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Atrial fibrillation in syphilitic aortic insufficiency
ATRIAL
FIBRILLATION
IN
SYPHILITIC
A. B. SMITH, M.D., AND THOMAS MEMPHIS,
AORTIC
INSUFFICIENCY
N. STERN, M.D.
TENN.
T
HE statement that atria1 fibrillation is rarely found in association with syphilitic heart disease may be found in any current medical or cardiology text. Thus, Stewart in Cecil and Loeb’s Textbook of Medicine’ states that fibrillation is associated infrequently with syphilitic forms of heart disease; White2 makes the statement that atria1 fibrillation is uncommon with cardiovascular syphilis; and PrinzmetaP in his monograph on the auricular arrhythmias says that syphilitic heart disease seldom is associated with atria1 fibrillation. Relying on these statements we frequently found ourselves in diagnostic error, disregarding syphilis which was later found to be present. In an effort to determine the actual incidence of atria1 fibrillation in syphilis, we reviewed the records of the John Gaston Hospital. During the period 19.50to 1954, there were 13,100 original admissions to the medical service of the hospital. Of this group the primary diagnosis of rheumatic heart disease was made in 279 patients. Atria1 fibrillation was present on electrocardiogram in 42, or 14.3 per cent, of these. The diagnosis of syphilitic aortic insufficiency was made 12.5 times during the same period and of these patients 14, or 11.2 per cent, were found to have atria1 fibrillation. In all casesthe diagnosis was made on the basis of classical aortic insufficiency, a positive serologic test for syphilis and/or a history of syphilis, and the absenceof history or findings The only exceptions were of other disease that might cause aortic insufficiency. For the purpose of this those cases proved at post-mortem to be syphilitic. series atria1 fibrillation was not considered to be present unless proved by ECG. Three typical case histories will be presented. Case No. 1, #225129.---H. M., a 4%year-old Negro man, was seen first in the Outpatient Department on Sept. 17, 1953, with symptoms and signs of cardiac insufficiency. The onset of symptoms was in 1950. They had progressed through exertional dyspnea,orthopnea,paroxysnocturia, and abdominal distention. There mal nocturnal dyspnea, ankle edema, frequency, was a history of gonorrhea 18 years previously and treatment for syphilis in 1950. Physical examination revealed a 6-foot, 142 pound man in no acute distress. There was neck vein distention. with the point There were fine moist inspiratory r&les in both lung bases. The heart was enlarged, of maxima1 impulse in the seventh left intercostal space in the anterior axillary line. No thrill was palpable. There was a loud blowing Grade 3 decrescendo, aortic diastolic murmur, and a An Austin-Flint murmur was thought to be present. high-pitched Grade 2 aortic systolic murmur. The blood pressure was 196/.50 mm. Hg and the pulse was totally irregular. A water-hammer pulse was palpable and a Duroziez sign was elicited. The liver was not enlarged and no evidence of ascites or edema was found. Three months later, however, when he was admitted to the hosGaston
From the Department Hospital, Memphis, Received for publication
of Medicine. University Term. Feb. 4, 1957.
of Tennessee
280
College
of Medicine,
and
the
John
?itl%zr “2”
SYPHILITIC
AORTIC
INSUFFICIENCY
281
pita1 in full-blown heart failure, there was ascites and the liver was paIpable 4 fingerbreadths below the right costal margin. The serologic test for syphilis was positive to 32 dilutions. The electrocardiogram showed atria1 fibrillation and also left ventricular hypertrophy, which was confirmed by x-ray. He was digitalized and treated with mercurial diuretics and discharged home well compensated. Case No. 2, #125171.-F. S., a 58-year-old Negro man, was admitted to the John Gaston Hospital in 1947, with symptoms but no signs of cardiac insufficiency. There was no history of rheumatic fever, but he had gonorrhea in 1927. He had a Grade 3 diastolic and a Grade 2 systolic murmur heard best over the aortic area. A systolic and diastolic thrill were present over the aortic area. A Corrigan pulse and Duroziez sign were demonstrable. The blood pressure was 170/66 mm. Hg. At this time his cardiac rhythm was regular at 84 beats per minute. He was diagnosed as having syphilitic aortitis, aortic insufficiency, and myocardial decompensation. He became asymptomatic on bed rest and a low-salt diet. Seven negative Kahn tests were obtained between the years 1947 and 19.51, with no positives occurring. He was admitted to the hospital again in July, 1951, with signs and symptoms of cardiac insufficiency. Once more the peripheral and central signs of aortic insufficiency were found. He now had atria1 fibrillation and because of this the diagnosis of syphilitic heart disease was questioned. Intensive therapy failed to revert the cardiac insufficiency, and he died. Autopsy: At autopsy (A-51-565) the heart weighed 910 grams and was massively dilated. The aortic valve circumference was 10 cm. There was thickening of the aortic valve characteristic of syphilis but no definite fusion or separation of the cusps. The mitral valve ring circumference was 13 cm. and the valve leaflets revealed no significant alterations. Grossly, the ascending aorta showed the classical tree-barking effect; microscopically, it showed medial degeneration and scarring, infiltration with lymphocytes, adventitial thickening, and thickening of the vasa vasorum. Case No. 3, #110473.