., Feigenbaum, H., Chang, S., Konecke, L. L., and Dillon, J. C.: Echocardiographic manifestations of discrete subaortic stenosis, Am. J. Caridol. 33:277, 1974. Chandraratna, P. A. N., Samet, P., Robinson, M. J., and
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16.
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19.
Byrd, C.: Echocardiography of the "floppy" aortic valve. Report of a case, Circulation 52:959, 1975. Johnson, M. L., Warren, S. G., Waugh, R. A., Kisslo, J. A., Sabiston, D. C., and Lester , R. G.: Echocardiography of the aortic yalve inJnon-rheumatic left ventricular outflow tract lesions, Radiology 1 12:677, 1974. Feizi, 6., Symons, C., and Yac~oub, M.: Echocardiography of the aortic valve. Studies of normal aortic valve, aortic stenosis, aortic regurgitation, and mixed aortic valve disease, Br. Heart J. 36:341, 1974. Brown, 0. R., DeMots, H., Kloster, F. E., Roberts, A., Menashe, V. D., and Beals, R. K.: Aortic root dilatation and mitral valve prolapse in Marfan's syndrome: An echocardiographic study, Circulation 52:651, 1975. Fontana, M. E., Wooley, C. F., Leighton, R. F., and Lewis, R. P.: Postural changes in left ventricular and mitral valvular dynamics in the systolic click-late murmur syndrome, Circulation 51:165, 1975.
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Annulo-aortic ectasia is a m o r p h o l o g i c a l t e r m which designates a lesion with a d i l a t a t i o n of the a n n u l u s of the aortic valve a n d fusiform a n e u r y s m of the ascending a o r t a t e r m i n a t i n g p r o x i m a l to the origin of the i n n o m i n a t e artery. 1 T h e basic histological lesion is cystic medial necrosis as previously described by E r d h e i m . ~ a n d is acc o m p a n i e d by o t h e r s o m a t i c s t i g m a s of M a r f a n ' s s y n d r o m e in m o s t cases. 3 T h e r e are only a few echocardiographic r e p o r t s on a n n u l o - a o r t i c ectasia a n d t h e y m e r e l y describe dilatation of the aortic roo~ as a m a n i f e s t a t i o n . 4-6 We p e r f o r m e d echocardiographic studies on patients with a n n u l o - a o r t i c ectasia a n d found not only dilatation of the aortic root b u t also u n u s u a l m o t i o n of the aortic wall a n d aortic valve. Subjects and methods T h e r e were 12 subjects. Eleven of t h e m exhibited skeletal a n d / o r o p h t h a l m i c findings of M a r f a n ' s syndrome. One lacked b o t h and was t h o u g h t to h a v e f o r m e fruste. 7 T e n were m a l e and two were female. T h e i r ages ranged from 10 to 55 years, the m e a n age being 33 years. T h e existence of a n n u l o - a o r t i c ectasia in all cases was proved by a n g i o c a r d i o g r a p h y a n d / o r surgery (Fig. 1). As control groups. 20 n o r m a l subjects (22 to 36 years. From the Section of Medical Cardiology, The Heart Institute Japan. Tokyo Women's Medical College, Tokyo, Japan. Received for publication Oct. 30. 1975. Reprint requests: Yoshihiko Atsuchi. M.D.. Medical Cardiology. The Heart Institute Japan, I0 Kawada-cho. Shinjuku-ku. Tokyo. Japan 162.
