249
sinus. The size of the observed gradient, in the absence of reperfusion and, thus, washout from the region of myocardial necrosis, is probably a consequence of dilution from blood flowing into the coronary sinus from non-infarcted myocardium. Although further studies would need to be performed, it may be that the lack of a substantial gradient of enzymes between the coronary sinus and the venous blood is a marker for unsuccessful reperfusion, and could obviate the need for left heart catheterisation objectively to demonstrate this.
Acknowledgement We are grateful manuscript.
International Elsevier
CARD10
to Miss Aasiah Rahim for typing the
Journal of Cardiology,
References in 1 Feola M, Click G. Cardiac lymph flow and composition acute myocardial infarction in dogs. Am .I Physiol 1975; 229:44-48. 2 Clark G, Robison AK, Gnepp DR, Roberts R. Sobel B. Effecs of lymphatic transport of enzyme on plasma creatine kinase time-activity curves after myocardial infarction in dogs. Circ Res 1978;43:162-169. R, Niccoli L, Visioli 0, Harris P. Myocardial 3 Ferrari metabolism during intracoronary thrombolysis. Two illustrative cases. Int J Cardiol 1987;15:293-299. 4 Blanke H. Von Hardenberg D, Cohen M et al. Patterns of creatine kinase release during acute myocardial infarction after nonsurgical reperfusion: comparison with conventional treatment and correlation with infarct size. J Am Co11 Cardiol 1984;3:675-680.
29 (1990) 249-252
11546
Isolated rheumatic carditis, a disease in search of diagnostic criteria Frank Bauwens, Daniel Duprez and Luc Jordaens Department
of Cardiovascular
(Received
Diseases, University Hospital, Ghent, Belgium
30 April 1990; revision accepted
12 June 1990)
We report a case of acute dilated cardiomyopathy with histologic proof of inflammatory myocarditis. Although immunosuppressive therapy seemed to induce the resolution of the inflammatory infiltrate, it had no effect on the clinical course. The diagnosis of isolated rheumatic carditis was considered hecause of the finding of a very high antistreptolysin 0 titre and lack of evidence for either viral disease, infection with group C and G streptococci or other causes of spuriously high titres of anti-streptolysin 0. In view of our conclusion longterm chemoprophylaxis was instituted. Key words:
Anti-Streptolysin
0; Myocarditis;
Rheumatic
carditis
Introduction Correspondence Diseases, University Belgium. 0167-5273/90/$03.50
to: D. Duprez, Dept. of Cardiovascular Hospital, De Pintelaan 185, B-9000 Ghent,
Acute Western
0 1990 Elsevier Science
Publishers
B.V. (Biomedical
rheumatic countries.
Division)
fever
is now
Its sequel,
rarely
chronic
encountered
rheumatic
in
heart
250
disease, nonetheless, is the most important single cause of valvar heart disease in the world [l]. Recent resurgence of the disease in different parts of the United States should arouse the medical community [2]. Because of the consequences of the diagnosis, it seems appropriate to reconsider the revised Jones criteria [3] in the case of isolated rheumatc carditis, as illustrated by the following report. Case Report A 29-year-old man was admitted to the intensive care unit because of severe shortness of breath, chest pain and high fever (39” C). Before admission, the symptoms had already lasted two weeks. Two days earlier, the patient had suffered an episode of hemoptysis. On physical examination, the patient was obviously ill and looked very pale. The blood pressure measured
Fig. 1. Endomyocardial
biopsy
with signs of inflammatory
120/90 mm Hg. Beside a regular tachycardia (llO/min) and a third heart sound, the examination also revealed inspiratory crackles over the right middle and upper lobes. Bibasilar rales suggested left ventricular failure. The electrocardiogram showed complete left bundle branch block. A chest radiograph revealed extreme cardiomegaly and pneumonia of the right middle and lobes. Echocardiography suggested dilated upper cardiomyopathy: the end-diastolic internal diameter of the left ventricle measured 80 mm while the systolic function was severely depressed (estimated ejection fraction of 20%). The pattern of altered contractility clearly simulated myocardial infarction. While the septum and apex were thin (7 mm) and akinetic, the posterior wall was thicker (18 mm) and hypokinetic. An endomyocardial biopsy, however, established the diagnosis of inflammatory myocarditis (more than 5 lymphocytes per high-power field together with
myocarditis (more than 5 lymphocytes myocytolysis).
