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Aneurysm of the aortic sinus of valsalva communicating with a fistulous cavity in the interventricular septum: Report of a case
CASE REPORT
ACKNO~,VLEDGMENTS T i m a t t t h o r s wish to t h a n k Mrs. W i n i fred Rinschler for p r e p a r i n g the m a n u script a n d Mrs. I d a N a t h a n f o r the pttotomicrographs.
REFERENCES 1. tlallgrimsson, J., Bj3rnsson, A., and (;udmundsson, G.: Meningioma of the neck. Case report. J. Ncurosurg., 32:695, 1970. 2. Suzuki, t!., Gilbert, E. F., and Zimmermann, B.: Primary extracranial meningioma. Arch. Path., 84:202, 1967. 3. Hoye, S. J., ttoar, C. S., and Murray, J. E.: Extracranial meningioma presenting as a tumor of the neck. Amer. J. Surg., 100:486, 1960. 4. Von der Zalm, H.: Extracranicel Menigeoom. Nederl. T. Geneesk., 108:492, 1964. 5. Dalforno, S., and Domm, A.: Neuroma Plessiforme della Parotide. Cancro, 22:529, 1969. 6. Foote, F. W., Jr., and Frazell, E. L.: Tumors of the Major Salivary Glands. AFIP Atlas of Tumor l'athology, Sect. IV., Fasc. 11., First Series. Washington, D.C., Armed Forces Institute of Pathology, 1954, p. 7. 7. Foote, F. W., Jr., and Frazell, E. L.: Tumors of the major salivary glands. Cancer, 6:1065, 1953.
8. Niccoli Vallesi, R.: Neurinoma della ghiandola sottolinguale. Arch. Vecchi Anat. Pat., 19:8t3, 1953. 9. Anson, B.J. (Editor): Morris' Human Anatomy. Ed. 12. New York, The Blakiston'Division, McGraw-Hill Book Company, 1966. 10. Schmidt, M. B.: Ueber die Pacchionischen Granulationen und ihr Verhaeltnis zu den Sarcomen und Psammomen der Dura mater. Virchows Arch. Path. Anat., 170:429, 1902. 11. Cushing, tt.: The meningiomas (dural endothelioma). Their source and favoured teats of origin. Brain, XLV (part 2):282, 1922. 12. Zachariae, L.: Case of extracranial primary meningioma. Acta l'ath. Microbiol. Scand., 31:57, 1952. 13. Willis, R. A.: The Borderland of Embryology and Pathology. Ed. 2. London, Butterworth & Co. (Publishers) Ltd., 1962. 14. Kernohan,J. W., and Sayre, G. P.: Tumors of the central nervous system: AFIP Atlas of Tumor Pathology, Scct. X, Fasc. 15. Washington, D.C., Armed Forces Institute of l'athology, 1952. 15. Aoyagi and Kytmo: Ueber die endothelien Zellzapfen in der Dura mater cerebri, and ihre Lokalisation in derselben. Neuroglia, H:I, 1912. Columbia University College of Physicians and Surgeons 630 West 168th Street New York, New York 10032
Case Report ANEURYSM OF THE AORTIC SINUS OF VALSALVA C O M M U N I C A T I N G WITH A FISTULOUS CAVITY IN THE INTERVENTRICULAR SEPTUM: REPORT OF A CASE HIROSHI NAKAZAWA,M.D.* LEOXARD S. BrieR, M.D.,t and SIGMUND "~VILENS,M.D.s+
Abstract An anemysm of the right corona U sinus is described that extended into large fi~tulous cavities of the ventricular septum and anterior wall of both *Assistant Clinical Professor of l'athology, Columbia University College of Physicians and Surgeons, New York. Assistant Director of I.aboratortes, Nyack ttospital, Nyack, New York. tAttending l'bysician, Department of Medicine, Nyack tlospital, Nyack, New York. ,+Professor of patlmlogy, Columbia University College of Physicians and Surgeons, New York, New York.
