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Syphilitic coronary occlusion in aortic insufficiency
S Y P H I L I T I C CORONARY OCCLUSION IN AORTIC I N S U F F I C I E N C Y ~:t J. H.
CANNON,
CHARLESTON,
M.D. S. C.
M P A I R E D circulation resulting from disease of the coronary arteries causing symptoms, heart failure and death is receiving an increasing amount of attention since Obrastzow and Straschezko in 1910,1 and I t e r r i c k in 1912, ~ called attention to the syndrome accompanying the sudden occlusion of these arteries. Since this condition is being recognized more generally by the profession, the f r e q u e n c y with which it occurs indicates that it is probably a far more common lesion t h a n has been suspected in the past. Those factors, therefore, having a causal relation to c o r o n a r y disease, whether predisposing to the precipitation of clots, proliferation of the intima, atheroma, or airy other change becomes of prime importance. Syphilis has long been recognized as causative in certain cases. It is this condition that I will discuss, presenting two case r e p o r t s and a syndrome which I believe will pernfit of recognition in certain cases. The first observations on c o r o n a r y disease seem to have been made by D r e l i n e o u r t (1700),3 and the association of c o r o n a r y and myocardial disease was first noted by Bellini (1703)2 L a t e r E d w a r d Jenner" and also P a r r y ~ believed disease of these arteries to be the u n d e r l y i n g lesion in angina peetoris, though Allbutt insisted that what J e n n e r really said was that, in patients dying of angina, c o r o n a r y sclerosis would be found, which he felt was quite a different matter. I n v o l v e m e n t of the aorta by syphilis, resulting" in aneurysm ]a relation suspected by Par~ 5 and later insisted upon by Laneisi (1728) and Morgagni (1761) ~ and established by Welch, (1876), Dohle (1888) ~ and confirmed by others] and also the d e f o r m i t y and destruction of tire aortic valve resulting in insufficiency (so well described by Dominiek Corrigan in 1832) '~ are common conditions in the southern negro. The combination of aortic insufficiency with c o r o n a r y closure is commented upon by most authors as of f r e q u e n t occurrence. Benson states t h a t syphilitic occlusion accounts for most closures of the coronaries and that the effect on the h e a r t is similar to other g'radual closures of these vessels where sufficient time has elapsed for anastomosis with the other c o r o n a r y to develop. According to Stokes, ~ in
I
* F r o m t h e D e p a r t m e n t of M e d i c i n e , M e d i c a l C o l l e g e o f t h e S t a t e of S o u t h C a r o l i n a . R e a d a t t h e F i f t h A n n u a l S c i e n t i f i c S e s s i o n of t h e A m e r i c a n H e a r t A s s o e i a t i o n , P o r t l a n d , O r e g o n , J u l y 9, 1929. t T h i s r e p o r t is p a r t o f t h e s t u d y of c a r d i o v a s c u l a r s y p h i l i s u n d e r t a k e n o n b e h a l f of t h e C o m m i t t e e f o r t h e C o o r d i n a t i o n of I n v e s t i g a t i o n of t h e A m e r i c a n H e a r t A s sociation.
93
94
THE AMERICAN HEART JOURNAL
a d v a n c e d grades of i n v o l v e m e n t of the aortic valve the e n c r o a c h m e n t on the coronaries m a y be expected to be severe. The s y m p t o m s m a y n o t be a guide, for even in mild grades of aortitis coronary change m a y be so severe as to menace life. Osler in his L u m e l i a n Lecture s stated, " A man m a y get on very comfortably w i t h practically a fourth of the w h o l e coronary s y s t e m , " and A l l b u t t 9 goes even farther and says that, "a m a n m a y get on w i t h the w h o l e system occluded, so far, at a n y rate, as the orifices and main trunks are c o n c e r n e d , " and concluded with K a n t h a c k that other things being equal, the factor of safety is the rate of occlusion. The f o l l o w i n g case reports illustrate certain features of interest: CASE REPORTS CASE 1.--E. E., well-nourished colored female, 26 years old, admitted January 22, died January 24, ]927. Stated that three weeks prior to admission she developed a cough, slight sore throat and soreness through her chest. One week later she developed shortness of breath and swelling of feet and legs. She had leucorrhea but denied venereal lesion. History otherwise not relevant.