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m e a n age 31), a n d 16 p a t i e n t s w i t h r h e u m a t i c aortic regurgitation (18 to 44 years, m e a n age 36) were selected. Echocardiographic e q u i p m e n t was a c o m m e r cially available Ultrasonic Cardiograph, Model WM-09 m a d e by Sanei-Sokki, and a 2.25 M H z . t r a n s d u c e r 12 m m . in diameter. T h e t e c h n i q u e o f recording the e c h o c a r d i o g r a m of the aortic and mitral valve a n d the m e t h o d of M - m o d e scan have been described previously. 8' 9 T h e aortic root d i a m e t e r was m e a s u r e d f r o m the a n t e r i o r edge of the anterior aortic wall echo to the a n t e r i o r edge of the posterior aortic wall echo a t the end of ventricular diastole w h e n the aortic valve first appeared in the aortic root while scanning between the m i t r a l and aortic root. This m e t h o d was adopted because of variable m e a s u r e m e n t values of the aortic root diameter, especially in annulo-aortic ectasia, w h e n the t r a n s d u c e r is tilted in a cephalad direction. Results A o r t i c root d i a m e t e r . T h e aortic root d i a m e t e r at t h e end of diastole was 2.9 _+ 0.35 cm. (range, 2.5 to 3.5 cm.) in the n o r m a l subjects, 3.6 • 0.37 cm. (range, 3.0 to 4.3 cm.) in aortic regurgitation, and 4.2 • 0.52 cm. (range 3.2 to 5.0 cm.) in annulo-aortic ectasiao T h e values were statistically significant for each group (p < 0.005). M o t i o n of aortic root. In the control groups b o t h the a n t e r i o r and p o s t e r i o r walls showed almost parallel f o r w a r d m o t i o n a f t e r the maxim u m opening of the aortic valve and c o n t i n u e d to
April, 1977, Vol. 93, No. 4, pp. 428-433
Echo diagnosis of annulo-aortic ectasia
Fig. 1. Angiocardiograms show pear-shaped dilatation of the ascending aorta and aortic regurgitation jet. Left; anteroposterior view; right; lateral view.
Fig. 2. Rheumatic aortic regurgitation. Both anterior (AW) and posterior (PW) walls show a parallel forward motion after the maximum opening of the aortic valve leaflets (Am). The onset of the opening (Ao) is coincident on the R wave of the ECG ("early opening"). Diastolic cusp separation (S) is observed. The distance between two consecutive vertical and horizontal dots equals 1 cm. and 1 second, respectively.
do so for a w h i l e a f t e r t h e a o r t i c v a l v e c l o s e d (Fig. 2). H o w e v e r , in a n n u l o - a o r t i c e c t a s i a , v e r y u n u s u a l m o t i o n o f t h e w a l l w a s o b s e r v e d ( F i g . 3). The posterior wall exhibited backward motion even a f t e r t h e m a x i m u m o p e n i n g o f t h e a o r t i c valve, a n d t h i s c o n t i n u e d u n t i l m i d d l e e j e c t i o n . T h i s m o t i o ~ is a p p a r e n t l y o p p o s i t e t o t h a t o f t h e
American Heart Journal
control groups and appears "paradoxical." This t y p e of " p a r a d o x i c a l " m o t i o n w a s o b s e r v e d in e i g h t cases. T h e r e m a i n i n g c a s e s s h o w e d a l m o s t " p a r a l l e l " m o t i o n , a l t h o u g h in t h r e e o f f o u r c a s e s the posterior wall appeared somewhat flat when c o m p a r e d w i t h t h e a n t e r i o r wall. Fig. 4 is a n e c h o c a r d i o g r a m of t h i s s a m e p a t i e n t a f t e r sur-
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Fig. 3. Annulo-aortic ectasia. The posterior wall of the aortic root shows backward ("paradoxical") motion during early to middle ejection period. The aortic valve leaflets show premature systolic closure (C), "early opening," and diastolic cusp separation. See Fig. 2 for abbreviations.
Fig. 4. Postoperative echogram. Abnormal motion is no longer present. Both anterior and posterior walls show a parallel forward motion. Prosthetic valve opens appreciably after the QRS of the ECG. See Fig. 2 for abbreviations.