ventricles without perforation into the cardiac chamber. A review of the literature revealed no previous similar report. The present case suggests that medial necrosis plays a role ill the formation of sztch an atteuo'sm. A n e u r y s m s of the aortic sinus are relatively u n c o m m o n . T h e m a j o r i t y o f t h e m a r e c o n g e n i t a l a n d a few a r e mycotic? T h e c o n g e n i t a l a n e u r y s m o f t h e ' a o r tic s i n u s is o f t e n associated with a n i n t e r v e n t r i c u l a r septal defect2- T h e m y c o t i c a n e u r y s m m a y a p p e a r as a r e s u l t o f bacterial e n d o c a r d i t i s 2 L o c a t e d at t h e c o m m e n c e m e n t 'of the aorta, the pocket-like spaces f o r m e d by the aortic c u s p s a n d t h e a o r t i c wall h a v e slight d i l a t a t i o n s . T h e s e t h r e e d i l a t a t i o n s are called t h e s i n u s o f Valsalva, w h i c h s o m e t i m e s Ires a c o n g e n i tal w e a k n e s s o f tim tissne s t r t t c t n r e . W h e n s t r n c t u r a l w e a k n e s s is p r e s e n t , a s i n u s m a y be b u l g e d by the k i g h p r e s s u r e o f t h e a o r t a i n t o a low p r e s s u r e c l t a m b e r o f tlm h e a r t , r e s u l t i n g i n a d i v e r t i c n l a r a n e u r y s m . Tiffs
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H U M A N P A T H O L O G Y - V O L U M E 2, N U M B E R 3 c o n d i t i o n is o f t e n u n r e c o g n i z e d clinically. W h e n it r e a c h e s a certain size, it o f t e n r u p t n r e s into a c a r d i a c c h a m b e r , a c c o m p a n i e d f r e q u e n t l y b y the s u d d e n onset o f chest o r a b d o m i n a l pain. T h e p a t i e n t develops rapid cardiac decompensation a n d d e a t h ensues. A l t ! l o u g h this c o n d i t i o n h a d b e e n o n e o f t h e fatal c a r d i a c lesions in the past, it i s n o w a m e n a b l e to o p e n heart surgery. Recently we o b s e r v e d a 31 )'ear old m a n w h o h a d b e e n a s y m p t o m a t i c , with t h e e x c e p t i o n o f occasional palpitations, a n d w h o s u d d e n l y died. T h e p o s t m o r t e m e x a m i n a t i o n r e v e a l e d an a n e u r y s m o f the r i g h t c o r o n a r y sinus, w h i c h c o n n n u n i c a t e d with a large fistulous cavity in the interv e n t r i c u l a r s e p t u m e x t e n d i n g into the a n t e r i o r left v e n t r i c u l a r wall. T h e r e was n o e v i d e n c e o f p e r f o r a t i o n into a c a r d i a c chamber. T h e p r e s e n t p a p e r describes the gross a n d m i c r o s c o p i c findings o f this u n u s u a l case a l o n g with a b r i e f discussion o f the conditions.
460
A 31 year old married Negro tailor had recently been told that he had an enlarged heart. T h e x-ray film had been taken when he left Haiti to come to the United States. He admitted to occasional episodes of palpitations but was otherwise asymptomatic. He denied dyspnea and other s y m p t o m s of cardiac decompensation. His past history was noncontributory. Physical examination revealed a well developed, well nourished man who appeared healthy. The blood pressure was 120/82 and the pulse rate was 72 per minute. T h e lungs were clear and there were no signs of cardiac failure. The apical impulse was in the sixth intercostal space 4 cm. outside the left midclavicular line and was forceful. T h e second pulmonic sound was widely split. A faint short earl)' systolic m u r m u r was heard along the left sternal border. T h e electrocardiogram showed normal sinus rhythm and left ventricular enlargement. T h e chest x-ray showed a heart that was markedly enlarged to the left. T h e remainder o f the examination was negative. Further investigation was planned; however, the patient died suddenly while preparing to leave his place of work. At necropsy only the heart showed significant findings. It weighed 740 grams. T h e pericardial cavity contained 150 ml. of clear amber fluid. T h e epicardium was unremarkable except for a 2 by 4 cm. area at the u p p e r anterior part of the left ventricle that was covered with
September 1971