Physical Exam~naticn.--Temperature 97°; pulse ranged between 88-130; respiration 28-44, labored and shallow; blood pressure, systolic 120 mm., diastolic 50 ram.; mucous membranes of mouth and throat congested; veins of neck prominent; r~les in lower lobes of both lungs posteriorly; cardiac dullness to sixth interspace, 12.5 cm. to left of midsternal line. Apex not visible, soft systolic murmur at apex poorly transmitted. No signs of fluid in abdomen, which was distended and tympanitic. Some swelling of feet and ankles. Hemoglobin 50 per cent. Red blood cells 2,100,000, leucocytes 9,000, 52 per cent polynuclears; catheterized u r i n e - acid, specific gravity 1.010. Acetone 1 plus, otherwise negative. Wassermann, Kolmer antigen, plus 4. Autopsy.--This subject was a young negro woman, about 26 years old, about 5 feet tall, weighing about 100 lbs., in well-nourished condition. There was a moderate anasarca, most noticeable in the legs and neck and general evidence of chronic heart failure in general marked passive congestion, edema, tissue degeneration and fibrosis, this being particularly in evidence in the lungs, liver and spleen. The kidneys showed merely cloudy swelling. The uterus was infantile, and there were bilateral dermoid cysts of the ovaries, each the size of a large orange. There was no arteriosclerosis. The aorta was pliable and elastic everywhere except in two areas. One of these was just distal to the aortic orifice and included a part of one of the aortic valve cusps. The other was up in the arch of the vessel. These areas wero quite similar in appearance, the proximal one being roughly pear-shaped and occupying about one square inch of intimal surface. I t began just behind the left posterior cusp, involving this cusp, and through its center coursed the completely obliterated mouth of the left coronary artery. The higher area was roughly rounded and about twice the size of the first. These areas were raised above the intimal level almost one fourth of an inch, were firm and of cartilagenous consistency. They were of a hyaline pale bluish appearance depressed and yellowish in the center. Over their surfaces were rough lines generally paralleling the course of the vessel. There were a few yellow atheromatous patches at other points in the intima. l~icroscopically, these areas are of typical syphilitic aortitis, with prominent vasa vasorum in the outer coats surrounded by lymphocytes, patches of necrosis in the media and marked hyaline fibrous thickening of the intima.
CANNON :
SYPHILITIC CORONARY OCCLUSION
95
The heart was extremely soft and flabby, a collapsing " d i s h - r a g ~' heart. The cavities were dilated but there was little or no hypertrophy. On section the muscle wall could be seen to have fibrous strands through it. There was slight thickening of the margins of the mitral leaflets. One cusp of the aortic valve was thickened, stiff, retracted and bound down into the syphilitic lesion about its base, as described above, giving definite valvular insufficiency. Microscopically some of the muscle of the heart wall showed definite, but not outstanding hypertrophy. There was general fibrosis, congestion and parenehymatous degeneration. Near the endoeardium was extreme parenchymatous and f a t t y degeneration and :fibrosis. The coronary vessels were open except as described above at the mouth of one main artery. The disability
in this case plainly
was the result
lesion at the aortic ring, giving slowly progressive and
at the same
coronary
artery.
time,
gradually
This nutritional
closing
the
impairment
of the
syphilitic
aortic insufficiency
mouth
of the involved
of t h e h e a r t
prevented
the ordinary response of cardiac hypertrophy and led to a failure the heart, unusually rapid in progress to completeness.
of
CASE 2.--M. G., Colored female, 26 years old, entered October 14, died, October' 19, 1927. For three months, she had noticed palpitation and shortness of breath aggravated by exertion. Forced to stop all work two weeks prior to admission. Feet, legs and abdomen began swelling ten days before admission. Three children living and well, no miscarriages. No history of initial sore. History otherwise no~ relevant.