per high-power
field together
with
251 myocytolysis and granular deposition of immunoglobulin G) (Fig. 1). Furthermore, imaging with indium lllmonoclonal antimyosin antibody (Myoscint scanning) showed diffuse uptake within the heart compatible with diffuse necrosis. Initially. the patient was treated with antibiotics for the lobar pneumonia. Steroids were given in immunosuppressive dose (prednisone 1.25 mg/kg). Captopril and oral anticoagulants were added and the patient recovered promptly. Viral serologic tests were negative but a very high titre of anti-streptolysin 0 was found (3300 IU/ml, normal O-250 IU/ml). There was no other evidence of a recent streptococcal infection of the throat in the last few weeks and cultures from the throat were negative. After three weeks of prednisone therapy. a second biopsy, together with the Myoscint scan, remained positive. Azathioprine (2 mg/kg) was added to the regimen, while prednisone was tapered off over the following 12 weeks to a maintenance dose of 0.33 mg/kg. At 14 weeks, a third biopsy no longer revealed signs of active myocarditis. The Myoscint scan, however, was still positive and the diastolic dimensions and ejection fraction of the left ventricle had not changed. Nevertheless, the patient was doing well. During maintenance therapy, the patient developed severe pancytopenia which necessitated withdrawal of immunosuppressive therapy. Four weeks later, after recovery of all series of blood cells, a new endomyocardial biopsy again revealed inflammatory changes of active myocarditis. The Myoscint scan was still positive and the indices of ventricular function had not changed. The titre of antistreptolysin 0 had gradually fallen in the first few weeks to a lower, but still elevated level (800 lU/ml). This has not changed over the remainder of the period of follow-up. Clinically, the patient was doing well, being in class II of the New York Heart Association criteria and, because of the severe pancytopenia, a new course of immunosuppressive therapy was not undertaken. Discussion One major manifestation (carditis), two minor manifestations (fever, elevated acute phase reactants) and an elevated anti-streptolysin 0 titre are sufficient to fulfil the revised Jones criteria [3]. As a consequence, the diagnosis of rheumatic carditis can be withheld in our case. At a time when the disease is rarely encountered, however. this deserves some comment. When T. Duckett Jones originally formulated his criteria in 1944, the disease was very common and the criteria worked well, even without emphasizing the importance of recent streptococcal infection. Now, most cases of inflamma-
tory peri-myocarditis are probably caused by viruses (coxsackievirus A and B, echovirus) and other agents such as Toxoplasma gondii and Mycoplasma pneumoniae. Thus, beside excluding these causes of carditis, establishing firm proof of a recent streptococcal infection seems mandatory for a correct diagnosis. In a recent study by Daly et al., only six of twelve patients who presented with congestive cardiac failure and histologically confirmed myocarditis after a recent influenza like illness, showed serological evidence of viral aetiology [4]. It seems very likely that most of the other six patients had also viral heart disease. Nevertheless, despite extensive virological investigations (serological screening as well as cultures of throat and stool swabs and myocardial biopsy specimens), the causative agent could not be identified. Therefore, excluding viral heart disease in all cases of inflammatory carditis is not possible. When absence of evidence of recent streptococcal infection makes the diagnosis of rheumatic carditis unlikely, the reverse is not true. As already pointed out, one single elevated value of the titer of antistreptolysin 0 is sufficient to fulfil the revised Jones criteria [3]. A rise in the titer nonetheless, may occur as a result of infection with hemolytic streptococci of groups C and G which do not cause rheumatic fever. Also. spuriously high levels may occur in right heart failure, hepatitis, some forms of hyperlipoproteinemia, in nephrotic syndrome and even in some cases of typical viral myocarditis caused