irregular fibrous strands. All chambers of the heart were dilated and were free of thrombi or emboli. T h e r e was a 3 by 4 cm. gray firm area at the endocardial surface involving the upper part o f the anterior septum below the tricuspid valve. T h e remainder o f the right and endocardial surface was not remarkable. T h e leaflets were smooth and edematous at the tip and were free of atheromatous plaques. T h e right ventricle measured 0.4 cm. in thickness. The pulmonary valve measured 7.7 cm. in circumference and the cusps were unremarkable. T h e mitral valve measured 13.2 cm. in circumference and the leaflets were smooth and glistening. T h e free edge was somewhat thickened but smooth. T h e left ventricular wall ranged from 1.4 to 3.5 cm. in thickness. T h e aortic valve measured 8.8 cm. in circumference at the ring. T h e cusps, free edge, and commissures were not remarkable. A round aneurysmal opening measuring 0.9 cm. iu diameter was present in the right aortic sinus at the level o f the cusp and communicated with an irregularly shaped cavity iu the interventricular septum. T h e cavity measured 5 by 4.5 by 2.5 cm. and occupied the major portion of the anterosuperior septum, extending into the anterolateral part of both ventricular walls. It communicated with three additional smaller cavities througl~ fistulous tracts averaging 1.0 cm. in diameter and 1.5 cm. in length. All these cavities were at the septum near the apex and measured 3.5 by 3.0 by 1.8, 3.6 by 2.5 by 1.5, and 3.0 by 2.0 by 1.5 cm., respectively. All four cavities produced the greater bulge into the chamber o f the left ventricle and the Smaller bulge into the chamber of the right ventricle. Thus, the septal wall varied from 1.0 to 2.8 cm. in thickness. T h e cavities contained a large amount of dark red blood clot. In the lower part of the largest and all additional three cavities, multiple red, brown, 6r gray, firm, laminated mural thrombi were attached to the inner surface of the cavity wall. Otherwise the cavity wall showed a gray, firm, and occasionally trabeculated surface resembling that o f the vascular intima. T h e siarrounding myocardium occasionally showed gray firm fibrosis. T h e r e was a longitudinal depression measuring 0.8 cm. in width and 0.3 cm. in depth at the aorta at the level of the aortic vah'e cusp. " T h e left and right coronary ostia were widely patent. T h e coronary arteries were free of atheromatous plaques and were widely patent throughout. T h e aorta was of normal circumference and unremarkable. T h e lungs weighed in toto 1060 grams and were not remarkable. Sections through the four aneurysmal cavities at the ~gptum and left ventricle disclosed that the cawty wall was composed of an endothelial lining with a hyalinized fibrous stroma,
CASE R E P O R T
Figure 1. Opened left ventricle and aortic valve showifig a large aneurysmal cavity at the interventricular septum branching out into smaller ones at the anterosuperior portion (tipper left) of the left ventricle and the apex (dotted line). The cavities con'tain dark red or gray, firm, laminated mural thrombi (large arrows). The aneurysm opens at the right aortic sinus (R). There is a depression (D) at the ascending aorta above the noncoronary sinus (N). RCO, Right coronary ostium. LCO, Left coronary ostium. L, Left aortic siuus.
which occasionally showed organizing o r organized thrombi. Adjacent to the cavity wall the myocardium was replaced b)" dense fibrous scar tissue s u r r o u n d e d b)" h y p e r t r o p h i e d cardiac mnscle cells. Examination o f the aneurysmal wall showed that the myocardium was fibroils and mostly replaced by hyaline stroma with foci of calcification. T h e depression at the aorta was characterized by focal d e g e n e r a t i o n o f the elastic fibers in the media, which were replaced by man)" cleft-like or irregular spaces filled with antorphous basophilic grOund substance. Examination o f the ascending aorta elsewhere disclosed that the m e d i a contained numerous cleft-like or small cyst-like spaces filled by basophilic naucinous, substance. T h e r e was no evidence o f mycotic infection o r atherosclerosis. Although longitudinal sections through the noncoronary sinus showed that the aortic media was continuous with the annulus fibrosus o f the aortic ring, a similar section through the aneurysmal sinus with the adjacent structure failed to d e m o n s t r a t e a clear-cut contintfity between the aortic media and the annulus fibrosus. No evidence o f p u l m o n a r y hypertension was present, as is often seen in this condition.
Anatomic diagnoses: 1. A n e u r y s m o f the right - coronary sinus o f Valsalva extending into the interventricular septum with fistulous cavity forma"tion a t the septum and both ventricles. 2. Focal medial sclerosis o f the ascending aorta and noncoronary sinus o f Valsalva with focal dissection, healed.
