The electrocardiogram in patients with polymyositis and dermatomyositis

THE

COOLIDGE

ELECTROCARDIOGRAM POLYMYOSITIS AND S. WAKAI,

M.D., ROBERT

IN PATIENTS DERMATOMYOSITIS

ROBERT 0. BRANDENBURG, R. KIERLAND, M.D.

ROCHESTER,

WITH

M.D.,

AND

MINN.

P

OLYMYOSITIS is one of the so-called collagen diseases which was first described by Wagner’ in 1863. It is a nonsuppurative, inflammatory, and degenerative disease of striated muscle without involvement of the skin. The late Dr. O’Leary, however, suggested that it is well to think of dermatomyositis as polymyositis with variable cutaneous lesions.2 Excellent clinical and pathologic reviews have been published3-” on polymyositis and dermatomyositis. According to O’Leary, cardiac abnormalities are to be expected, especially in the severe cases of dermatomyositis with generalized muscular disease. He noted myocarditis with cardiac hypertrophy in some of these patients. The present study was undertaken to determine whether the electrocardiogram would disclose abnormalities in patients with proved polymyositis and dermatomyositis. During the period from January, 1950, through December, 1954, a total of 169 patients having polymyositis or dermatomyositis were encountered at the Mayo Clinic. Our paper will be limited to the electrocardiographic findings on 27 patients in whom the clinical diagnosis was confirmed by muscle biopsy or necropsy. Electromyograms were done on 26 of these patients; all results were reported as being compatible with those seen in diseasesof skeletal muscles. An abnormal electromyographic finding alone is not pathognomonic of dermatomyositis or polymyositis, as it has been observed in other myopathies7 Most of the patients whose electrocardiograms were reviewed for this study did not have symptoms or signs referable to the heart. The literature on this subject indicates no unanimity of opinion with regard to the presence or absence of myocardial involvement. Gunther* in 1940, stated that no electrocardiographic changes are found in polymyositis. Keil,5 also in 1940, stated that the low voltage in dermatomyositis is due to increased resistance of the involved muscles, subcutaneous tissues, and skin to electrical current. Consequently, low-voltage complexes are not always a positive proof of myocardial disease.5 In a case of dermatomyositis associated with hypertrichosis reported by Reich and Reinhart,g the electrocardiographic changes were interpreted as being due to variance of cutaneous resistance induced by aggravation of the From the Mayo Clinic and Mayo Foundation. School of the University of Minnesota. Received for publication July 16. 1956.

The

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EC’G IN PATIENTS

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disease (since the heart is not involved in dermatomyositis). In 1954, an author of a text on heart diseases stated that “the cardiac muscle has not been conclusively proven to be involved” in dermatomyositis.‘” Several cases of myocardial involvement have been reported in the lit-erature fi.11-18however, and in some of these cases the myocardial involvement was considered to be the cause of death. The most common electrocardiographic abnormalities according to Domzalski and Morgan 3 are low-voltage compkues, evidence of nonspecific myocardial injury, and both right ventricular hypertroI)hyr and left ventricular hypertrophy with strain. WXiams” interpreted the T-w,lvt abnormalities of a 63-year-old patient with dermatomyositis as being due to generalized myocardial involvement such as is seen in anemia, mnlrtutritil)n, ;1 II d cachexia. T.~BLE

cases Polymyositis Dermatomyositis _- _---__Confirmation of clinical Biopsy Necropsy

I.

SUMMARY

OF DATA

Total

---~-~

27 5 22

I

diagnosis 25 2

.--

Electromyogram (26 done) Abnormality found ---_-.-..- ~Sex Males Females Xge,

I

I

-

, 4 2.4

I

o--i3 443 0 18

,

‘l‘emperat ure, elevated Range, “F.

17 9

26 26

,

range in years Mean. years Less than 40 years, patients More than 40 years, patients

,

99.8-102 4

’ /

I

i-68 .?9.%+ I2 14 100-103 9

Necrops) Myocardial changes Normal heart *Age of one adult tMinor histologic

not specifled. changes were

not considered CLINICAL

significant. DAT:\

Five of our 27 patients were given the diagnosis of polymyositis, and 22 There had the combined muscular and cutaneous changes of dermatomyositis. were 4 men and 23 women in the group. The mean age was 44.3 years.