Pl~ysieal Ex~m~nation.--Temperature ranged from 96°-99 °, reaching 100.5 ° ttu~ day before death. Pulse averaged 120. Respiration averaged 26 but was labored and shallow. Blood pressure 114 ram. systolic, 46 ram. diastolic. H e a r t not ap parently enlarged; apex in fifth space 8 cm. to left of midsternal line. Thrill over precordium corresponding to to-and-fro m u r m u r ; moderate general edema. Hemo globin 75 per cent~ red blood cells 3,800,000; white blood cells 34,000; polynuclears 80; Wassermann plus 4; catheterized urine--acid, specific gravity 1.010, albumin plus 1; casts, hyaline plus 1; finely granular plus 2; otherwise negative. Blood culture negative. Autopsy.--This subject was a negro woman about 26 years of age, of good development and state of nourishment and with marked general edema, anasarca, ascites and some excess fluid in pleural and pericardial cavities. There was general evidence of chronic heart failure in passive congestion, edema, tissue degeneration and fibrosis, this being prominent in lungs, liver, splee_a and kidneys. There was s well-marked sclerosis with calcification of the walls of the small arteries in the uterus, and this organ was fibrous. The ovaries contahmd several small cysts. The kidneys showed some sclerosis of small arteries with some glomerular fibrous obliteration, patchy cortical fibrosis with lymphocytosis. I n the first part of the aorta, involving two cusps of the aortic valve and the mouth of the right coronary artery was an elevated, bluish, hyaline plaque~ with rough and striated center. The valve cusps were thickened, uniformly shortened, retracted, and were agglutinated to each other at their adjoining ends. The mouth of the involved coronary was completely closed and the lumen obliterated by fibrosis to the first branch. The remainder of the coronary system was open. Section of this area in the aortic wall showed characteristic syphilitic aortitis, with fibrous thickening and surrounding lymphocytosis of the vasa vasorum, degeneration, necrosis and pronounced fibrosis of media and int[ma. The heart was little if at all hypertrophied, some of its fibers being large on section. The musculature was extremely flabby and pale, and the cavities were
96
TIIE AMERICAN
HEART
JOURNAL
dilated. The muscle fibers generally were granular and near the endocardium vary markedly vacuolated. Here particu]arly~ were marked congestion and fibrosis. The capillaries generally were engorged with blood and along their courses were many polynuclear leucocytes. There were some diffuse fibrosis and noticeable mononucleosis. Beneath the epicardium, especially at the base posteriorly, were nmnerous peteehial hemorrhages.
This h e a r t could be r e a s o n a b l y called t h a t of chronic myocarditis, or carditis, since all its p a r t s were involved in a process which was more t h a n the o r d i n a r y d e g e n e r a t i o n and fibrosis. While the state m a y be one of syphilitic carditis, it a p p e a r s t h a t the m a i n condition and disability was the result of the syphilitic lesion at the aortic ring with resulting aortic valve insufficiency and a coincident occlusion of the m o u t h of one c o r o n a r y artery, again so h a n d i c a p p i n g the o r g a n as to b r i n g a b o u t a p r o g r e s s i v e failure to completion in a period of time and with a r a p i d i t y of d e v e l o p m e n t of signs of cardiac failure different f r o m simple aortic insufficiency, even of such origin. These two cases were selected as the basis for this r e p o r t because of t h e i r s t r i k i n g similarity in so m a n y features. In each case the age was 26 years. While it is g e n e r a l l y a c c e p t e d t h a t the time elapsing f r o m the chancre to the d e v e l o p m e n t of the a o r t i c leak a v e r a g e s f r o m 18 to 20 years, it is also well k n o w n t h a t this is n o t always the case. B r o o k s '° r e p o r t s 5 cases in p a t i e n t s u n d e r 30 y e a r s of age and states t h a t t h e y showed a most s t r i k i n g and extensive c o r o n a r y involvement. The p a t i e n t s here r e p o r t e d were b o t h women. I n a series of 146 consecutive cases r e c e n t l y a d m i t t e d to the c a r d i o v a s c u l a r service of R o p e r Hospital, there were 83 males and 19 females. Both of the cases r e p o r t e d here were colored. The course was r a p i d in each case, 3 weeks and 3 months respectively. I t is possible t h a t the patients m a y have been sick longer t h a n t h e y stated, and t h a t t h e y d a t e d the onset f r o m the time they noted disabling symptoms. However, as Allb u t t ll states, " F o r I would r e p e a t t h a t the course of cardio-aortie syphills combined, as it too often is, with c o r o n a r y disease m a y be stealthily swift. Indeed, it is a k i n d of microbic e n d o e a r d i t i s . " Brooks TM records a case of p e r f o r a t i o n of one of the c o r o n a r y sinuses before the seconda r y rash appeared. The systolic blood pressure was not elevated, b u t the diastolic was low, resulting in an increased pulse pressure, 114-46 in one, a n d 12050 in the other. The r e s p i r a t i o n in each of these cases was notable for being labored r a t h e r t h a n f o r r a p i d i t y , a v e r a g i n g a r o u n d 27 in one case, and 35 in the other. The W a s s e r m a n n reaction was positive (plus 4) in each and was 89 p e r cent positive for the g r o u p of 38 colored w o m e n in our series, while for the 6 white w o m e n of the group, there were only 33 per cent positive reactions. The p e r c e n t a g e of positive reactions for the group of 146 cases was 82 per cent, 48 p e r cent for the white and 89 per cent f o r the colored.