-T. M., a 72-year-old Negro man, was admitted to the John Gaston Hospital Dec. 3, 1951, for the twelfth and last time since 1948. There was a history of a penile lesion in 1928, with partial therapy for syphilis at that time. In 1949, a serologic test for syphilis showed 10 Kahn units. On each admission the patient had signs and symptoms of cardiac insufficiency. He was found repeatedly to have cardiomegaly with predominantly left ventricular enlargement. A precordial to-and-fro murmur was described, the diastolic component being decrescendo, replacing the second heart sound and loudest just to left of sternum in the fifth intercostal space. The systolic component was rather coarse and was loudest over the primary aortic area. A Corrigan pulse, Duroziez sign, and “pistol shot” sound over the femoral artery were described on these admissions. On most admissions the cardiac rhythm was reported as being irregular, but not until this last admission was an electrocardiogram obtained which showed atria1 fibrillation. He died in intractable cardiac failure. Autopsy: At autopsy (ASl-864) the heart weighed 720 grams. The aortic valve ring circumference measured only 8 cm. but there was “rolling of the edges of the valve cusps with separation of the cusps,” and the valve was demonstrated to be incompetent. Throughout the aortic valve leaflets there was round cell perivascular infiltration. Toward the base of the leaflets there was a fair amount of elastic tissue and moderate cellularity. Toward the tip there was a rather abrupt change from the previously described tissue to a dense, hyalinized fibrous tissue with very scant cellularity. The mitral valve ring circumference measured 12 cm. and the leaflets showed no significant changes. The aorta presented both a gross and microscopic picture of syphilitic involvement. There were numerous small areas of scarring in the media surrounded by round cells. “The adventitia is thickened and fibrosed and in some areas is actually thicker than the media. There is an obliterating endarteritis and perivascular cuffing of the vasa vasorum. The adventitia is also sprinkled with round cells, some of which are seen as small islands. The intima is thickened and fibrosed and there is evidence of calcification.” DISCUSSION
The prevalent concept that atria1 fibrillation and syphilis are infrequently associated would appear to derive from various studies of atria1 fibrillation in which syphilis was unimportant on a percentage basis as an underlying cause of
282
SMITH
AND
STERN
Am. Heart J. August, 1957
fibrillation. This conclusion obviously does not depend solely on the relationship of syphilis and fibrillation but would vary with the number of cases of syphilis as compared to those of other types of heart disease. Evans.4 found that syphilis was present in 1.5 per cent of 2,958 cases of established atria1 fibrillation; the figures of Parkinson and Campbell5 were 2.5 per cent, In neither of these papers, however, is the reverse considered, that is, the incidence of fibrillation in syphilitic heart disease. This question was considered by Plice and Pfister6 who found that 45 out of 244 syphilitic patients had atria1 fibrillation. This incidence of 18.4 per cent compares well with our findings of 11.2 per cent. These values assume even more significance when compared to 14.3 per cent, the incidence of fibrillation in rheumatic heart disease at our hospital, and 12.5 per cent, the incidence of fibrillation in an unclassified group of 3,000 cardiac patients studied by White and Jones.7 Why do atria fibrillate? It is generally agreed3ss that the principal factor in producing fibrillation is the stretching or distention of the atria as the result of increased intra-atria1 pressure. Vagal stimulation is believed to play an as yet undetermined role in producing this arrhythmia.g There are no demonstrable pathologic changes to explain the phenomenon. The most common cause of increased atria1 pressure is left ventricular failure, regardless of the etiology of the heart disease. The second most common cause of increase in atria1 pressure is mitral stenosis. In mitral stenosis the atria1 distention and intra-atria1 pressure increase necessary to precipitate atria1 fibrillation may occur well before clinical “heart failure.” As a result of this fact atria1 fibrillation occurs earlier in cases with rheumatic heart disease than in cases with syphilitic heart disease. We are unable to find any anatomic, pathologic, physiologic, or philosophic reason for atria1 fibrillation being “rarely” associated with syphilitic heart disease. Our statistics, as well as those of others, indicate that the incidence is relatively high. There may be a socioeconomic explanation however. It well may be that the incidence of syphilitic heart disease seen late and untreated is low except in large charity centers. This, however, should not justify the perpetuation of inaccurate conclusions. In our location, atria1 fibrillation occurs statistically as often in syphilitic heart disease as it does in rheumatic heart disease. It is our opinion that atria1 fibrillation should not be used as a differentiating point between rheumatic heart disease and syphilitic heart disease, except possibly in patients who have never shown signs or symptoms of cardiac insufficiency. SUMMARY
Atria1 fibrillation has been found to be present in 11.2 per cent of all cases of syphilitic aortic insufficiency at the John Gaston Hospital. This percentage compares roughly with the incidence in rheumatic fever (14.3 per cent) at our hospital, and the overall incidence of fibrillation in a large series? of unselected cardiac cases (12.5 per cent). It is concluded that presence or absence of fibrillation cannot be used as a differential diagnostic point between syphilitic and rheumatic aortic regurgitation.