756

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AmtiH;a;; a,

KIERLAND

SJi

Seven patients who were afebrile had pulse rates between 100 and 120 per minute, and only one of the 4 patients with temperatures of more than 99.6” F. had a ventricular rate of more than 100 per minute. Cardiac enlargement was evident in the roentgenograms of the thorax of 2 patients, without associated hypertension or evidence of valvular or coronary heart disease. Physical examinations disclosed heart murmurs in 9 patients, all systolic in time and of low intensity with the exception of a “sea gull” murmur, Grade 4, in one patient. These murmurs were heard over all valve regions except that of the tricuspid valve. Comparable data in 26 cases from the literature are listed along with our findings in Table 1. ELECTROCARDIOGRAPHIC

AND

HISTOPATHOLOGIC

DATA

It was to be anticipated that the diffuse type of myocardial involvement presumably occurring in some cases of polymyositis or dermatomyositis would be reflected in a nonspecific type of electrocardiographic abnormality. On the basis of previous studies it seemed reasonable also to conclude that the electrocardiogram would probably be normal in the majority of patients with this disease. In 19 (70 per cent) of this series of 27 patients the electrocardiograms were normal. A review of the literature indicates that 14 of 26 patients (54 per cent) had normal electrocardiograms3.6*gJ5-26 (Table II). II.

TABLE

INTERPRETATIONS

OF ELECTROCARDIOGRAMS AND POLYMYOSITIS

IN PROVED

I

MAYO

CASES

I

I

/

I

CLINIC

LITERATURE

-

Total cases Normal electrocardiogram Low

amplitude

Myocardial

QRS

27 19 in standard

*Necropsy tNecropsy SNecropsy

5 2 0

26 I

TOTAL

NO. ---.-

PERCENT

53

100

14*

33

62

st

1 10

2 (“myocarditis”) 4$ 2

---___-

j

4 I

19 7.5

I 4

7.5

2

4

block

hypertrophy in 4 cases, in 3 cases, in 2 cases,

1

--___-

involvement

Conduction disturbance Bundle branch block Intermittent sinoauricular Auricular fibrillation Ventricular

leads

OF DERMATOMYOSITIS

: 1

1

see text. wx text. see text.

The most frequent electrocardiographic patients in our series was a low-amplitude

abnormality QRS complex

in the remaining 8 in the standard leads

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which was present in 5 patients (Fig. 1). The criteria used in the interpretation of low amplitude in the standard leads were deflections of less than 5 mm. in all QRS complexes. 27 The interpretation of the electrocardiograms from the literature was that of the author reporting each case. The observation that only 5 of

Fig. I.-Typical

electrocardiogram

disclosing low amplitude noted in 5 of 27 patients.

of the QRS complexes

the 27 clinic patients with extensive skin and muscle disease disclosed this abnormality seems to indicate that cutaneous resistance or other local factors in the extremities would not explain this abnormality and that it is perhaps related to myocardial disease. It seems to be somewhat similar to myxedema in this respect. Electrocardiographic abnormalities indicative of previous anteroseptal myocardial injury, probably on the basis of coronary disease, were noted in two trac-

7.58

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ings in each group. This does not suggest, however, is part of the disease process of polymyositis.

that myocardial

Am. Heart J. May, 1957

infarction

Disturbances of conduction and rhythm, such as intermittentrr-19 sinoauricular block and auricular fibrillation, have been reported, but we did not encounter any of these disturbances. One of the patients with the relic of myocardial infarction subsequently had incomplete left bundle branch block. One patient had electrocardiographic evidence of left ventricular hypertrophy. The P-R interval was 0.12 to 0.18 second in 26 patients. In one patient it was 0.10 second. The QRS interval was 0.06 to 0.08 second in 23 patients, 0.10 second in 3 patients, and 0.12 second in one other patient. The Q-T interval was 0.32 second in one patient with a cardiac rate of 124 per minute. In another patient the Q-T interval was 0.40 second with a cardiac rate of 82 per minute. The amplitude of the T wave was 1.5 to 2 mm. in 17 patients, 2.5 to 3 mm. in 5 patients, 3.5 to 4 mm. in 2 patients, and 0.1 mm. in 3 patients. Two of the 19 clinic patients who had normal electrocardiograms subsequently died of their disease, and necropsy was performed. The myocardium of In this patient, mild changes of the rheumatic one of these patients was normal. type were noted in the mitral valve, and the coronary sclerosis was Grade 1 (on the grading basis of 1 to 4). In the second patient many foci of small scars were present in the myocardium. These scars contained macrophages and lymphocytes. The histologic changes in the myocardium in this case were not similar to those in the skeletal muscle and were considered to be of a nonspecific type (Table II). Necropsy was performed also in 9 of the cases reported in the literature. In 4 of these the electrocardiograms were normal. In one of these 4 cases a flabby heart was found; fragmentation and variation in size and shape of the fibers were evident.15 The heart was normal in the second case except for interstitial edema and focal round-cell infiltration.6 The hearts in the third and fourth cases were normal.21-24 In 3 cases in which necropsy was performed the electrocardiograms showed low amplitude of the QRS complexes in the standard leads. In one of these 3 cases, necropsy disclosed an edematous myocardium with moderate atrophy, but without any changes similar to those in the skeletal muscle.28 In the second case, pronounced interstitial edema of the myocardium was noted. 6 In the third case the mitral and aortic valves were sclerotic, the myocardium was pale but normal, and secondary fibrosis was present.16 Necropsy in one case of conduction disturbance disclosed myocardial degeneration with round-cell infiltration and beginning connective tissue formation.” In another case with right bundle branch block the heart was found to be normal at necropsy except for a myxoma of the pulmonary valve.18 COMMENT