by Coxsackie B. Furthermore. after a streptococcal infection, the titre of anti-streptolysin 0 does not return to pre-infection levels within the first 6 to 12 months. During this time, infections with other agents may occur. Thus, a raised titre does not always indicate recent infection with a rheumatogenic strain. And, even when it does, it cannot be concluded that symptoms are related to that infection. After an upper respiratory tract infection with streptococci, a chronic carrier state will follow. During this state, no further immune response occurs, but positive throat swab cultures will be found at 11 weeks in 90% of the patients. Also, hemolytic streptococci can be isolated in as many as 20% of asymptomatic persons in closed communities. So, throat swab cultures will also fail to resolve the issue [5]. From the data presented above, it becomes clear that establishing a firm diagnosis of isolated rheumatic carditis is not possible. For the physician nonetheless as well as for the patient, a diagnosis of rheumatic carditis is of utmost importance. Long-term chemoprophylaxis should be instituted and continued indefinitely. There is a significant correlation between the attack rate of
252
rheumatic fever and the immunologic response (rise in anti-streptolysin 0) after a throat infection due to group A streptococci: the higher the titre of anti-streptolysin, the higher the risk of developing rheumatic fever [S]. In a recent epidemic in the United States, all patients had a titre of more than 500 Todd units [2]. Institution of chemoprophylaxis seems warranted after the finding of high titres when no clear evidence of viral disease (clinical, epidemiological and serological), infection with group C and G streptococci and other causes of spuriously high levels of anti-streptolysin 0 is found.
Acknowledgement
The authors the manuscript.
want to thank Mrs. L. Packet for typing
References Hall RJC, Julian DG. Rheumatic fever and its sequelae. In: Hall RCJ, Julian DG, eds. Diseases of the cardiac valves. New York: Churchill Livingstone, 1989;19-27. Papdimos T, Escamilla J, Garst P et al. Acute rheumatic fever at a navy base training center, San Diego, California. J Am Med Assoc 1988;259:1782-1787. Ad hoc committee to revise the Jones criteria (modified) of the council on rheumatic fever and congenital heart disease of the American Heart Association. Jones criteria (revised) for guidance in the diagnosis of rheumatic fever. Circulation 1984;69:203A-208A. Daly K, Richardson PJ, Olsen EGJ et al. Acute myocarditis. Role of histological and virological examination in the diagnosis and assessment of immunosuppressive treatement. Br Heart J 1984;51:30-35. Denny FW. T. Duckett Jones and rheumatic fever in 1986. T. Duckett Jones Memorial Lecture. Circulation 1987;76: 963-970.
Internatronal Journal of Cardiology, 29 (1990) 252-254 Elsevier
CARD10
11547
Elevated atria1 natriuretic factor in the plasma: cause or effect in “idiopathic orthostatic hypotension”? A. Mark Richards and Ian G. Crozier Departments
of Cardiology and Endocrinology, (Received
20 March
The Princess Margarer Hospiral, Christchurch, New Zealand 1990: revision accepted
14 June 1990)
A patient with idiopathic orthostatic hypotension exhibited low activity of norepinephrine and renin in plasma which failed to respond to standing. Levels of atrial natriuretic factor were inappropriately elevated and failed to fall on standing. The possible role of the atrial natriuretic factor in idiopathic orthostatic hypotension is discussed. Key words:
Atria1 natriuretic
factor;
Orthostatic
hypotension
Introduction Idiopathic orthostatic hypotension is an uncommon, disabling, condition with an ill-understood pathophysiCorrespondence to: Dr. I.G. Crazier, Dept. of Cardiology, The Princess Margaret Hospital, Christchurch 2, New Zealand.
0167-5273/90/$03.50
0 1990 Elsevier Science Publishers
ology. It is characterised by pronounced falls in arterial pressure with orthostasis and absence of appropriate reflex tachycardia. Syncope, anhydrosis, nocturia and impotence may occur. Resting concentrations of catecholamines in the plasma are low, and fail to respond stimuli
B.V. (Biomedical
appropriately [l].