DISCUSSION T h i s case d e m o n s t r a t e s a n a n e u r y s m o f t i l e r i g h t c o r o n a r y s i n u s o f Valsalva. Aneurysms or aneurysmal dilatations may o r i g i n a t e in o n e o r m o r e o f t h e s i n u s e s o f V a l s a h ' a o f t h e a o r t i c valve. 3 T i i e r i g h t c o r o n a r y s i n u s is by f a r t h e m o s t c o m m o n site o r o r i g i n f o r a c o n g e n i t a l a n e u r y s m . O f :59 cases o f a n e u r y s m o f tile a o r t i c sinus reviewed by Kieffer and Winchell, 4 55 o r i g i n a t e d in t h e r i g h t c o r o n a r y sinus, 16 in t h e n o n c o r o n a r y sinus, a n d o n l y o n e case in t h e left c o r o n a r y sinus. As is c h a r acteristic of aneurysms of the sinns of V a l s a l v a , tile ~ r e s e n t anenrysm was
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HUMAN
PATHOLOGY--VOLUME
2, N U M B E R 3
formed by all inferior extension of tile right coronary sinus and the aneurysmal wall was composed of the sinus. Aneurysms of the right coronary sinus most frequently r u p t u r e into the cavity of the right ventricle and less fi'equently into the right atrial cavity. In all instances the ru p tu r e has created aortocardiac fistulas. T h e r e are only two cases of an aneurysm of the right coronary sinus with r u p t u r e into the left ventricle. One of these two cases reported by Warthen ~ resembles our case; his case showed an aneurysm of tire right coronary sinus dissecting into the interventricular septum for a distance of 4 cin. below tlte level o f the non- or left coronary sinus, which, however, perforated into the left ventricular aspect of the interventricular septum. T h e present aneurysm could have started as a congenital weakness or defect of the tissue structure at the right coronary sinus that r u p t u r e d into the muscular portion of the interventricular septum. In their careful histologic stndies on aneurysms o f the coronary sinus,-Edwards and Burcheli ~ have clearly showed that the lesion results from a defect between the aortic media and the annulus fibrosus at the base o f the aorta. However, such a clear-cut defect was absent in our case. T h e inner surface of the fistulous cavities in the septum and ventricular wall was microscopically indistinguishable fi-om the hyalinized vascular intima. T h e r e were nulnerous fi'esh to organized mural thrombi at t h e endothelialized cavity wall. This indicates that following t h e r u p ture o f the aneurysm the cavity became a part o f the outflow tract long before death. On the other hand, the aneurysm might have started as a focal teal" resulting fi'om the medial necrosis at the right coronary sinus that developed into an aneurysm and then dissected into the interventricular septum. This interpretation is based on the fact that there was scar tissue in the media at the noncoronary sinus and elsewhere in the ascending aorta. T h e relationship between aneurysms
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of the aortic sinus and medial necrosis has been stressed by Steinberg and G e l l e : in their three cases of Marfan's syndrome. Although their patients lacked ocular anomalies, all their three cases were characterized by araclmodactyly, aneurysm of the aortic sinuses, and medial sclerosis of tile aorta. Tile)' believed that aneurysmal dilatation of the aortic sinuses in patients with arachnodactyly is closely related to aortic medial degeneration. However, there was no evidence of ocular or skeletal anomalies in our patient.
REFERENCES !. Edwards, J. E., and Burchell, tt. B.: Specimen exhibiting the essential lesion in aneurysm of the aortic sinus. Proc. Staff Meet. Mayo Clin., 31:407-412, 1956. 2. Holloran, K. tt., qhluer, N. S., and Browne, M. J.: A study of ventricular septal defect associated with aortic insufficiency. Amer. tteart J., 69:320-337, 1965. 3. Hudson, R. E.: Congenital anomalies of the heart valves. In Cardiovascular Pathology, 1968-1969. Bahimore, The Williams &" Wilkins Co., 1965, Vol. 2. -t. Kieffer, S. A., and Wincfiell, 1'.: Congenital aneurysms of the aortic sinuses with cardioaortic fistula. Dis. Chest, 38:79-96, 1960. 5. Wartfien, R. D.: Congenital aneurysms of the right anterior sinus of Valsalva (interventricular aneurysm) with spontaneous rupture into the left ventricle. Amer. Heart J., 37:975-981, 1949. 6. Steinberg, I., and GeIler, W.: Aneurysmal dilatation of aortic sinuses in arachnodactyly. Diagnosis during life in three cases. Ann. Int. Med., 43!120-132, 1955. ACKNOSVLEDGMENT
We are grateful for assist,'ince to Dr. William C. Manion of tile Armed Forces Institute o f Pathology, Dr. Mark Masch and the Cardiology Staff of N);ack Hospital, Dr. Robert L. Yeager and Dr. ~V. G. C. Munroe of Summit Park Hospital, and Mrs. Josephine Nifontoff. Nyack Hospital Nyack, New York
10960 (Dr. Nakazawa)
ACKNO~,VLEDGMENTS T i m a t t t h o r s wish to t h a n k Mrs. W i n i fred Rinschler for p r e p a r i n g the m a n u script a n d Mrs. I d a N a t h a n f o r the pttotomicrographs.