Hence, this study does not indicate clinicopathologic correlation as to the frequency, type, or nature of the myocardial involvement presumably occurring in polymyositis. The relative similarity of the electrocardiographic data in our

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cases to those in the cases reported in the literature suggests that a nonspecific type of elect,rocardiographic abnormality is not unusual in patients with polymyositis, but occurs in only a minority of the patients. SUMMARY

AND

CONCLUSIONS

The electrocardiograms of 27 patients given histologic and clinical diagnoses of dermatomyositis or polymyositis at the Mayo Clinic, from 19.50 to 1954, and of 26 patients reported on in the literature were reviewed. ElectrocardThe electrocardioiograms were normal in 19 (70 per cent) of our patients. graphic changes when present were relatively infrequent and were nonspecific in nature. Low amplitude of the QRS complexes of the standard leads was the This was found in 5 of our 27 patients. Tachycardia, most frequent abnormality. out of proportion to fever, was a rather common manifestation, since it was noted in 8 of our patients. Arrhythmias and conduction disturbances were not noted in any of our patients, but a few were reported in the literature. REFERENCES

1. :: 4. :. 7: 9”: 10. 11. 12. 13. it: :? 18: 19. 20. 21. ;3: 2;1. 2 27: 28.

Wagner, E.: Quoted by Wolf, Abner, and Wilens, S. L.: Am. J. Path. 12:235, 19.36. M. Clin. North America 33:21, 1949. O’Leary, P. A.: Domzalski, C. A., and Morgan, V. C.: Am. J. Med. 19:370, 1955. Neuroloev 4:245. 1954. Eaton. L. M.: Kei!, harry: Arch. Int.?Med. &:109, 1940. Wamger, C. K., and Lever, W. F.: Arch. Dermat. & Syph. 59:196, 1949. O’Leary, P. A., Lambert, E. H.! and Sayre, G. P.: J. Invest. Dermat. 24:301, 195.5. GtiFther: Quoted by Lepeschkm, E., p. 252.a7 I&;:;, g. E., and Reinhart, J. B.: Arch. Dermat & Syph. 57:72.5, 1948. . The Uncommon Heart Diseases, Springfield, III., 1954, Charles C Thomas, ‘518 pp: Kinney, T. D., and Maher, Mary M.: Am. J. Path. 16:.561, 1940. O’Leary, P. A., and Waisman, Morris: Arch. Dermat. & Syph. 41:1001, 1940. Roberts, Helen M., and Brunsting! L. A.: Postgrad. Med. 16:396, 1954. Williams, Conger: Quoted by Wamger, C. K., and Lever, W. F.6 Smith, H. G.: Brit. M. J. 1:770, 1955. Goldman, Douglas: Arch. Int. Med. 70:822, 1942. Christensen, Erna, and Levison, Herman: Acta psychiat. et neurol. 25:137, 19.50. Case Records of the Massachusetts General Hospital: Case 37471, New England J. Med. 245:822, 1951. Jager, B. V., and Grossman, L. A.: Arch. Int. Med. 73:271, 1944. Anderson, T. E.: Brit. J. Dermat. 64:71, 1952. Case 38151, New England J. Med. Case Records of the Massachusetts General Hospital: 246:585, 1952. Friedman, E. D.: M. J. & Record 123:382, 1926. Hazel, 0. G., and Hull, W. M.: South. M. Jo 33:809, 1940. M. J, 38:184, Hoaglin, L. L., De May, G. H., Moody, W. B., and Schenken, J. R.: Nebraska 1953. Omens, D. V., Omens, H. D., and Kagen, M. S.: Arch. Dermat. & Syph. 53:188, 1946. Stuckey, E. S.: Brit. J. Dermat. 47:85, 1935. Lepeschki;, Eugene: Modern Electrocardiography, Vol. I, The P-Q-R-S-T-I’ Complex, Baltimore, 1951, Williams & Wilkins Company. Marcus, I. H., and Weinstein, Joseph: Ann. Int. Med. 9:406, 1935.