%2K?“2”
SYPHILITIC
AORTIC
283
INSUFFICIENCY
REFERENCES
1. 2. 3. 4. 5. 6. 7. 8. 9.
Cecil,
R. L., and Loeb, R. F.: A Textbook of Medicine, ed. 9 Philadelphia, Saunders Company, p. 1351. White, P. D.: Heart Disease, ed. 4, New York, 1951, Macmillan, p. 418. Prinzmetal, M., Corday, E., Brill, I. C., Oblath R. W., and Kruger, H. E.: Arrhythmias, Springfield, 1952, Charles C Thomas, pp. 220, 257. Evans, W. A.: Ann. Int. Med., 9:1171, 1936. Parkinson, J., and Campbell, M.: Quart. J. Med. 24:67, 1930. Plice, S. G., and Pfister, C. W.: Urol. & Cutan. Rev. 53545, 1949. White, P. D., and Jones, T. D.: AM. HEART J. 3:302, 1928. Luster, D., and Jeffreys, E. 0.: J. A. M. A. 107:2099, 1936. AM. HEART J. 18:1, 1939. Altschule, M. D.:
1954, W. B. The
Auricular
FIBRILLATION
IN
SYPHILITIC
A. B. SMITH, M.D., AND THOMAS MEMPHIS,
AORTIC
INSUFFICIENCY
N. STERN, M.D.
TENN.
T
HE statement that atria1 fibrillation is rarely found in association with syphilitic heart disease may be found in any current medical or cardiology text. Thus, Stewart in Cecil and Loeb’s Textbook of Medicine’ states that fibrillation is associated infrequently with syphilitic forms of heart disease; White2 makes the statement that atria1 fibrillation is uncommon with cardiovascular syphilis; and PrinzmetaP in his monograph on the auricular arrhythmias says that syphilitic heart disease seldom is associated with atria1 fibrillation. Relying on these statements we frequently found ourselves in diagnostic error, disregarding syphilis which was later found to be present. In an effort to determine the actual incidence of atria1 fibrillation in syphilis, we reviewed the records of the John Gaston Hospital. During the period 19.50to 1954, there were 13,100 original admissions to the medical service of the hospital. Of this group the primary diagnosis of rheumatic heart disease was made in 279 patients. Atria1 fibrillation was present on electrocardiogram in 42, or 14.3 per cent, of these. The diagnosis of syphilitic aortic insufficiency was made 12.5 times during the same period and of these patients 14, or 11.2 per cent, were found to have atria1 fibrillation. In all casesthe diagnosis was made on the basis of classical aortic insufficiency, a positive serologic test for syphilis and/or a history of syphilis, and the absenceof history or findings The only exceptions were of other disease that might cause aortic insufficiency. For the purpose of this those cases proved at post-mortem to be syphilitic. series atria1 fibrillation was not considered to be present unless proved by ECG. Three typical case histories will be presented. Case No. 1, #225129.---H. M., a 4%year-old Negro man, was seen first in the Outpatient Department on Sept. 17, 1953, with symptoms and signs of cardiac insufficiency. The onset of symptoms was in 1950. They had progressed through exertional dyspnea,orthopnea,paroxysnocturia, and abdominal distention. There mal nocturnal dyspnea, ankle edema, frequency, was a history of gonorrhea 18 years previously and treatment for syphilis in 1950. Physical examination revealed a 6-foot, 142 pound man in no acute distress. There was neck vein distention. with the point There were fine moist inspiratory r&les in both lung bases. The heart was enlarged, of maxima1 impulse in the seventh left intercostal space in the anterior axillary line. No thrill was palpable. There was a loud blowing Grade 3 decrescendo, aortic diastolic murmur, and a An Austin-Flint murmur was thought to be present. high-pitched Grade 2 aortic systolic murmur. The blood pressure was 196/.50 mm. Hg and the pulse was totally irregular. A water-hammer pulse was palpable and a Duroziez sign was elicited. The liver was not enlarged and no evidence of ascites or edema was found. Three months later, however, when he was admitted to the hosGaston
From the Department Hospital, Memphis, Received for publication
of Medicine. University Term. Feb. 4, 1957.