Division)
The
to
changes
in
posture
renin-angiotensin-aldosterone
or
other system
sinus. The size of the observed gradient, in the absence of reperfusion and, thus, washout from the region of myocardial necrosis, is probably a consequence of dilution from blood flowing into the coronary sinus from non-infarcted myocardium. Although further studies would need to be performed, it may be that the lack of a substantial gradient of enzymes between the coronary sinus and the venous blood is a marker for unsuccessful reperfusion, and could obviate the need for left heart catheterisation objectively to demonstrate this.
Acknowledgement We are grateful manuscript.
International Elsevier
CARD10
to Miss Aasiah Rahim for typing the
Journal of Cardiology,
References in 1 Feola M, Click G. Cardiac lymph flow and composition acute myocardial infarction in dogs. Am .I Physiol 1975; 229:44-48. 2 Clark G, Robison AK, Gnepp DR, Roberts R. Sobel B. Effecs of lymphatic transport of enzyme on plasma creatine kinase time-activity curves after myocardial infarction in dogs. Circ Res 1978;43:162-169. R, Niccoli L, Visioli 0, Harris P. Myocardial 3 Ferrari metabolism during intracoronary thrombolysis. Two illustrative cases. Int J Cardiol 1987;15:293-299. 4 Blanke H. Von Hardenberg D, Cohen M et al. Patterns of creatine kinase release during acute myocardial infarction after nonsurgical reperfusion: comparison with conventional treatment and correlation with infarct size. J Am Co11 Cardiol 1984;3:675-680.
29 (1990) 249-252
11546
Isolated rheumatic carditis, a disease in search of diagnostic criteria Frank Bauwens, Daniel Duprez and Luc Jordaens Department
of Cardiovascular
(Received
Diseases, University Hospital, Ghent, Belgium
30 April 1990; revision accepted
12 June 1990)
We report a case of acute dilated cardiomyopathy with histologic proof of inflammatory myocarditis. Although immunosuppressive therapy seemed to induce the resolution of the inflammatory infiltrate, it had no effect on the clinical course. The diagnosis of isolated rheumatic carditis was considered hecause of the finding of a very high antistreptolysin 0 titre and lack of evidence for either viral disease, infection with group C and G streptococci or other causes of spuriously high titres of anti-streptolysin 0. In view of our conclusion longterm chemoprophylaxis was instituted. Key words:
Anti-Streptolysin
0; Myocarditis;
Rheumatic
carditis
Introduction Correspondence Diseases, University Belgium. 0167-5273/90/$03.50
to: D. Duprez, Dept. of Cardiovascular Hospital, De Pintelaan 185, B-9000 Ghent,
Acute Western
0 1990 Elsevier Science
Publishers
B.V. (Biomedical
rheumatic countries.
Division)
fever
is now
Its sequel,
rarely
chronic
encountered
rheumatic
in
heart
250
disease, nonetheless, is the most important single cause of valvar heart disease in the world [l]. Recent resurgence of the disease in different parts of the United States should arouse the medical community [2]. Because of the consequences of the diagnosis, it seems appropriate to reconsider the revised Jones criteria [3] in the case of isolated rheumatc carditis, as illustrated by the following report. Case Report A 29-year-old man was admitted to the intensive care unit because of severe shortness of breath, chest pain and high fever (39” C). Before admission, the symptoms had already lasted two weeks. Two days earlier, the patient had suffered an episode of hemoptysis. On physical examination, the patient was obviously ill and looked very pale. The blood pressure measured
Fig. 1. Endomyocardial
biopsy
with signs of inflammatory
120/90 mm Hg. Beside a regular tachycardia (llO/min) and a third heart sound, the examination also revealed inspiratory crackles over the right middle and upper lobes. Bibasilar rales suggested left ventricular failure. The electrocardiogram showed complete left bundle branch block. A chest radiograph revealed extreme cardiomegaly and pneumonia of the right middle and lobes. Echocardiography suggested dilated upper cardiomyopathy: the end-diastolic internal diameter of the left ventricle measured 80 mm while the systolic function was severely depressed (estimated ejection fraction of 20%). The pattern of altered contractility clearly simulated myocardial infarction. While the septum and apex were thin (7 mm) and akinetic, the posterior wall was thicker (18 mm) and hypokinetic. An endomyocardial biopsy, however, established the diagnosis of inflammatory myocarditis (more than 5 lymphocytes per high-power field together with
myocarditis (more than 5 lymphocytes myocytolysis).