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gical correction by the m e t h o d of Singh and Bentall2 ~ T h e a b n o r m a l systolic m o t i o n is no longer present and b o t h anterior and posterior walls show parallel forward motion. T h e aortic root diameter revealed more dilatation in the cases of " p a r a d o x i c a l " types t h a n "parallel" types, and values were statistically significant (p < 0.005) (Fig. 5). Aortic valve. T h e aortic valve leaflets opened rapidly with the onset of systole, and t h e n exhibited a b r u p t p r e m a t u r e partial closure immediately after ventricular ejection (Fig. 3). T h e closure occurred consistently right after the cusps opening in all cases and was never seen during middle or late systole. T h e onset of the opening of the a b n o r m a l valve is almost coincident on the R waves of the electrocardiogram and reached a fully open position at the J point of the QRS. A similar opening was also observed in r h e u m a t i c aortic regurgitation (Fig. 2). Usually, the aortic valve opens at an average of 112 msec. after the Q wave~1; this is a p p a r e n t l y "early opening." T h e postoperative echocardiogram (Fig. 4) shows t h a t the Bj6rk-Shiley prosthetic valves reached a fully open position appreciably after the QRS. T h e "early opening" was in all b u t one instance with a comparatively small aortic valve a n n u l u s diameter and where aortic regurgitation and diastolic cusp separation have n o t been recognized yet. Atrioventricular valves. T h e r e were no specific findings for annulo-aortic ectasia. Diastolic fluttering of the mitral valve was observed in four cases, and systolic prolapse of the m i t r a l valve in one case. T h e r e was no a b n o r m a l m o t i o n in the tricuspid valve.
Discussion In annulo-aortic ectasia the aortic root diameters showed a r e m a r k a b l e dilatation and t h e y exceeded t h a t of the r h e u m a t i c regurgitation group. When the aortic root d i a m e t e r measures more t h a n 4.0 cm., it is necessary to p a y a t t e n t i o n to the motion of the aortic wall and aortic valve leaflets and to suspect annulo-aortic ectasia. We assume t h a t histological changes of the aortic wall greatly c o n t r i b u t e to the cause of abnormal motion of the aortic wall. It is a wellknown fact t h a t cystic medial necrosis of the aortic wall is f r e q u e n t l y associated with Marfan's syndrome, 3 resulting in a decreasing of the elasticity of the wall. Consequently, the aortic wall expansion in annulo-aortic ectasia appears to be
American Heart Journal
cm
P< 0.005
6 L
.%
O
-0"0-
2
Parallel Paradoxical n=4
n=8
Fig. 5. Aortic root diameter vs. aortic wall motion. "Paradoxical" type shows a more dilated diameter than "parallel" type. AO = aortic root. more influenced by the blood flow a n d / o r t u r b u lence a r o u n d the markedly dilated sinus of Valsalva t h a n a normal aortic wall. Yuste and associates 12 reported an echocardiogram with dissecting a n e u r y s m of the ascending a o r t a in which the posterior wall exhibited p o s t e r i o r motion during early to middle systole. E v e n though the etiology is different, this p h e n o m e n o n is similar to t h a t of annulo-aortic ectasia from the viewpoint of a decreasing of elasticity of the aortic wall. Assuming t h a t the a b n o r m a l m o t i o n of aortic wall is caused by the above-mentioned factor, the cases t h a t now present "parallel" type have the possibility of showing "paradoxical" type with the progression of the histological changes and a decreasing of elasticity of t h e aortic wall. T h e p r e m a t u r e systolic partial closure of the aortic valve is t h o u g h t to be caused b y a completely different h e m o d y n a m i c change as seen in idiopathic h y p e r t r o p h i c subaortic stenosis 13 or discrete subaortic stenosis 14 which has a dynamic obstruction in the left ventricular outflow tract. Recently, C h a n d r a r a t n a and associates 1~ reported a similar closing m o t i o n in a