of Tennessee
280
College
of Medicine,
and
the
John
?itl%zr “2”
SYPHILITIC
AORTIC
INSUFFICIENCY
281
pita1 in full-blown heart failure, there was ascites and the liver was paIpable 4 fingerbreadths below the right costal margin. The serologic test for syphilis was positive to 32 dilutions. The electrocardiogram showed atria1 fibrillation and also left ventricular hypertrophy, which was confirmed by x-ray. He was digitalized and treated with mercurial diuretics and discharged home well compensated. Case No. 2, #125171.-F. S., a 58-year-old Negro man, was admitted to the John Gaston Hospital in 1947, with symptoms but no signs of cardiac insufficiency. There was no history of rheumatic fever, but he had gonorrhea in 1927. He had a Grade 3 diastolic and a Grade 2 systolic murmur heard best over the aortic area. A systolic and diastolic thrill were present over the aortic area. A Corrigan pulse and Duroziez sign were demonstrable. The blood pressure was 170/66 mm. Hg. At this time his cardiac rhythm was regular at 84 beats per minute. He was diagnosed as having syphilitic aortitis, aortic insufficiency, and myocardial decompensation. He became asymptomatic on bed rest and a low-salt diet. Seven negative Kahn tests were obtained between the years 1947 and 19.51, with no positives occurring. He was admitted to the hospital again in July, 1951, with signs and symptoms of cardiac insufficiency. Once more the peripheral and central signs of aortic insufficiency were found. He now had atria1 fibrillation and because of this the diagnosis of syphilitic heart disease was questioned. Intensive therapy failed to revert the cardiac insufficiency, and he died. Autopsy: At autopsy (A-51-565) the heart weighed 910 grams and was massively dilated. The aortic valve circumference was 10 cm. There was thickening of the aortic valve characteristic of syphilis but no definite fusion or separation of the cusps. The mitral valve ring circumference was 13 cm. and the valve leaflets revealed no significant alterations. Grossly, the ascending aorta showed the classical tree-barking effect; microscopically, it showed medial degeneration and scarring, infiltration with lymphocytes, adventitial thickening, and thickening of the vasa vasorum. Case No. 3, #110473.-T. M., a 72-year-old Negro man, was admitted to the John Gaston Hospital Dec. 3, 1951, for the twelfth and last time since 1948. There was a history of a penile lesion in 1928, with partial therapy for syphilis at that time. In 1949, a serologic test for syphilis showed 10 Kahn units. On each admission the patient had signs and symptoms of cardiac insufficiency. He was found repeatedly to have cardiomegaly with predominantly left ventricular enlargement. A precordial to-and-fro murmur was described, the diastolic component being decrescendo, replacing the second heart sound and loudest just to left of sternum in the fifth intercostal space. The systolic component was rather coarse and was loudest over the primary aortic area. A Corrigan pulse, Duroziez sign, and “pistol shot” sound over the femoral artery were described on these admissions. On most admissions the cardiac rhythm was reported as being irregular, but not until this last admission was an electrocardiogram obtained which showed atria1 fibrillation. He died in intractable cardiac failure. Autopsy: At autopsy (ASl-864) the heart weighed 720 grams. The aortic valve ring circumference measured only 8 cm. but there was “rolling of the edges of the valve cusps with separation of the cusps,” and the valve was demonstrated to be incompetent. Throughout the aortic valve leaflets there was round cell perivascular infiltration. Toward the base of the leaflets there was a fair amount of elastic tissue and moderate cellularity. Toward the tip there was a rather abrupt change from the previously described tissue to a dense, hyalinized fibrous tissue with very scant cellularity. The mitral valve ring circumference measured 12 cm. and the leaflets showed no significant changes. The aorta presented both a gross and microscopic picture of syphilitic involvement. There were numerous small areas of scarring in the media surrounded by round cells. “The adventitia is thickened and fibrosed and in some areas is actually thicker than the media. There is an obliterating endarteritis and perivascular cuffing of the vasa vasorum. The adventitia is also sprinkled with round cells, some of which are seen as small islands. The intima is thickened and fibrosed and there is evidence of calcification.” DISCUSSION