REFERENCES 1. tlallgrimsson, J., Bj3rnsson, A., and (;udmundsson, G.: Meningioma of the neck. Case report. J. Ncurosurg., 32:695, 1970. 2. Suzuki, t!., Gilbert, E. F., and Zimmermann, B.: Primary extracranial meningioma. Arch. Path., 84:202, 1967. 3. Hoye, S. J., ttoar, C. S., and Murray, J. E.: Extracranial meningioma presenting as a tumor of the neck. Amer. J. Surg., 100:486, 1960. 4. Von der Zalm, H.: Extracranicel Menigeoom. Nederl. T. Geneesk., 108:492, 1964. 5. Dalforno, S., and Domm, A.: Neuroma Plessiforme della Parotide. Cancro, 22:529, 1969. 6. Foote, F. W., Jr., and Frazell, E. L.: Tumors of the Major Salivary Glands. AFIP Atlas of Tumor l'athology, Sect. IV., Fasc. 11., First Series. Washington, D.C., Armed Forces Institute of Pathology, 1954, p. 7. 7. Foote, F. W., Jr., and Frazell, E. L.: Tumors of the major salivary glands. Cancer, 6:1065, 1953.
8. Niccoli Vallesi, R.: Neurinoma della ghiandola sottolinguale. Arch. Vecchi Anat. Pat., 19:8t3, 1953. 9. Anson, B.J. (Editor): Morris' Human Anatomy. Ed. 12. New York, The Blakiston'Division, McGraw-Hill Book Company, 1966. 10. Schmidt, M. B.: Ueber die Pacchionischen Granulationen und ihr Verhaeltnis zu den Sarcomen und Psammomen der Dura mater. Virchows Arch. Path. Anat., 170:429, 1902. 11. Cushing, tt.: The meningiomas (dural endothelioma). Their source and favoured teats of origin. Brain, XLV (part 2):282, 1922. 12. Zachariae, L.: Case of extracranial primary meningioma. Acta l'ath. Microbiol. Scand., 31:57, 1952. 13. Willis, R. A.: The Borderland of Embryology and Pathology. Ed. 2. London, Butterworth & Co. (Publishers) Ltd., 1962. 14. Kernohan,J. W., and Sayre, G. P.: Tumors of the central nervous system: AFIP Atlas of Tumor Pathology, Scct. X, Fasc. 15. Washington, D.C., Armed Forces Institute of l'athology, 1952. 15. Aoyagi and Kytmo: Ueber die endothelien Zellzapfen in der Dura mater cerebri, and ihre Lokalisation in derselben. Neuroglia, H:I, 1912. Columbia University College of Physicians and Surgeons 630 West 168th Street New York, New York 10032
Case Report ANEURYSM OF THE AORTIC SINUS OF VALSALVA C O M M U N I C A T I N G WITH A FISTULOUS CAVITY IN THE INTERVENTRICULAR SEPTUM: REPORT OF A CASE HIROSHI NAKAZAWA,M.D.* LEOXARD S. BrieR, M.D.,t and SIGMUND "~VILENS,M.D.s+
Abstract An anemysm of the right corona U sinus is described that extended into large fi~tulous cavities of the ventricular septum and anterior wall of both *Assistant Clinical Professor of l'athology, Columbia University College of Physicians and Surgeons, New York. Assistant Director of I.aboratortes, Nyack ttospital, Nyack, New York. tAttending l'bysician, Department of Medicine, Nyack tlospital, Nyack, New York. ,+Professor of patlmlogy, Columbia University College of Physicians and Surgeons, New York, New York.