CANNON:
SYPHILITIC CORONARY OCCLUSION
!17
The p o s t - m o r t e m e x a m i n a t i o n disclosed syphilitic aortitis with aortic insufficiency in each case. A l l b u t t 1'~ states t h a t aortic valve involvem e n t would a p p e a r to be present in a b o u t 30 per cent of all cases of syphilitic aortitis, mild and severe. Arneth in 202 cases of tertiar.v syphilis states he f o u n d 3.2 per cent of aortitis and 2 per cent of ~ortic" regurgitation. In our series, the diagnosis of i n c o m p e t e n c y of th~ aortic valve was made 51 times in about 35 per cent of all cases or ol' cases with aortitis. Some of these cases were readmissions, but if w,' take the actual n u m b e r of new eases, 107, aortic insufficiency was p r e s e n t in 23 cases (21 per cent). Both these cases had a main b r a n c h of the c o r o n a r y occluded at the ostium; in one, the r i g h t branch, and in the other, the left. All authors agree t h a t this is common in aortitis, t h o u g h I could not find any figures quoted. G. A. Allen, ~:~ in analyzing 1,000 consecutive postmortems, f o u n d macroscopic lesions of the coronaries in 371 cases. In 97, there was definite n a r r o w i n g or blocking of the lumen, only 7 of which were due to syphilis. According to Allbutt H the right is usually first involved, and he quotes B r o a d b e n t an insisting t h a t this is the reason t h a t cases of syphilitic aortic insufficiency do badly. Clinically, there was no a p p r e c i a b l e difference in the two cases suggesting any v a r i a t i o n d e p e n d i n g on which a r t e r y is occluded. The p a t i e n t with blocking" of the left a r t e r y was sick only three weeks and showed inueh more congestion of the mucous m e m b r a n e s of mouth and t h r o a t and dilated neck veins; the one with the occlusion of the rig'ht branch was sick three months and showed more a n a s a r e a and ascites. E a c h of these two cases failed to exhibit the usual response to inc o m p e t e n c y of the aortic valve with h y p e r t r o p h y . W h e t h e r this w~ls due to the lack of a n o r m a l m y o e a r d i u m as p o s t u l a t e d b y K r e h l '4 or' to the lack of a d e q u a t e n u t r i t i o n due to blockage of the c o r o n a r y is questionable. Personally, I think the latter is more likely and l h a t the i m p a i r e d n u t r i t i o n itself c o n t r i b u t e d to a more r a p i d d e g e n e r a t i o n of the muscle and the absence of h y p e r t r o p h y . I t is r e g r e t t a b l e t h a t owing to the stress of routine, these sections were not stained for the spirochete. There is much in the p i c t u r e to su~'gest t h a t they wouht have been found. SUMMARY
Two cases of syphilitic aortitis with insufficiency and occlusion of a main b r a n c h of a c o r o n a r y a r t e r y are reported. The s t r i k i n g simil a r i t y of the cases is c o m m e n t e d upon, and statistics f r o m analysis ()f a small series of cases are utilized. I t would seem t h e r e f o r e t h a t in y o u n g adults witt~ syphilis and aortic insufficiency who do not exhibit the usual c o m p e n s a t o r y h y p e r t r o p h y and whose p r o g r e s s is r a p i d l y t o w a r d a fatal outcome, one m a y reasonably p r e s u m e the i n v o l v e m e n t of one or more of the c o r o n a r y openings in the syphilitic process.
98
THE AMERICAN HEART JOURNAL REFERENCES
1. 2. 3. 4. 5. 6. 7. 8. 9. 10. 11. 12. 13. 14.
Quoted by Hammon, L.: Am. J. :~. Sc. 168: 786, 1927. Herrick, J. B.: J . A . M . A . 59: 2015, 1912. Quoted by Benson, R . L . : Arch. Path. & Lab. Med. 2: 905, 1926. Parry quoted by Allbutt, Clifford: Diseases of Arteries and Angina Pectoris, New York, 1915, p. 354, Macmillan & Co. Cecil's Textbook of Medicine, Philadelphia, 1927~ p. 1069, W. B. Saunders & Co. Corrigan, Dominick, quoted by Smith, Calvin: Heart Affections, Philadelphia, 1921, p. 254, F. A. Davis & Co. Stokes, J . H . : Modern Syphilology, Philadelphia, 1927, p. 889, W. B. Saunders & Co. Osler, Wm.: Quoted by Allbutt: Diseases of Arteries and Angina Pectoris, Lumelian Lectures, Lancet, March 26, 1910, p. 360. Allbutt, Clifford: Diseases of Arteries and Angina Pectoris, Lancet, l~areh 26, 1910, p. 361. Brooks, Harlow: Am. J. Syph. 5: 223, ]921. Allbutt, Clifford: Brit. Med. J. 2: 179, 1921. Brooks, Itarlow: Am. J. Svph. 5: 221, 1921. Allen, J . A . : Brit. M. J. 2" 232, 1928. Krehl: The Basis of Symptoms, Philadelphia, 1916, p. 32, Lippincott & Co. (For d ~ s i o n ,
see page 115.)