per high-power
field together
with
251 myocytolysis and granular deposition of immunoglobulin G) (Fig. 1). Furthermore, imaging with indium lllmonoclonal antimyosin antibody (Myoscint scanning) showed diffuse uptake within the heart compatible with diffuse necrosis. Initially. the patient was treated with antibiotics for the lobar pneumonia. Steroids were given in immunosuppressive dose (prednisone 1.25 mg/kg). Captopril and oral anticoagulants were added and the patient recovered promptly. Viral serologic tests were negative but a very high titre of anti-streptolysin 0 was found (3300 IU/ml, normal O-250 IU/ml). There was no other evidence of a recent streptococcal infection of the throat in the last few weeks and cultures from the throat were negative. After three weeks of prednisone therapy. a second biopsy, together with the Myoscint scan, remained positive. Azathioprine (2 mg/kg) was added to the regimen, while prednisone was tapered off over the following 12 weeks to a maintenance dose of 0.33 mg/kg. At 14 weeks, a third biopsy no longer revealed signs of active myocarditis. The Myoscint scan, however, was still positive and the diastolic dimensions and ejection fraction of the left ventricle had not changed. Nevertheless, the patient was doing well. During maintenance therapy, the patient developed severe pancytopenia which necessitated withdrawal of immunosuppressive therapy. Four weeks later, after recovery of all series of blood cells, a new endomyocardial biopsy again revealed inflammatory changes of active myocarditis. The Myoscint scan was still positive and the indices of ventricular function had not changed. The titre of antistreptolysin 0 had gradually fallen in the first few weeks to a lower, but still elevated level (800 lU/ml). This has not changed over the remainder of the period of follow-up. Clinically, the patient was doing well, being in class II of the New York Heart Association criteria and, because of the severe pancytopenia, a new course of immunosuppressive therapy was not undertaken. Discussion One major manifestation (carditis), two minor manifestations (fever, elevated acute phase reactants) and an elevated anti-streptolysin 0 titre are sufficient to fulfil the revised Jones criteria [3]. As a consequence, the diagnosis of rheumatic carditis can be withheld in our case. At a time when the disease is rarely encountered, however. this deserves some comment. When T. Duckett Jones originally formulated his criteria in 1944, the disease was very common and the criteria worked well, even without emphasizing the importance of recent streptococcal infection. Now, most cases of inflamma-
tory peri-myocarditis are probably caused by viruses (coxsackievirus A and B, echovirus) and other agents such as Toxoplasma gondii and Mycoplasma pneumoniae. Thus, beside excluding these causes of carditis, establishing firm proof of a recent streptococcal infection seems mandatory for a correct diagnosis. In a recent study by Daly et al., only six of twelve patients who presented with congestive cardiac failure and histologically confirmed myocarditis after a recent influenza like illness, showed serological evidence of viral aetiology [4]. It seems very likely that most of the other six patients had also viral heart disease. Nevertheless, despite extensive virological investigations (serological screening as well as cultures of throat and stool swabs and myocardial biopsy specimens), the causative agent could not be identified. Therefore, excluding viral heart disease in all cases of inflammatory carditis is not possible. When absence of evidence of recent streptococcal infection makes the diagnosis of rheumatic carditis unlikely, the reverse is not true. As already pointed out, one single elevated value of the titer of antistreptolysin 0 is sufficient to fulfil the revised Jones criteria [3]. A rise in the titer nonetheless, may occur as a result of infection with hemolytic streptococci of groups C and G which do not cause rheumatic fever. Also. spuriously high levels may occur in right heart failure, hepatitis, some forms of hyperlipoproteinemia, in nephrotic syndrome and even in some cases of typical viral myocarditis caused by Coxsackie B. Furthermore. after a