ventricles without perforation into the cardiac chamber. A review of the literature revealed no previous similar report. The present case suggests that medial necrosis plays a role ill the formation of sztch an atteuo'sm. A n e u r y s m s of the aortic sinus are relatively u n c o m m o n . T h e m a j o r i t y o f t h e m a r e c o n g e n i t a l a n d a few a r e mycotic? T h e c o n g e n i t a l a n e u r y s m o f t h e ' a o r tic s i n u s is o f t e n associated with a n i n t e r v e n t r i c u l a r septal defect2- T h e m y c o t i c a n e u r y s m m a y a p p e a r as a r e s u l t o f bacterial e n d o c a r d i t i s 2 L o c a t e d at t h e c o m m e n c e m e n t 'of the aorta, the pocket-like spaces f o r m e d by the aortic c u s p s a n d t h e a o r t i c wall h a v e slight d i l a t a t i o n s . T h e s e t h r e e d i l a t a t i o n s are called t h e s i n u s o f Valsalva, w h i c h s o m e t i m e s Ires a c o n g e n i tal w e a k n e s s o f tim tissne s t r t t c t n r e . W h e n s t r n c t u r a l w e a k n e s s is p r e s e n t , a s i n u s m a y be b u l g e d by the k i g h p r e s s u r e o f t h e a o r t a i n t o a low p r e s s u r e c l t a m b e r o f tlm h e a r t , r e s u l t i n g i n a d i v e r t i c n l a r a n e u r y s m . Tiffs
459
H U M A N P A T H O L O G Y - V O L U M E 2, N U M B E R 3 c o n d i t i o n is o f t e n u n r e c o g n i z e d clinically. W h e n it r e a c h e s a certain size, it o f t e n r u p t n r e s into a c a r d i a c c h a m b e r , a c c o m p a n i e d f r e q u e n t l y b y the s u d d e n onset o f chest o r a b d o m i n a l pain. T h e p a t i e n t develops rapid cardiac decompensation a n d d e a t h ensues. A l t ! l o u g h this c o n d i t i o n h a d b e e n o n e o f t h e fatal c a r d i a c lesions in the past, it i s n o w a m e n a b l e to o p e n heart surgery. Recently we o b s e r v e d a 31 )'ear old m a n w h o h a d b e e n a s y m p t o m a t i c , with t h e e x c e p t i o n o f occasional palpitations, a n d w h o s u d d e n l y died. T h e p o s t m o r t e m e x a m i n a t i o n r e v e a l e d an a n e u r y s m o f the r i g h t c o r o n a r y sinus, w h i c h c o n n n u n i c a t e d with a large fistulous cavity in the interv e n t r i c u l a r s e p t u m e x t e n d i n g into the a n t e r i o r left v e n t r i c u l a r wall. T h e r e was n o e v i d e n c e o f p e r f o r a t i o n into a c a r d i a c chamber. T h e p r e s e n t p a p e r describes the gross a n d m i c r o s c o p i c findings o f this u n u s u a l case a l o n g with a b r i e f discussion o f the conditions.
460
A 31 year old married Negro tailor had recently been told that he had an enlarged heart. T h e x-ray film had been taken when he left Haiti to come to the United States. He admitted to occasional episodes of palpitations but was otherwise asymptomatic. He denied dyspnea and other s y m p t o m s of cardiac decompensation. His past history was noncontributory. Physical examination revealed a well developed, well nourished man who appeared healthy. The blood pressure was 120/82 and the pulse rate was 72 per minute. T h e lungs were clear and there were no signs of cardiac failure. The apical impulse was in the sixth intercostal space 4 cm. outside the left midclavicular line and was forceful. T h e second pulmonic sound was widely split. A faint short earl)' systolic m u r m u r was heard along the left sternal border. T h e electrocardiogram showed normal sinus rhythm and left ventricular enlargement. T h e chest x-ray showed a heart that was markedly enlarged to the left. T h e remainder o f the examination was negative. Further investigation was planned; however, the patient died suddenly while preparing to leave his place of work. At necropsy only the heart showed significant findings. It weighed 740 grams. T h e pericardial cavity contained 150 ml. of clear amber fluid. T h e epicardium was unremarkable except for a 2 by 4 cm. area at the u p p e r anterior part of the left ventricle that was covered with
September 1971