CANNON,
CHARLESTON,
M.D. S. C.
M P A I R E D circulation resulting from disease of the coronary arteries causing symptoms, heart failure and death is receiving an increasing amount of attention since Obrastzow and Straschezko in 1910,1 and I t e r r i c k in 1912, ~ called attention to the syndrome accompanying the sudden occlusion of these arteries. Since this condition is being recognized more generally by the profession, the f r e q u e n c y with which it occurs indicates that it is probably a far more common lesion t h a n has been suspected in the past. Those factors, therefore, having a causal relation to c o r o n a r y disease, whether predisposing to the precipitation of clots, proliferation of the intima, atheroma, or airy other change becomes of prime importance. Syphilis has long been recognized as causative in certain cases. It is this condition that I will discuss, presenting two case r e p o r t s and a syndrome which I believe will pernfit of recognition in certain cases. The first observations on c o r o n a r y disease seem to have been made by D r e l i n e o u r t (1700),3 and the association of c o r o n a r y and myocardial disease was first noted by Bellini (1703)2 L a t e r E d w a r d Jenner" and also P a r r y ~ believed disease of these arteries to be the u n d e r l y i n g lesion in angina peetoris, though Allbutt insisted that what J e n n e r really said was that, in patients dying of angina, c o r o n a r y sclerosis would be found, which he felt was quite a different matter. I n v o l v e m e n t of the aorta by syphilis, resulting" in aneurysm ]a relation suspected by Par~ 5 and later insisted upon by Laneisi (1728) and Morgagni (1761) ~ and established by Welch, (1876), Dohle (1888) ~ and confirmed by others] and also the d e f o r m i t y and destruction of tire aortic valve resulting in insufficiency (so well described by Dominiek Corrigan in 1832) '~ are common conditions in the southern negro. The combination of aortic insufficiency with c o r o n a r y closure is commented upon by most authors as of f r e q u e n t occurrence. Benson states t h a t syphilitic occlusion accounts for most closures of the coronaries and that the effect on the h e a r t is similar to other g'radual closures of these vessels where sufficient time has elapsed for anastomosis with the other c o r o n a r y to develop. According to Stokes, ~ in
I
* F r o m t h e D e p a r t m e n t of M e d i c i n e , M e d i c a l C o l l e g e o f t h e S t a t e of S o u t h C a r o l i n a . R e a d a t t h e F i f t h A n n u a l S c i e n t i f i c S e s s i o n of t h e A m e r i c a n H e a r t A s s o e i a t i o n , P o r t l a n d , O r e g o n , J u l y 9, 1929. t T h i s r e p o r t is p a r t o f t h e s t u d y of c a r d i o v a s c u l a r s y p h i l i s u n d e r t a k e n o n b e h a l f of t h e C o m m i t t e e f o r t h e C o o r d i n a t i o n of I n v e s t i g a t i o n of t h e A m e r i c a n H e a r t A s sociation.
93
94
THE AMERICAN HEART JOURNAL
a d v a n c e d grades of i n v o l v e m e n t of the aortic valve the e n c r o a c h m e n t on the coronaries m a y be expected to be severe. The s y m p t o m s m a y n o t be a guide, for even in mild grades of aortitis coronary change m a y be so severe as to menace life. Osler in his L u m e l i a n Lecture s stated, " A man m a y get on very comfortably w i t h practically a fourth of the w h o l e coronary s y s t e m , " and A l l b u t t 9 goes even farther and says that, "a m a n m a y get on w i t h the w h o l e system occluded, so far, at a n y rate, as the orifices and main trunks are c o n c e r n e d , " and concluded with K a n t h a c k that other things being equal, the factor of safety is the rate of occlusion. The f o l l o w i n g case reports illustrate certain features of interest: CASE REPORTS CASE 1.--E. E., well-nourished colored female, 26 years old, admitted January 22, died January 24, ]927. Stated that three weeks prior to admission she developed a cough, slight sore throat and soreness through her chest. One week later she developed shortness of breath and swelling of feet and legs. She had leucorrhea but denied venereal lesion. History otherwise not relevant.