The prevalent concept that atria1 fibrillation and syphilis are infrequently associated would appear to derive from various studies of atria1 fibrillation in which syphilis was unimportant on a percentage basis as an underlying cause of
282
SMITH
AND
STERN
Am. Heart J. August, 1957
fibrillation. This conclusion obviously does not depend solely on the relationship of syphilis and fibrillation but would vary with the number of cases of syphilis as compared to those of other types of heart disease. Evans.4 found that syphilis was present in 1.5 per cent of 2,958 cases of established atria1 fibrillation; the figures of Parkinson and Campbell5 were 2.5 per cent, In neither of these papers, however, is the reverse considered, that is, the incidence of fibrillation in syphilitic heart disease. This question was considered by Plice and Pfister6 who found that 45 out of 244 syphilitic patients had atria1 fibrillation. This incidence of 18.4 per cent compares well with our findings of 11.2 per cent. These values assume even more significance when compared to 14.3 per cent, the incidence of fibrillation in rheumatic heart disease at our hospital, and 12.5 per cent, the incidence of fibrillation in an unclassified group of 3,000 cardiac patients studied by White and Jones.7 Why do atria fibrillate? It is generally agreed3ss that the principal factor in producing fibrillation is the stretching or distention of the atria as the result of increased intra-atria1 pressure. Vagal stimulation is believed to play an as yet undetermined role in producing this arrhythmia.g There are no demonstrable pathologic changes to explain the phenomenon. The most common cause of increased atria1 pressure is left ventricular failure, regardless of the etiology of the heart disease. The second most common cause of increase in atria1 pressure is mitral stenosis. In mitral stenosis the atria1 distention and intra-atria1 pressure increase necessary to precipitate atria1 fibrillation may occur well before clinical “heart failure.” As a result of this fact atria1 fibrillation occurs earlier in cases with rheumatic heart disease than in cases with syphilitic heart disease. We are unable to find any anatomic, pathologic, physiologic, or philosophic reason for atria1 fibrillation being “rarely” associated with syphilitic heart disease. Our statistics, as well as those of others, indicate that the incidence is relatively high. There may be a socioeconomic explanation however. It well may be that the incidence of syphilitic heart disease seen late and untreated is low except in large charity centers. This, however, should not justify the perpetuation of inaccurate conclusions. In our location, atria1 fibrillation occurs statistically as often in syphilitic heart disease as it does in rheumatic heart disease. It is our opinion that atria1 fibrillation should not be used as a differentiating point between rheumatic heart disease and syphilitic heart disease, except possibly in patients who have never shown signs or symptoms of cardiac insufficiency. SUMMARY
Atria1 fibrillation has been found to be present in 11.2 per cent of all cases of syphilitic aortic insufficiency at the John Gaston Hospital. This percentage compares roughly with the incidence in rheumatic fever (14.3 per cent) at our hospital, and the overall incidence of fibrillation in a large series? of unselected cardiac cases (12.5 per cent). It is concluded that presence or absence of fibrillation cannot be used as a differential diagnostic point between syphilitic and rheumatic aortic regurgitation.
%2K?“2”
SYPHILITIC
AORTIC
283
INSUFFICIENCY
REFERENCES
1. 2. 3. 4. 5. 6. 7. 8. 9.
Cecil,
R. L., and Loeb, R. F.: A Textbook of Medicine, ed. 9 Philadelphia, Saunders Company, p. 1351. White, P. D.: Heart Disease, ed. 4, New York, 1951, Macmillan, p. 418. Prinzmetal, M., Corday, E., Brill, I. C., Oblath R. W., and Kruger, H. E.: Arrhythmias, Springfield, 1952, Charles C Thomas, pp. 220, 257. Evans, W. A.: Ann. Int. Med., 9:1171, 1936. Parkinson, J., and Campbell, M.: Quart. J. Med. 24:67, 1930. Plice, S. G., and Pfister, C. W.: Urol. & Cutan. Rev. 53545, 1949. White, P. D., and Jones, T. D.: AM. HEART J. 3:302, 1928. Luster, D., and Jeffreys, E. 0.: J. A. M. A. 107:2099, 1936. AM. HEART J. 18:1, 1939. Altschule, M. D.:
1954, W. B. The
Auricular