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p a t i e n t w i t h a "floppy" aortic valve. T h i s f a c t m a y suggest t h a t the closure is due to the redund a n t aortic valve leaflets f r e q u e n t l y associated with M a r f a n ' s syndrome. However, we a n d o t h e r a u t h o r s observed such a closure in the p a t i e n t s with r u p t u r e d 16 a n d even u n r u p t u r e d sinus of Valsalva a n e u r y s m ; a d e f o r m i t y of a n e u r y s m a l dilatation of the sinus of V a l s a l v a also seems to be c o m p o u n d e d b y either the lower pressure s y s t e m or a m o r e a u g m e n t e d " p u l l - b a c k " m e c h a n i s m . T h e m e c h a n i s m of "early opening" of the aortic valve is far conclusive. Since the electrical systolic events precede the m e c h a n i c a l systolic events, the aortic valve n o r m a l l y opens a f t e r t h e QRS. H o w ever, in the p a t i e n t s with a n n u l o - a o r t i c ectasia and in some cases of r h e u m a t i c aortic regurgitation this u n u s u a l m o t i o n was observed. V e r y similar m o t i o n is also observed in the echocardiog r a m r e p o r t e d b y D e M a r i a a n d associates ~ a n d Feigenbaum2 The common echocardiographic findings in all cases is the presence of the diastolic cusp s e p a r a t i o n which has been described as one of the findings of aortic r e g u r g i t a t i o n " ; an incomplete c o a p t a t i o n of the aortic valve leaflets d u r i n g diastole m a y be one of the c o n t r i b u t i n g factors. This is only speculation t h a t awaits definitive explanation. We could n o t obtain findings specific to annulo-aortic ectasia f r o m the m o t i o n o f t h e a t r i o v e n t r i c u l a r valves. An interesting point is t h e incidence of systolic prolapse of the m i t r a l valve. Brown a n d associates is r e p o r t e d t h a t a m o n g 35 cases with M a r f a n ' s s y n d r o m e , t h e y f o u n d a leafl e t prolapse in 32 cases. On the c o n t r a r y , we recorded only one case a m o n g 12. This discrepancy is p r o b a b l y due to a different left v e n t r i c u l a r volume. F o n t a n a and associates I~ s p e c u l a t e d t h a t the degree of m i t r a l valve prolapse is closely related to the left v e n t r i c u l a r end-diastolic volume, a n d an increase of v e n t r i c u l a r v o l u m e would p r o d u c e m o r e tension on the m i t r a l v a l v e leaflets and chordae by increasing the distance between the v e n t r i c u l a r wall a n d p a p i l l a r y muscles and t h e valve ring, and l a t e r a n d less leaflet prolapse would t h e n result during systole. W h e n this h e m o d y n a m i c m e c h a n i s m is applied to a n n u l o - a o r t i c ectasia with a m a r k e d l y dilated left ventricle due to severe aortic regurgitation, t h e end-diastolic v o l u m e is increased and, therefore, the degree of m i t r a l valve prolapse decreases a n d possibly disappears as a n n u l o - a o r t i c ectasia progresses. T h e only case with a prolapsing m i t r a l
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valve is the one with a c o m p a r a t i v e l y s m a l l aortic valve a n n u l u s where aortic r e g u r g i t a t i o n has n o t been recognized yet, a n d in addition, we h a v e found a m i t r a l valve prolapse in f o u r out of five o t h e r cases of M a r f a n ' s s y n d r o m e which were n o t included in this s t u d y because of t h e absence of a n n u l o - a o r t i c ectasia. In conclusion, our d a t a indicate t h a t echocardio g r a p h y provides a reliable, sensitive, and practical noninvasive m e a n s of a c c u r a t e l y diagnosing annulo-aortic ectasia w i t h a b n o r m a l m o t i o n of the aortic wall and aortic valve.
Summary T h e echocardiographic f e a t u r e s of annuloaortic ectasia were studied in 12 patients. Eleven of t h e m exhibited skeletal a n d / o r o p h t h a l m i c findings of M a r f a n ' s s y n d r o m e a n d one was considered as having forme fruste. Echocardiog r a m s revealed not only m a r k e d d i l a t a t i o n of the aortic root b u t also unique m o t i o n of the aortic wall a n d aortic valve. Posterior m o t i o n of the posterior aortic wall during early to middle ejection period, i.e., " p a r a d o x i c a l " m o t i o n , was n o t e d in eight cases, a n d p r e m a t u r e systolic p a r t i a l closure of the aortic valve was seen in all cases. The authors express their gratitude to Mrs. Fumiko Adachi for her skillful technical assistance.
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