Physical Exam~naticn.--Temperature 97°; pulse ranged between 88-130; respiration 28-44, labored and shallow; blood pressure, systolic 120 mm., diastolic 50 ram.; mucous membranes of mouth and throat congested; veins of neck prominent; r~les in lower lobes of both lungs posteriorly; cardiac dullness to sixth interspace, 12.5 cm. to left of midsternal line. Apex not visible, soft systolic murmur at apex poorly transmitted. No signs of fluid in abdomen, which was distended and tympanitic. Some swelling of feet and ankles. Hemoglobin 50 per cent. Red blood cells 2,100,000, leucocytes 9,000, 52 per cent polynuclears; catheterized u r i n e - acid, specific gravity 1.010. Acetone 1 plus, otherwise negative. Wassermann, Kolmer antigen, plus 4. Autopsy.--This subject was a young negro woman, about 26 years old, about 5 feet tall, weighing about 100 lbs., in well-nourished condition. There was a moderate anasarca, most noticeable in the legs and neck and general evidence of chronic heart failure in general marked passive congestion, edema, tissue degeneration and fibrosis, this being particularly in evidence in the lungs, liver and spleen. The kidneys showed merely cloudy swelling. The uterus was infantile, and there were bilateral dermoid cysts of the ovaries, each the size of a large orange. There was no arteriosclerosis. The aorta was pliable and elastic everywhere except in two areas. One of these was just distal to the aortic orifice and included a part of one of the aortic valve cusps. The other was up in the arch of the vessel. These areas wero quite similar in appearance, the proximal one being roughly pear-shaped and occupying about one square inch of intimal surface. I t began just behind the left posterior cusp, involving this cusp, and through its center coursed the completely obliterated mouth of the left coronary artery. The higher area was roughly rounded and about twice the size of the first. These areas were raised above the intimal level almost one fourth of an inch, were firm and of cartilagenous consistency. They were of a hyaline pale bluish appearance depressed and yellowish in the center. Over their surfaces were rough lines generally paralleling the course of the vessel. There were a few yellow atheromatous patches at other points in the intima. l~icroscopically, these areas are of typical syphilitic aortitis, with prominent vasa vasorum in the outer coats surrounded by lymphocytes, patches of necrosis in the media and marked hyaline fibrous thickening of the intima.
CANNON :
SYPHILITIC CORONARY OCCLUSION
95
The heart was extremely soft and flabby, a collapsing " d i s h - r a g ~' heart. The cavities were dilated but there was little or no hypertrophy. On section the muscle wall could be seen to have fibrous strands through it. There was slight thickening of the margins of the mitral leaflets. One cusp of the aortic valve was thickened, stiff, retracted and bound down into the syphilitic lesion about its base, as described above, giving definite valvular insufficiency. Microscopically some of the muscle of the heart wall showed definite, but not outstanding hypertrophy. There was general fibrosis, congestion and parenehymatous degeneration. Near the endoeardium was extreme parenchymatous and f a t t y degeneration and :fibrosis. The coronary vessels were open except as described above at the mouth of one main artery. The disability
in this case plainly
was the result
lesion at the aortic ring, giving slowly progressive and
at the same
coronary
artery.
time,
gradually
This nutritional
closing
the
impairment
of the
syphilitic
aortic insufficiency
mouth
of the involved
of t h e h e a r t
prevented
the ordinary response of cardiac hypertrophy and led to a failure the heart, unusually rapid in progress to completeness.
of
CASE 2.--M. G., Colored female, 26 years old, entered October 14, died, October' 19, 1927. For three months, she had noticed palpitation and shortness of breath aggravated by exertion. Forced to stop all work two weeks prior to admission. Feet, legs and abdomen began swelling ten days before admission. Three children living and well, no miscarriages. No history of initial sore. History otherwise no~ relevant.
Pl~ysieal Ex~m~nation.--Temperature ranged from 96°-99 °, reaching 100.5 ° ttu~ day before death. Pulse averaged 120. Respiration averaged 26 but was labored and shallow. Blood pressure 114 ram. systolic, 46 ram. diastolic. H e a r t not ap parently enlarged; apex in fifth space 8 cm. to left of midsternal line. Thrill over precordium corresponding to to-and-fro m u r m u r ; moderate general edema. Hemo globin 75 per cent~ red blood cells 3,800,000; white blood cells 34,000; polynuclears 80; Wassermann plus 4; catheterized urine--acid, specific gravity 1.010, albumin plus 1; casts, hyaline plus 1; finely granular plus 2; otherwise negative. Blood culture negative. Autopsy.--This subject was a negro woman about 26 years of age, of good development and state of nourishment and with marked general edema, anasarca, ascites and some excess fluid in pleural and pericardial cavities. There was general evidence of chronic heart failure in passive congestion, edema, tissue degeneration and fibrosis, this being prominent in lungs, liver, splee_a and kidneys. There was s well-marked sclerosis with calcification of the walls of the small arteries in the uterus, and this organ was fibrous. The ovaries contahmd several small cysts. The kidneys showed some sclerosis of small arteries with some glomerular fibrous obliteration, patchy cortical fibrosis with lymphocytosis. I n the first part of the aorta, involving two cusps of the aortic valve and the mouth of the right coronary artery was an elevated, bluish, hyaline plaque~ with rough and striated center. The valve cusps were thickened, uniformly shortened, retracted, and were agglutinated to each other at their adjoining ends. The mouth of the involved coronary was completely closed and the lumen obliterated by fibrosis to the first branch. The remainder of the coronary system was open. Section of this area in the aortic wall showed characteristic syphilitic aortitis, with fibrous thickening and surrounding lymphocytosis of the vasa vasorum, degeneration, necrosis and pronounced fibrosis of media and int[ma. The heart was little if at all hypertrophied, some of its fibers being large on section. The musculature was extremely flabby and pale, and the cavities were
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dilated. The muscle fibers generally were granular and near the endocardium vary markedly vacuolated. Here particu]arly~ were marked congestion and fibrosis. The capillaries generally were engorged with blood and along their courses were many polynuclear leucocytes. There were some diffuse fibrosis and noticeable mononucleosis. Beneath the epicardium, especially at the base posteriorly, were nmnerous peteehial hemorrhages.