irregular fibrous strands. All chambers of the heart were dilated and were free of thrombi or emboli. T h e r e was a 3 by 4 cm. gray firm area at the endocardial surface involving the upper part o f the anterior septum below the tricuspid valve. T h e remainder o f the right and endocardial surface was not remarkable. T h e leaflets were smooth and edematous at the tip and were free of atheromatous plaques. T h e right ventricle measured 0.4 cm. in thickness. The pulmonary valve measured 7.7 cm. in circumference and the cusps were unremarkable. T h e mitral valve measured 13.2 cm. in circumference and the leaflets were smooth and glistening. T h e free edge was somewhat thickened but smooth. T h e left ventricular wall ranged from 1.4 to 3.5 cm. in thickness. T h e aortic valve measured 8.8 cm. in circumference at the ring. T h e cusps, free edge, and commissures were not remarkable. A round aneurysmal opening measuring 0.9 cm. iu diameter was present in the right aortic sinus at the level o f the cusp and communicated with an irregularly shaped cavity iu the interventricular septum. T h e cavity measured 5 by 4.5 by 2.5 cm. and occupied the major portion of the anterosuperior septum, extending into the anterolateral part of both ventricular walls. It communicated with three additional smaller cavities througl~ fistulous tracts averaging 1.0 cm. in diameter and 1.5 cm. in length. All these cavities were at the septum near the apex and measured 3.5 by 3.0 by 1.8, 3.6 by 2.5 by 1.5, and 3.0 by 2.0 by 1.5 cm., respectively. All four cavities produced the greater bulge into the chamber o f the left ventricle and the Smaller bulge into the chamber of the right ventricle. Thus, the septal wall varied from 1.0 to 2.8 cm. in thickness. T h e cavities contained a large amount of dark red blood clot. In the lower part of the largest and all additional three cavities, multiple red, brown, 6r gray, firm, laminated mural thrombi were attached to the inner surface of the cavity wall. Otherwise the cavity wall showed a gray, firm, and occasionally trabeculated surface resembling that o f the vascular intima. T h e siarrounding myocardium occasionally showed gray firm fibrosis. T h e r e was a longitudinal depression measuring 0.8 cm. in width and 0.3 cm. in depth at the aorta at the level of the aortic vah'e cusp. " T h e left and right coronary ostia were widely patent. T h e coronary arteries were free of atheromatous plaques and were widely patent throughout. T h e aorta was of normal circumference and unremarkable. T h e lungs weighed in toto 1060 grams and were not remarkable. Sections through the four aneurysmal cavities at the ~gptum and left ventricle disclosed that the cawty wall was composed of an endothelial lining with a hyalinized fibrous stroma,
CASE R E P O R T
Figure 1. Opened left ventricle and aortic valve showifig a large aneurysmal cavity at the interventricular septum branching out into smaller ones at the anterosuperior portion (tipper left) of the left ventricle and the apex (dotted line). The cavities con'tain dark red or gray, firm, laminated mural thrombi (large arrows). The aneurysm opens at the right aortic sinus (R). There is a depression (D) at the ascending aorta above the noncoronary sinus (N). RCO, Right coronary ostium. LCO, Left coronary ostium. L, Left aortic siuus.
which occasionally showed organizing o r organized thrombi. Adjacent to the cavity wall the myocardium was replaced b)" dense fibrous scar tissue s u r r o u n d e d b)" h y p e r t r o p h i e d cardiac mnscle cells. Examination o f the aneurysmal wall showed that the myocardium was fibroils and mostly replaced by hyaline stroma with foci of calcification. T h e depression at the aorta was characterized by focal d e g e n e r a t i o n o f the elastic fibers in the media, which were replaced by man)" cleft-like or irregular spaces filled with antorphous basophilic grOund substance. Examination o f the ascending aorta elsewhere disclosed that the m e d i a contained numerous cleft-like or small cyst-like spaces filled by basophilic naucinous, substance. T h e r e was no evidence o f mycotic infection o r atherosclerosis. Although longitudinal sections through the noncoronary sinus showed that the aortic media was continuous with the annulus fibrosus o f the aortic ring, a similar section through the aneurysmal sinus with the adjacent structure failed to d e m o n s t r a t e a clear-cut contintfity between the aortic media and the annulus fibrosus. No evidence o f p u l m o n a r y hypertension was present, as is often seen in this condition.
Anatomic diagnoses: 1. A n e u r y s m o f the right - coronary sinus o f Valsalva extending into the interventricular septum with fistulous cavity forma"tion a t the septum and both ventricles. 2. Focal medial sclerosis o f the ascending aorta and noncoronary sinus o f Valsalva with focal dissection, healed.