streptococcal infection, the titre of anti-streptolysin 0 does not return to pre-infection levels within the first 6 to 12 months. During this time, infections with other agents may occur. Thus, a raised titre does not always indicate recent infection with a rheumatogenic strain. And, even when it does, it cannot be concluded that symptoms are related to that infection. After an upper respiratory tract infection with streptococci, a chronic carrier state will follow. During this state, no further immune response occurs, but positive throat swab cultures will be found at 11 weeks in 90% of the patients. Also, hemolytic streptococci can be isolated in as many as 20% of asymptomatic persons in closed communities. So, throat swab cultures will also fail to resolve the issue [5]. From the data presented above, it becomes clear that establishing a firm diagnosis of isolated rheumatic carditis is not possible. For the physician nonetheless as well as for the patient, a diagnosis of rheumatic carditis is of utmost importance. Long-term chemoprophylaxis should be instituted and continued indefinitely. There is a significant correlation between the attack rate of
252
rheumatic fever and the immunologic response (rise in anti-streptolysin 0) after a throat infection due to group A streptococci: the higher the titre of anti-streptolysin, the higher the risk of developing rheumatic fever [S]. In a recent epidemic in the United States, all patients had a titre of more than 500 Todd units [2]. Institution of chemoprophylaxis seems warranted after the finding of high titres when no clear evidence of viral disease (clinical, epidemiological and serological), infection with group C and G streptococci and other causes of spuriously high levels of anti-streptolysin 0 is found.
Acknowledgement
The authors the manuscript.
want to thank Mrs. L. Packet for typing
References Hall RJC, Julian DG. Rheumatic fever and its sequelae. In: Hall RCJ, Julian DG, eds. Diseases of the cardiac valves. New York: Churchill Livingstone, 1989;19-27. Papdimos T, Escamilla J, Garst P et al. Acute rheumatic fever at a navy base training center, San Diego, California. J Am Med Assoc 1988;259:1782-1787. Ad hoc committee to revise the Jones criteria (modified) of the council on rheumatic fever and congenital heart disease of the American Heart Association. Jones criteria (revised) for guidance in the diagnosis of rheumatic fever. Circulation 1984;69:203A-208A. Daly K, Richardson PJ, Olsen EGJ et al. Acute myocarditis. Role of histological and virological examination in the diagnosis and assessment of immunosuppressive treatement. Br Heart J 1984;51:30-35. Denny FW. T. Duckett Jones and rheumatic fever in 1986. T. Duckett Jones Memorial Lecture. Circulation 1987;76: 963-970.
Internatronal Journal of Cardiology, 29 (1990) 252-254 Elsevier
CARD10
11547
Elevated atria1 natriuretic factor in the plasma: cause or effect in “idiopathic orthostatic hypotension”? A. Mark Richards and Ian G. Crozier Departments
of Cardiology and Endocrinology, (Received
20 March
The Princess Margarer Hospiral, Christchurch, New Zealand 1990: revision accepted
14 June 1990)
A patient with idiopathic orthostatic hypotension exhibited low activity of norepinephrine and renin in plasma which failed to respond to standing. Levels of atrial natriuretic factor were inappropriately elevated and failed to fall on standing. The possible role of the atrial natriuretic factor in idiopathic orthostatic hypotension is discussed. Key words:
Atria1 natriuretic
factor;
Orthostatic
hypotension
Introduction Idiopathic orthostatic hypotension is an uncommon, disabling, condition with an ill-understood pathophysiCorrespondence to: Dr. I.G. Crazier, Dept. of Cardiology, The Princess Margaret Hospital, Christchurch 2, New Zealand.
0167-5273/90/$03.50
0 1990 Elsevier Science Publishers
ology. It is characterised by pronounced falls in arterial pressure with orthostasis and absence of appropriate reflex tachycardia. Syncope, anhydrosis, nocturia and impotence may occur. Resting concentrations of catecholamines in the plasma are low, and fail to respond stimuli
B.V. (Biomedical
appropriately [l].
Division)
The
to
changes
in
posture
renin-angiotensin-aldosterone
or
other system