This h e a r t could be r e a s o n a b l y called t h a t of chronic myocarditis, or carditis, since all its p a r t s were involved in a process which was more t h a n the o r d i n a r y d e g e n e r a t i o n and fibrosis. While the state m a y be one of syphilitic carditis, it a p p e a r s t h a t the m a i n condition and disability was the result of the syphilitic lesion at the aortic ring with resulting aortic valve insufficiency and a coincident occlusion of the m o u t h of one c o r o n a r y artery, again so h a n d i c a p p i n g the o r g a n as to b r i n g a b o u t a p r o g r e s s i v e failure to completion in a period of time and with a r a p i d i t y of d e v e l o p m e n t of signs of cardiac failure different f r o m simple aortic insufficiency, even of such origin. These two cases were selected as the basis for this r e p o r t because of t h e i r s t r i k i n g similarity in so m a n y features. In each case the age was 26 years. While it is g e n e r a l l y a c c e p t e d t h a t the time elapsing f r o m the chancre to the d e v e l o p m e n t of the a o r t i c leak a v e r a g e s f r o m 18 to 20 years, it is also well k n o w n t h a t this is n o t always the case. B r o o k s '° r e p o r t s 5 cases in p a t i e n t s u n d e r 30 y e a r s of age and states t h a t t h e y showed a most s t r i k i n g and extensive c o r o n a r y involvement. The p a t i e n t s here r e p o r t e d were b o t h women. I n a series of 146 consecutive cases r e c e n t l y a d m i t t e d to the c a r d i o v a s c u l a r service of R o p e r Hospital, there were 83 males and 19 females. Both of the cases r e p o r t e d here were colored. The course was r a p i d in each case, 3 weeks and 3 months respectively. I t is possible t h a t the patients m a y have been sick longer t h a n t h e y stated, and t h a t t h e y d a t e d the onset f r o m the time they noted disabling symptoms. However, as Allb u t t ll states, " F o r I would r e p e a t t h a t the course of cardio-aortie syphills combined, as it too often is, with c o r o n a r y disease m a y be stealthily swift. Indeed, it is a k i n d of microbic e n d o e a r d i t i s . " Brooks TM records a case of p e r f o r a t i o n of one of the c o r o n a r y sinuses before the seconda r y rash appeared. The systolic blood pressure was not elevated, b u t the diastolic was low, resulting in an increased pulse pressure, 114-46 in one, a n d 12050 in the other. The r e s p i r a t i o n in each of these cases was notable for being labored r a t h e r t h a n f o r r a p i d i t y , a v e r a g i n g a r o u n d 27 in one case, and 35 in the other. The W a s s e r m a n n reaction was positive (plus 4) in each and was 89 p e r cent positive for the g r o u p of 38 colored w o m e n in our series, while for the 6 white w o m e n of the group, there were only 33 per cent positive reactions. The p e r c e n t a g e of positive reactions for the group of 146 cases was 82 per cent, 48 p e r cent for the white and 89 per cent f o r the colored.