DISCUSSION T h i s case d e m o n s t r a t e s a n a n e u r y s m o f t i l e r i g h t c o r o n a r y s i n u s o f Valsalva. Aneurysms or aneurysmal dilatations may o r i g i n a t e in o n e o r m o r e o f t h e s i n u s e s o f V a l s a h ' a o f t h e a o r t i c valve. 3 T i i e r i g h t c o r o n a r y s i n u s is by f a r t h e m o s t c o m m o n site o r o r i g i n f o r a c o n g e n i t a l a n e u r y s m . O f :59 cases o f a n e u r y s m o f tile a o r t i c sinus reviewed by Kieffer and Winchell, 4 55 o r i g i n a t e d in t h e r i g h t c o r o n a r y sinus, 16 in t h e n o n c o r o n a r y sinus, a n d o n l y o n e case in t h e left c o r o n a r y sinus. As is c h a r acteristic of aneurysms of the sinns of V a l s a l v a , tile ~ r e s e n t anenrysm was
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formed by all inferior extension of tile right coronary sinus and the aneurysmal wall was composed of the sinus. Aneurysms of the right coronary sinus most frequently r u p t u r e into the cavity of the right ventricle and less fi'equently into the right atrial cavity. In all instances the ru p tu r e has created aortocardiac fistulas. T h e r e are only two cases of an aneurysm of the right coronary sinus with r u p t u r e into the left ventricle. One of these two cases reported by Warthen ~ resembles our case; his case showed an aneurysm of tire right coronary sinus dissecting into the interventricular septum for a distance of 4 cin. below tlte level o f the non- or left coronary sinus, which, however, perforated into the left ventricular aspect of the interventricular septum. T h e present aneurysm could have started as a congenital weakness or defect of the tissue structure at the right coronary sinus that r u p t u r e d into the muscular portion of the interventricular septum. In their careful histologic stndies on aneurysms o f the coronary sinus,-Edwards and Burcheli ~ have clearly showed that the lesion results from a defect between the aortic media and the annulus fibrosus at the base o f the aorta. However, such a clear-cut defect was absent in our case. T h e inner surface of the fistulous cavities in the septum and ventricular wall was microscopically indistinguishable fi-om the hyalinized vascular intima. T h e r e were nulnerous fi'esh to organized mural thrombi at t h e endothelialized cavity wall. This indicates that following t h e r u p ture o f the aneurysm the cavity became a part o f the outflow tract long before death. On the other hand, the aneurysm might have started as a focal teal" resulting fi'om the medial necrosis at the right coronary sinus that developed into an aneurysm and then dissected into the interventricular septum. This interpretation is based on the fact that there was scar tissue in the media at the noncoronary sinus and elsewhere in the ascending aorta. T h e relationship between aneurysms
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of the aortic sinus and medial necrosis has been stressed by Steinberg and G e l l e : in their three cases of Marfan's syndrome. Although their patients lacked ocular anomalies, all their three cases were characterized by araclmodactyly, aneurysm of the aortic sinuses, and medial sclerosis of tile aorta. Tile)' believed that aneurysmal dilatation of the aortic sinuses in patients with arachnodactyly is closely related to aortic medial degeneration. However, there was no evidence of ocular or skeletal anomalies in our patient.
REFERENCES !. Edwards, J. E., and Burchell, tt. B.: Specimen exhibiting the essential lesion in aneurysm of the aortic sinus. Proc. Staff Meet. Mayo Clin., 31:407-412, 1956. 2. Holloran, K. tt., qhluer, N. S., and Browne, M. J.: A study of ventricular septal defect associated with aortic insufficiency. Amer. tteart J., 69:320-337, 1965. 3. Hudson, R. E.: Congenital anomalies of the heart valves. In Cardiovascular Pathology, 1968-1969. Bahimore, The Williams &" Wilkins Co., 1965, Vol. 2. -t. Kieffer, S. A., and Wincfiell, 1'.: Congenital aneurysms of the aortic sinuses with cardioaortic fistula. Dis. Chest, 38:79-96, 1960. 5. Wartfien, R. D.: Congenital aneurysms of the right anterior sinus of Valsalva (interventricular aneurysm) with spontaneous rupture into the left ventricle. Amer. Heart J., 37:975-981, 1949. 6. Steinberg, I., and GeIler, W.: Aneurysmal dilatation of aortic sinuses in arachnodactyly. Diagnosis during life in three cases. Ann. Int. Med., 43!120-132, 1955. ACKNOSVLEDGMENT
We are grateful for assist,'ince to Dr. William C. Manion of tile Armed Forces Institute o f Pathology, Dr. Mark Masch and the Cardiology Staff of N);ack Hospital, Dr. Robert L. Yeager and Dr. ~V. G. C. Munroe of Summit Park Hospital, and Mrs. Josephine Nifontoff. Nyack Hospital Nyack, New York
10960 (Dr. Nakazawa)