CANNON:
SYPHILITIC CORONARY OCCLUSION
!17
The p o s t - m o r t e m e x a m i n a t i o n disclosed syphilitic aortitis with aortic insufficiency in each case. A l l b u t t 1'~ states t h a t aortic valve involvem e n t would a p p e a r to be present in a b o u t 30 per cent of all cases of syphilitic aortitis, mild and severe. Arneth in 202 cases of tertiar.v syphilis states he f o u n d 3.2 per cent of aortitis and 2 per cent of ~ortic" regurgitation. In our series, the diagnosis of i n c o m p e t e n c y of th~ aortic valve was made 51 times in about 35 per cent of all cases or ol' cases with aortitis. Some of these cases were readmissions, but if w,' take the actual n u m b e r of new eases, 107, aortic insufficiency was p r e s e n t in 23 cases (21 per cent). Both these cases had a main b r a n c h of the c o r o n a r y occluded at the ostium; in one, the r i g h t branch, and in the other, the left. All authors agree t h a t this is common in aortitis, t h o u g h I could not find any figures quoted. G. A. Allen, ~:~ in analyzing 1,000 consecutive postmortems, f o u n d macroscopic lesions of the coronaries in 371 cases. In 97, there was definite n a r r o w i n g or blocking of the lumen, only 7 of which were due to syphilis. According to Allbutt H the right is usually first involved, and he quotes B r o a d b e n t an insisting t h a t this is the reason t h a t cases of syphilitic aortic insufficiency do badly. Clinically, there was no a p p r e c i a b l e difference in the two cases suggesting any v a r i a t i o n d e p e n d i n g on which a r t e r y is occluded. The p a t i e n t with blocking" of the left a r t e r y was sick only three weeks and showed inueh more congestion of the mucous m e m b r a n e s of mouth and t h r o a t and dilated neck veins; the one with the occlusion of the rig'ht branch was sick three months and showed more a n a s a r e a and ascites. E a c h of these two cases failed to exhibit the usual response to inc o m p e t e n c y of the aortic valve with h y p e r t r o p h y . W h e t h e r this w~ls due to the lack of a n o r m a l m y o e a r d i u m as p o s t u l a t e d b y K r e h l '4 or' to the lack of a d e q u a t e n u t r i t i o n due to blockage of the c o r o n a r y is questionable. Personally, I think the latter is more likely and l h a t the i m p a i r e d n u t r i t i o n itself c o n t r i b u t e d to a more r a p i d d e g e n e r a t i o n of the muscle and the absence of h y p e r t r o p h y . I t is r e g r e t t a b l e t h a t owing to the stress of routine, these sections were not stained for the spirochete. There is much in the p i c t u r e to su~'gest t h a t they wouht have been found. SUMMARY
Two cases of syphilitic aortitis with insufficiency and occlusion of a main b r a n c h of a c o r o n a r y a r t e r y are reported. The s t r i k i n g simil a r i t y of the cases is c o m m e n t e d upon, and statistics f r o m analysis ()f a small series of cases are utilized. I t would seem t h e r e f o r e t h a t in y o u n g adults witt~ syphilis and aortic insufficiency who do not exhibit the usual c o m p e n s a t o r y h y p e r t r o p h y and whose p r o g r e s s is r a p i d l y t o w a r d a fatal outcome, one m a y reasonably p r e s u m e the i n v o l v e m e n t of one or more of the c o r o n a r y openings in the syphilitic process.
98
THE AMERICAN HEART JOURNAL REFERENCES
1. 2. 3. 4. 5. 6. 7. 8. 9. 10. 11. 12. 13. 14.
Quoted by Hammon, L.: Am. J. :~. Sc. 168: 786, 1927. Herrick, J. B.: J . A . M . A . 59: 2015, 1912. Quoted by Benson, R . L . : Arch. Path. & Lab. Med. 2: 905, 1926. Parry quoted by Allbutt, Clifford: Diseases of Arteries and Angina Pectoris, New York, 1915, p. 354, Macmillan & Co. Cecil's Textbook of Medicine, Philadelphia, 1927~ p. 1069, W. B. Saunders & Co. Corrigan, Dominick, quoted by Smith, Calvin: Heart Affections, Philadelphia, 1921, p. 254, F. A. Davis & Co. Stokes, J . H . : Modern Syphilology, Philadelphia, 1927, p. 889, W. B. Saunders & Co. Osler, Wm.: Quoted by Allbutt: Diseases of Arteries and Angina Pectoris, Lumelian Lectures, Lancet, March 26, 1910, p. 360. Allbutt, Clifford: Diseases of Arteries and Angina Pectoris, Lancet, l~areh 26, 1910, p. 361. Brooks, Harlow: Am. J. Syph. 5: 223, ]921. Allbutt, Clifford: Brit. Med. J. 2: 179, 1921. Brooks, Itarlow: Am. J. Svph. 5: 221, 1921. Allen, J . A . : Brit. M. J. 2" 232, 1928. Krehl: The Basis of Symptoms, Philadelphia, 1916, p. 32, Lippincott & Co. (For d ~ s i o n ,
see page 115.)